Download MBBS (Bachelor of Medicine, Bachelor of Surgery) Final Year Medicine Notes CNS
Nervous system
Letuda's ote
Version 2.0
Written, edited and digitalized by
Prithwiraj Maiti
Final year MBBS (Batch 2015)
R.G.Kar Medical College
03.08.2014
Table of contents
Contents
Page no.
Introduction to CNS
3-8
Contents: UMN lesion, LMN lesion, Corticospinal fibres,
Corticonuclear fibres, Specialty of facial nerve, Localization
of corticospinal tract lesion
Cranial nerves
9-27
Contents: CN1, CN2, Optic neuritis, CN3, CN4, CN6, Ptosis,
Squint, Light reflex, Accommodation reaction, CN5,
Corneal refle , Trige i al euralgia, CN , Bell's pals , CN ,
CN 9,10,11,12; Bulbar and pseudobulbar palsy, CN11
Examination of nervous system: General topic
28-34
Contents: Higher function, Motor function (tone, power,
reflex), Sensory function, Sensory-motor coordination
Spinal cord
35-53
Contents: Myelopathy, Compressive myelopathy,
Complete/ partial cord compression, Extramedullary/
intramedullary compression, Paraplegia in flexion/
extension, Chronic cord compression, Dx of cord
compression; Non-compressive myelopathy, Acute
2
transverse myelitis, Motor neurone disease, Amyotrophic
lateral sclerosis.
Cerebrovascular accidents (CVA)
54-71
Contents: Transient ischemic attack (TIA), Stroke, Lacunar
infarct, Brainstem signs, Subarachnoid hemorrhage (SAH),
Saccular aneurysm.
Neuropathies
72-81
Contents: GB syndrome, Miller-fisher syndrome, Polio,
Peripheral
neuropathy, Myasthenia gravis, Lambert-Eaten
Myasthenia Syndrome
Miscellaneous topics
82-92
Contents: Subacute combined degeneration of spinal cord,
Fredrick's ata ia, Tabes dorsalis, Multiple sclerosis,
Parkinsonism, Conus medullaris and Cauda equina lesions.
Myopathy
93-95
Contents: Myopathy in general, Myotonic dystrophy,
Duche e's uscular d stroph , Becker's uscular
dystrophy.
Infective diseases of CNS
96-102
Contents: Pyogenic meningitis, Tubercular meningitis,
Brain abscess.
Brain tumor, seizure and related topics
103-115
Contents: Brain tumor, Pseudotumor cerebri/ Benign
intracranial hypertension (BIH), Seizure, Status epilepticus.
Syncope and headache
116-122
Contents: Syncope, Headache, Migraine, Tension
headache, Cluster headache.
Extras
123-127
Contents: Involuntary movements (Tremor, Chorea,
Athetosis, Fasciculation), Dementia
3
Introduction to nervous system
Upper and lower motor neuron (UMN and LMN)
There are 2 main groups of nerve fibres coming down from the cerebral cortex:
1. Corticospinal fibres
2. Corticonuclear fibres.
These 2 group of fibres relay on the following stations respectively:
1. Anterior horn cell
2. Cranial nerve nuclei.
Til these 2 statio s, the pathway are alled upper otor euro s .
After this stations, the following 2 sets of nerves starts respectively and they
follow their original course:
1. Spinal nerves
2. Cranial nerves.
They o prise the lower otor euro s .
4
Actions of UMN and LMN:
Through upper motor neurons, cortex exerts a negative control over the
lower motor neurons. So, the main function of UMN is control of voluntary
movements.
The main function of LMN is control of muscle movements.
Basic feature of UMN and LMN lesions:
UMN lesion: Loss of voluntary movement
LMN lesion: Paralysis of muscle.
Details of features of UMN and LMN lesions:
Points
UMN lesion*
LMN lesion*
Muscle power
Decreased
Muscle tone
Hypertonia/ spasticity
Hypotonia/ flaccidity
Wasting/ atrophy of
Absent
Present
muscles
(due to denervation)
Deep tendon reflex (DTR)/
Exaggerated
Absent
Jerk/ Stretch reflex
(clonus may be present)
Superficial reflex
Planter: Extensor
Plantar: Unresponsive
Other reflexes: Lost
Other reflexes: Lost
Above signs will be present in that part of the body which is innervated by the affected/
damaged UMN/LMN.
*UMN may be described as the overhead wire over the railways and LMN may be
described as the rail track. If the overhead wire gets cut, there is still a chance of
running the train by other methods (like motor/ diesel etc.) but if the rail track is
damaged, there is no way to run the train.
Just like that, if UMN is damaged, the muscles can still work (and in reality they
work with hyperactivity due to loss of cortical control) but if LMN is damaged, the
us les a t work at all a d they e o e fla id, atrophied with loss of all
reflexes.
5
Corticospinal fibres
Course
Corticospinal fibres
This level is
very important
6
Corticospinal fibres control movement of contralateral (opposite) half of
the body.
So, if corticospinal tract is damaged in the brain, effect will be contralateral
to the side of lesion.
If corticospinal tract is damaged within the spinal cord, effect will be
ipsilateral (same) to the side of lesion.
So, in a corticospinal tract lesion, UMN signs will be present in that part of
the body which is controlled by the fibres below the level of the lesion.
Corticonuclear fibres
Course
Corticonuclear fibres
7
They arise from cortex and ultimately innervate different motor cranial
nerve nuclei in the brainstem, forming UMN pathway of that particular
cranial nerve.
Therefore, all of the motor cranial nerve nuclei are bilaterally innervated by
corticonuclear fibres (except lower half of 7th cranial nerve nucleus).
The case of 7th cranial nerve corticonuclear fibres
CN7 nucleus has an upper ? and a lower ? from which, upper and lower ?
of facial muscles are innervated.
Corticonuclear fibres of the upper ? of the CN7 nucleus act exactly like the
other motor cranial nerve nuclei. Therefore, upper ? of CN7 nucleus is
innervated by corticonuclear fibres from both side of motor cortex.
But, corticonuclear fibres destined for lower ? of CN7 nucleus leave rest of
the pyramidal fibres at the level of midbrain and decussate to innervate the
nuclei.
Therefore, lower ? of CN7 nucleus has got unilateral corticonuclear
innervation which comes from contralateral side.
8
Localization of corticospinal tract lesion (compare with the pictures above)
Site
Structure damaged
Effect
Corticospinal tract
Contralateral hemiplegia
Corticonuclear fibre innervating
Paralysis of contralateral lower ? of
lower ? of CN7 nucleus
face (UMN type CN7 palsy)
Cortex
Higher cortical areas
Higher cortical dysfunction
(Ex: speech area damage: aphasia)
Fibres of visual field
Visual field defect
Because the pyramidal fibres are widely separated in cortex, a cortical
lesion often causes isolated monoparesis/ asymmetrical weakness of the
limbs/ weakness of contralateral lower ? of face.
Internal
Corticospinal tract
Contralateral hemiplegia
capsule
Corticonuclear fibre innervating
Paralysis of contralateral lower ? of
lower ? of CN7 nucleus
face (UMN type CN7 palsy)
Midbrain
Corticospinal tract
Contralateral hemiplegia
Corticonuclear fibre innervating
Paralysis of contralateral lower ? of
lower ? of CN7 nucleus
face (UMN type CN7 palsy)
CN3 nucleus
Ipsilateral LMN type CN3 palsy
CN4 nucleus
Ipsilateral LMN type CN4 palsy
Pons
Corticospinal tract
Contralateral hemiplegia
Corticonuclear fibre innervating
Paralysis of contralateral lower ? of
lower ? of CN7 nucleus
face (UMN type CN7 palsy)
CN6 nucleus
Ipsilateral LMN type CN6 palsy
Principal sensory nucleus of CN5
Loss of touch sensation from
ipsilateral ? of face
Sympathetic trunk
Ipsilateral Hor er s sy dro e
Lateral spinothalamic tract
Loss of pain and temperature
sensation from contralateral ? of body
Medulla
Corticospinal tract
Contralateral hemiplegia
CN 9,10,11,12 nucleus
LMN type bulbar palsy
Sympathetic trunk
Ipsilateral Hor er s sy dro e
Lateral spinothalamic tract
Loss of pain and temperature
sensation from contralateral ? of body
Spinal nucleus of CN5
Loss of pain and temperature
sensation from ipsilateral ? of face
9
Cranial nerves
CN1
Simplified olfactory pathway
Hyposmia/ Anosmia:
1. Transport defect: DNS/ Polyp/ Rhinitis
2. Receptor defect: Viral infection
3. Neural defect:
a. Trauma/ fracture of cribriform plate
b. Tumor: Meningioma of olfactory groove
c. Alzhei er s disease
d. Chronic smokers
e. Kal a
s sy dro e a os ia + hypogo adis
f. Foster-Kennedy syndrome (anosmia + ipsilateral optic atrophy + contralateral
papilledema).
CN2
Functions of the optic nerve
1. Visual acuity
2. Color vision
3. Visual field.
10
(White: Lost field of vision)
Lesions at different levels and their effects
Level of lesion
Effect
Optic nerve
Monocular loss of vision from both the fields of the affected
side
In the affected eye:
Direct light reflex (DLR): Absent
Consensual light reflex (CLR): Preserved
- This defect is called RAPD (Relative afferent pupillary
defect)/ Marcus Gunn Pupil.
Optic chiasma
Bitemporal hemianopia (both temporal fields are lost)
Some of the important causes are:
Pituitary tumor
Supra-sellar tumor
Craniopharyngioma.
Optic tract, LGB and optic radiation lesion: Homonymous hemianopia
(identical half of the visual field of each eye is lost), crossed/ contralateral in types (as
the contralateral half of the visual field is lost).
11
Optic tract
Congruous in type (as the fibres within optic tract are densely
arranged; when the visual field loss is mapped out, they look
symmetrical/ identical).
Optic radiation
Congruous in type (The fibres within optic radiation are
widely separated, but the fibres responsible for superior and
inferior quadrantic vision group together and pass through
parietal and temporal lobes, respectively).
Optic neuritis (SN)
Introduction
Inflammation of optic nerve.
Etiology
1. Multiple sclerosis (MS)
2. Viral infection
3. Devi s disease.
Clinical features
1. Monocular loss of vision
2. Orbital/ retro-orbital pain, particularly on upward gaze due to inflammation of
the superior rectus tendon
3. Loss of vision from nasal as well as temporal field
4. Affected eye: DLR lost but CLR present.
Investigation
MRI orbit
Treatment
High dose corticosteroid
CN3, CN4 and CN6
Function
1. Innervate extraocular muscles and thereby mediate movement of eyeball.
Innervation of EOM: [ALL]3 [SO]4 [LR]6
12
2. CN3 innervates Levator palpebrae superioris (LPS), which is an eyelid elevator.
3. CN3 causes constrictor pupillae.
4. CN3 mediates accommodation reaction.
Affected nerve
Symptoms and sign of palsy
CN3
Symptoms
Diplopia: When the patient tries to look towards the field of
vision of the affected muscle, diplopia occurs.
Often diplopia gets masked due to complete ptosis.
Ptosis: Drooping of the upper eyelid.
Signs
Attitude of the eyeball: Divergent squint
Weakness of the affected muscle is elicited
Light reflex: Lost (as the efferent pathway is through CN3)
Accommodation reflex: Lost.
CN4
Diplopia: Particularly when the patient climbs downstairs
(Superior oblique action acts more)
Weakness of superior oblique is elicited.
CN6
Diplopia: On lateral gaze of the affected eye (Lateral rectus acts
more)
Attitude of the eyeball: Convergent squint.
13
Some important causes of CN3, CN4 and CN6 palsy
Causes of CN3 palsy
Group
Cause
Diseases of midbrain
CVA
Tumor
Abscess
Meningeal diseases
In bacterial meningitis, the inflammatory exudate often
strangulate the nerve at the basal meninges.
Diseases of
Hemorrhage: Particularly in aneurysm of posterior
Subarachnoid space
communicating artery (PCA).
Diseases of
Cavernous sinus thrombosis
Cavernous sinus
Carotid-cavernous fistula
Lateral extension of pituitary (uncommon).
Orbital diseases
Tumor
Cellulitis.
Others
Mononeuritis multiplex (secondary to diabetes and
vasculitis)
14
Causes of CN4 palsy (rare)
1. Head injury
2. Midbrain diseases
3. Meningeal diseases
4. Cavernous sinus diseases
5. Orbital diseases.
[Point 2-5 are same as CN3 palsy]
Causes of CN6 palsy
1. Raised ICT: As CN6 has the longest intracranial course, fibres often get stretched
when ICT raises; giving rise to some false localizing signs in addition to specific
signs of CN6 palsy.
2. Pontine lesion (as the nucleus of CN6 lies in the pons)
3. Meningeal diseases
4. Cavernous sinus diseases
5. Orbital lesions
6. Mononeuritis multiplex.
[Points 3-6 are same as CN3 palsy]
Some short notes related to CN3, 4 and 6
Ptosis (SN)
Introduction
Drooping of upper eyelid.
Causes of
ptosis
Horner's
Myasthenia
Ocular
CN3 palsy
syndrome
gravis
myopathy
Damage to
Sympathetic
Pathology in
Weakness of
nucleus
trunk damage
N-M junction
mucle itself
Damage to
Muller's
In Myotonic
nerve fibre
muscle palsy
dystrophy
15
Clinical features according to the causes of ptosis
CN3 palsy
Hor er s sy dro e
Myasthenia gravis
Complete ptosis
Partial ptosis/ pseudo-ptosis
Fatigable ptosis
Non-fatigable
Non-fatigable
Usually unilateral
Usually unilateral
Usually bilateral
Pupil: Large
Pupil: Small
Pupil: Normal
Other signs of CN3 palsy are
Other features of Hor er s
Fatigable weakness of other
present
syndrome are present
muscles in the body may be
present
Squint: Brief discussion
Squint is of 2 types, the features of which are being discussed in brief:
Non-paralytic
Paralytic
Present since birth
Usually develops later
No diplopia
Diplopia present
Movement of eyeball not restricted
Movement of eyeball restricted
(as there is weakness of EOM)
No signs of cranial nerve palsy
Signs of cranial nerve palsy present
Light reflex
Pathway of light reflex
Light reflex (LR)
fibres [emerging
Optic nerve
Optic tract
from retinal ganglion
cells]
Parasympathetic
Before LGB, LR fibres
Ipsilateral and
fibres accompanying
leave and terminate
contralateral EWN
CN3 (not branches of
in pretectal nucleus
CN3)
Constrictor/
Ciliary ganglion
Short ciliary nerve
Sphincter pupillae
16
Note: Parasympathetic fibres (which constricts pupil) pass along CN3 fibres and
therefore, often damaged in CN3 nerve lesions.
Loss of light reflex
Causes:
1. Optic nerve lesion
2. Optic tract lesion (pre-geniculate lesion):
The affe ted eye i this ase is al ed Wer icke's he ia opic pupil : DLR is lost i
this eye when light is thrown from nasal half, but DLR is present when light is
thrown from temporal half. (Remember- W:DT)
In contralateral eye: DLR is lost when light is thrown from temporal half and DLR
is present when light is thrown from nasal half.
3. CN3 palsy
4. Argyll Robertson Pupil (SN):
It is an abnormality of pupil usually seen in patients of neurosyphilis.
Component:
a. Usually bilateral
b. Pupil is small in size and dilates poorly to mydriatics
17
c. Light reflex is lost
d. Accommodation reaction: Present (Remember: ARP in ARP).
Accommodation reaction
Component:
1. Medial convergence of eyeball
2. Pupillary constriction
3. Anterior-posterior bulging of lens.
Lost accommodation reaction:
Seen in CN3 palsy (as CN3 supplies both medial rectus and constrictor pupillae).
CN5
Functions:
1. Motor function:
Innervates masticatory muscles:
a. Temporalis
b. Masseter
c. Pterygoid.
2. Sensory function:
Carries superficial sensation from ipsilateral half of face through 3 branches:
a. Ophthalmic
b. Maxillary
c. Mandibular.
3. Reflex:
CN5 mediates the following reflexes:
a. Corneal reflex
b. Jaw jerk.
18
Corneal reflex pathway
Loss of corneal reflex
Causes:
1. CN5 palsy (damage to ophthalmic division):
In the affected side, direct corneal reflex will be lost but consensual corneal reflex
will be present.
2. CN7 palsy:
In the affected side, both type of corneal reflex (direct and consensual) will be
lost.
3. Deep coma.
Features of CN5 palsy
1. Motor dysfunction:
a. Weakness of masticatory muscles leading to difficulty in mastication
b. Wasting of temporalis and masseter, leading to hollowing of the face.
2. Sensory dysfunction:
Loss/ impairment of sensation from ipsilateral half of face.
In many cases, only one sensory branch of trigeminal nerve gets damaged,
leading to sensory impairment from the distribution of the affected branch
only.
19
3. Loss of corneal reflex.
Causes of CN5 palsy
1. Brainstem lesion: CVA
a. Pontine lesion:
Motor paralysis
Touch sensation lost
20
b. Medullary lesion:
Pain sensation lost
Temperature sensation lost
2. Posterior fossa lesion:
CP angle tumor (Acoustic neuroma)
3. Trigeminal ganglion lesion:
a. Tumor
b. Trauma
c. Grade igo s sy dro e
4. Cavernous sinus pathology:
Thrombosis.
Trigeminal neuralgia (SN)
Introduction
It is a condition characterized by excruciating pain along the distribution of trigeminal
nerve.
Etiology
1. Idiopathic
2. Multiple sclerosis
3. Intracranial space occupying lesion (IC-SOL): Tumor stretches the nerve fibres
4. In some cases, the nerve gets irritated by an aberrant blood vessel.
Clinical features
Pain:
a. Site: Usually hemifacial pain, particularly in the chick and chin
b. Nature: Severe, often electric shock like pain and typically paroxysmal
c. Triggering factor: Often triggered by face washing, shaving, chewing
d. The pain is so severe that it throws the face; causing facial spasm and the patient
involuntarily starts wincing like a tic; therefore the condition is also called Tic
douloureux*.
[* It has been described as among the most painful conditions known to humankind.]
21
Investigation
Often not required as it is a clinical diagnosis. Relevant investigations to look for any
underlying diseases are performed in selected cases.
Treatment
1. Medical:
Carbamazepine/ Oxcarbamazepine
2. Interventional:
a. Radiofrequency ablation of trigeminal ganglion
b. Microvascular compression of aberrant vessels.
CN7
Functions:
Innervates all muscles of face except masticatory muscles (muscles of facial expression)
which are supplied by CN5.
There are 2 other types of fibres which accompany facial nerve almost along its entire
course and therefore considered to be a part of CN7:
a. Secretomotor fibres to (lacrimal + sublingual + submandibular) gland
b. Taste fibre from ant. 2/3rd of the tongue (post 1/3rd: supplied by CN9).
CN7 palsy
It is of 2 types:
UMN type of CN7 palsy
LMN type of CN7 palsy
Due to lesions of the corticonuclear fibres Due to nucleus damage/ infra-nuclear
innervating lower ? of contralateral CN7
lesion
nucleus
Lesion is anywhere above pons (where
Lesion is in the pons/ anywhere along the
CN7 nucleus lies)
course of CN7
Feature:
Feature:
Weakness/ paralysis of lower ? of
Weakness/ paralysis of whole of the
contralateral side of face
ipsilateral side of face
22
Features of CN7 palsy
Inability to elevate the eyebrows
Loss of forehead furrowing/ wrinkling
Inability to frown
Inability to close the eyelid:
Eyeball is seen to be rolled upwards on attempted closure of eye. This is called
Bel 's phe o e o . Su h a eye is at risk of developing keratitis.
Weakness of buccinator leading to following manifestations:
On puffing out of chick, air leaks through the angle of the mouth of the
affected half
Loss of nasolabial fold
Lower eyelid sacs down so that punctum no longer remains in contact.
Drooping of the angle of the mouth to the affected side: saliva dribbles through
angle of mouth
Angle of mouth is deviated towards healthy site when patient attempts to smile.
23
Localization of LMN type of Facial palsy
Site of lesion
Disease
Features (Compare with the picture above)
Pons
CVA
a. Ipsilateral LMN type CN7 palsy
b. Ipsilateral CN6 palsy
c. Contralateral hemiplegia (CST damage)
d. Loss of secretomotor (taste) function.
CP angle
Acoustic neuroma
a. Ipsilateral LMN type CN7 palsy
b. Ipsilateral CN5 palsy
c. Ipsilateral sensorineural deafness (CN8 damage)
d. Ipsilateral cerebellar signs
e. Loss of secretomotor function.
Temporal bone
Trauma
a. Ipsilateral LMN type CN7 palsy
Tumor
b. Loss of secretomotor function
Petrositis
c. Hyperacusis (nerve to stapedius)
Grade igo s sy dro e
Geniculate
Herpes zoster (Ramsey-
Same as temporal bone lesion + Painful herpetic vesicles
ganglion
Hunt syndrome)
over external ear and pinna
Stylomastoid
Bel s palsy
Ipsilateral LMN type CN7 palsy
foramen
Parotid gland
Tumor
Ipsilateral LMN type CN7 palsy
24
Surgery
Peripheral
GB syndrome
Ipsilateral LMN type CN7 palsy
branches
Neurosarcoidosis
Ly e s disease
Complication of forceps
delivery
Bell's palsy SN
Introduction
It is a rapidly developing LMN type of ipsilateral CN7 palsy due to inflammation of the
nerve at/near the stylomastoid foramen.
Etiopathogenesis
It has been postulated that the inflammation of nerve is probably triggered out by HSV
infection. Rapidly developing inflammatory exudate strangulates the nerve, leading to
palsy. Because there is no permanent structural damage of the nerve in many cases,
there is usually complete functional recovery.
Clinical features
1. Onset: Usually acute/ subacute: develops over few days
2. Often there is H/O a preceding exposure to cold air/ an attack of common cold
3. Clinical features of CN7 palsy.
Investigation
None required as it is a clinical diagnosis.
Treatment
1. Drugs:
A. Systemic corticosteroid: Most effective if given within 24-48 hours of onset of
symptoms. Usually a course of 10-14 days is given.
B. Acyclovir: Although its role is doubtful, many clinicians prefer to prescribe it.
2. Prevention of exposure keratitis:
A. Protective eyepad
B. Lubricant eye drop/ ointment.
3. Physiotherapy.
25
Complications
1. Incomplete recovery leading to residual palsy
2. Contracture of facial muscles
3. Aberrant regeneration:
a. Fibres originally innervating facial muscles side-track and innervate lacrimal
gland, leading to lacrimation during eating (gustatory lacrimation/ crocodile
tear).
b. Fibres originally innervating orbicularis oculi side-track and innervate
orbicularis oris, leading to involuntary twitching of angle of mouth during
attempted closure of eye.
CN8
CN 9, 10, 11 & 12
These cranial nerves are also called ulbar cranial nerves as the nuclei lies in the bulb
(medulla).
Functions:
CN9: Carries sensation from pharynx, palate, tonsillar region; taste sensation
from post. 1/3rd of tongue.
CN10: Supplies all muscles of pharynx (except stylopharyngeus, which is supplied
by CN9), palate and intrinsic muscles of larynx.
CN11: Supplies sternocleidomastoid (rotate the head towards the opposite side)
and trapezius (elevation and retraction of shoulder).
CN12: Supplies tongue muscles.
Reflexes mediated by bulbar cranial nerves:
1. Pharyngeal/ Gag reflex:
On stimulating the posterior pharyngeal wall/ palate with a swab stick, there is
reflex contraction of pharyngeal/ palatal muscles, respectively.
26
The afferent pathway is formed by: CN9
The efferent pathway is formed by: CN10.
2. Jaw jerk:
Patient is asked to open his mouth slightly and an area of chin just below the
lower lip is tapped, which leads to reflex closure of mouth.
In most normal individuals, response is non-elicitable and therefore, if the reflex
deviation of lower jaw can be seen clearly, the jerk is considered to be brisk/
exaggerated.
Causes of bulbar palsy
Bulbar palsy
UMN Type/ Pseudobulbar palsy
LMN type/ True bulbar palsy
Damage to the corticobulbar
fibres of both side
Nucleus damage
Infranucleur lesion
Multi-infarct cerebrovascular
GB syndrome
disease
CVA
Motor neurone disease
MND
Bulbar poliomyelitis
Myelin sheath damage:
Diphtheritic polyneuritis
Cerebral pontine myelinosis
Symptoms and signs of bulbar palsy (Pharyngo-laryngo-palatoglossal palsy)
Site
Symptom/ sign
Pharynx
Dysphagia
Nasal regurgitation of food
Larynx
Dyspnea
Recurrent episodes of aspiration
pneumonia/ choking
Palate
Dysarthria
Nasal intonation of voice
27
Differences between UMN type (pseudobulbar) and LMN type (true) bulbar palsy
Features
UMN type (Pseudo) bulbar palsy
LMN type (True)bulbar palsy
Dysarthria
Spastic dysarthria
Flaccid dysarthria
Wasting
No wasting
Wasting present
Fasciculation
No fasciculation
Fasciculation present
Jaw jerk
Brisk
Absent
Gag reflex
Lost
Other feature
Emotionally unstable (laughs
No such feature
without any cause)
Special features
As reflex movements are often
As all movements are affected,
spared, there features are not so
nasal intonation of voice and
prominent
nasal regurgitation of food are
quite prominent
CN11 palsy
Clinical features
Sternocleidomastoid (SCM)
1. Paralysis/ weakness of SCM: Inability to rotate the head towards the opposite
side
2. Wasting of SCM (in LMN type of lesion)
3. On attempted neck flexion, chin deviates towards the affected side.
Trapezius (TZ)
1. Inability to retract and elevate the shoulder
2. Wasting of TZ leading to loss of normal curvature of shoulder (in LMN type of
lesion).
28
General topics
Topics to be discussed:
1. Higher function
2. Motor function: Tone, Power, Reflex, Atrophy
3. Sensory function
4. Sensory-motor coordination.
Higher function
Speech:
a. Dysphasia
b. Aphasia:
Wer i ke's
Bro a's.
Features
Bro a's aphasia
Wer i ke's aphasia
Description
Due to lesion of inf.frontal gyrus
Due to lesion of sup. temporal gyrus
(supplied by sup.division of MCA)
(supplied by inf.division of MCA)
Comprehension Intact
Impaired
Expression
Impaired
Voluminous
Word finding difficulty
Contains little information
Short answers
Full of paraphasic errors
Non-fluent aphasia/
Neologism
telegraphic speech.
Fluent aphasia/ Jargon speech
29
Motor function
Tone of muscles
Tone
Hypotonia/
Hypertonia/ Rigidity
Flaccidity
Klasp knife type
Shock stage of UMN
Parkinsonism
(UMN lesion)
lesion
Lead pipe type
LMN lesion (same
(uniform; rigidity >
segment)
tremor)
Cogwheel type
(interrupted,
Cerebellar lesion
tremor> rigidity)
30
Power of muscles
Power
Description
0
No movement
1
Flickering movement
2
Unable to move against gravity
3
Movement possible against gravity but not against resistance
4
Movement possible against resistance but with some weakness
5
Normal power
Reflex
Deep tendon reflex (DTR)
Reflex
Spinal level
Peripheral nerve
Biceps jerk
C5-C6
Musculocutaneous nerve
Supinator jerk
C5-C6
Radial nerve
Triceps jerk
C6-C7
Radial nerve
Finger flexion jerk
C7-C8
Median and ulnar nerve
Knee jerk
L2, L3, L4
Femoral nerve
Ankle jerk
L5, S1
Sciatic nerve
Abnormality of deep tendon reflexes:
1. Brisk/ exaggerated:
Cause: UMN lesion from non-neurological causes (anxiety/ thyrotoxicosis)
Below the level of lesion, all reflexes will be brisk.
2. Clonus:
It is the external manifestation of an exaggerated DTR characterized by
rhythmical repeated contraction of a muscle in response to sudden
sustained stretching of its tendon, leading to oscillatory movement of a part
of the body. It is best seen in the knee (patellar clonus) and in the ankle
(ankle clonus).
31
Features
Pseudo-clonus/
True-clonus/
Physiological clonus
Pathological clonus
Extension
Ill sustained (<5-6)
Well sustained (7)
Signs of UMN lesion
Absent
Present
Cause
Anxiety
UMN lesion
3. Absent deep reflex:
Causes:
LMN lesion: Reflex(es) mediated by the particular damaged LMN
pathway will be lost
Spinal shock stage of UMN lesion.
Je drassik's Ma euver
It is a special maneuver performed while examining DTR when they seem to
be absent/ diminished. It must be performed before concluding that the
jerk is absent.
In case of upper limb jerks, the patient is asked to clench his teeth tightly.
In case of lower limb jerks, the patient is asked to hold his fingers tightly
with each other and suddenly pull them up.
Tapping of the tendon should coincide with this maneuver.
Mechanism: This maneuver increases the excitability of reflex response by:
a. By increasing the excitability of AHC
b. B i reasi g the re ruit e t of -fusiform fibres of the muscle spindle.
Superficial reflex
Reflex
Spinal level
Abdominal reflex
T7-T12
Cremasteric reflex
L1-L2
Plantar reflex
S1
Anal reflex
S2-S4
Bulbocavernosus reflex
Loss of superficial reflex:
In UMN lesion: Below the level of lesion
In LMN lesion: At the level of lesion.
32
Plantar reflex
Normal response:
1. Plantar flexion of the great toe
2. Plantar flexion and adduction of the other toes
3. Contraction of tensor fascia lata.
Why the lateral aspect of the sole is stroke?
The reflexogenic area of the plantar reflex lies on the lateral aspect of sole
If the medial aspect of sole is stroke, plantar reflex occurs due to grasp
response.
Wh the all of the great toe should 't e stroke?
If it is stroke, due to direct stimulation of muscle fibres; dorsiflexion of toe occurs
giving an erroneous interpretation of extensor plantar.
Abnormality of plantar response:
Extensor plantar/ Babinski's sign/ Upgoing plantar
1. Dorsiflexion of great toe
2. Dorsiflexion and fanning out of the other toes
3. Triple flexion: Dorsiflexion at ankle + Flexion at knee + Flexion at hip.
33
Causes of Extensor plantar:
1. Structural damage of corticospinal/ pyramidal tract (usually bilateral
extensor is seen)
2. Functional impairment of corticospinal tract:
a. Newborn infant
b. Any cause of deep unconsciousness/ coma
c. Post-ictal stage.
Absence of plantar response:
1. LMN lesion of S1 segment
2. Thick sole
3. Anesthesia of sole.
Withdrawal response:
When the entire limb is withdrawn from nociceptive stimulation (usually occurs
when patient is very sensitive to touch/ the sole is stroke forcefully).
Equivocal response:
Incomplete response. This is considered to be a part of extensor plantar response.
Ex: Great toe dorsiflexed but other toes plantiflexed.
Plantar equivalent:
In some patients with UMN lesion, the reflexogenic area of plantar reflex widens;
leading to extensor plantar response when different parts of the lower limb are
stimulated. Ex:
Gordo 's sig : When the calf muscle (Gastrocnemius) is squeezed
Chaddock's sig : When the lateral malleolus is stroke in semicircular
pattern
Oppenheim's sign: When pressure is applied over the medial side of tibia in
a downward direction.
Causes of extensor plantar with absent ankle and knee jerk
1. Spinal shock stage of UMN lesion
34
2. Compressive myelopathy where compression occurs in the fragment
controlling the above jerk (S1)
Other uncommon causes:
1. Subacute combined degeneration of spinal cord
2. Fredri k's ata ia
3. Tabes dorsalis.
Muscle atrophy (Wasting)
Causes:
1. LMN atrophy
2. Myopathy.
Pseudo-hypertrophy:
The muscle bulk looks high due to abnormal accumulation of fibro-fatty tissues. It
occurs typically in Duche
e's uscular dystrophy (which is a congenital
myopathy) typically affecting the calf muscle.
Sensory function
1. Pain (mediated by lateral spinothalamic tract)
2. Temperature (-do-)
3. Touch (mediated by dorsal column)
4. Joint, position and vibration (-do-)
5. Cortical sensation.
Sensory-motor coordination
1. Finger nose test
2. Finger nose finger test
3. Keel knee test.
35
Spinal cord
Important structures of spinal cord:
Motor:
Corti ospi al tra t
Anterior horn cell
Sensory:
Lateral spi othala i tra t
Dorsal olu .
[: As e di g tra t, : Des e di g tra t]
Myelopathy
Introduction:
Diseases affecting spinal cord.
Types:
Acute
Compressive
Chronic
Myelopathy
Acute
Non-
compressive
Chronic
Compressive myelopathy
Introduction:
Compression of different structures within the spinal cord.
Structures which gets compressed in compressive myelopathy:
1. Anterior horn cell (AHC)
36
2. Corticospinal tract (CST)
3. Spinothalamic tract: Lateral and anterior
4. Dorsal column
5. Dorsal root
6. Fibres controlling sphincteric function.
Etiology:
Compressive
myelopathy
Acute cord
Chronic cord
compression
compression
Trauma
Intramedullary
Extramedullary
(Vertebral
fracture/
collapse)
Intradural
Extradural
Tumor
Hematomyelin
Menionioma
Tumor
Osteoporotic
(Bleeding within
crush fracture
spinal cord)
Vertebral
Neurofibroma
Spinal epidural
metastasis
abscess
Degenerative
Glioma
disc disease
Prolapsed
intervertebral
disc (PIVD)
Bronchogenic
TB spine
Breast CA
CA
Ependymoma
Gynaecological
Bowel CA
cancer
Prostate CA
37
Symptoms:
1. Motor:
Symmetrical
Asymmetrical
Weakness
Monoparesis
Acute
Paraparesis
Chronic
Qudriparesis
2. Sensory:
a. Impairment/ loss of sensation
Symmetrical
Asymmetrical
Impairment/
loss of
sensation
Unilateral
Acute
Bilateral
Chronic
Often patient complain of a definite upper level below which sensations
are impaired.
38
b. Root pain:
Site: In the dermatome of the compressed root
Character: Often severe, deep seated pain, may be aggravated
with movements, coughing, sneezing.
c. Girdle sensation:
A constricting sensation in a dermatome due to irritation of sensory
fibres/ posterior root. Classically seen in thoracic cord compression.
3. Sphincteric disturbances:
a. Bowel dysfunction: Incontinence
b. Bladder dysfunction: Retention/ incontinence.
Signs:
Complete cord compression/ transection
At the level of compression:
1. Bilateral LMN lesion (due to damage to AHC)
2. Impairment of all sensations due to compression of dorsal root/ sensory
fibres entering through it.
Below the level of compression:
1. Bilateral UMN lesion (due to damage to CST of both side)
2. Impairment of all sensory modalities (superficial and deep) bilaterally.
39
- Therefore in complete transection of spinal cord, the upper level of sensory
impairment corresponds to the dermatome of compressed segment.
Partial/ incomplete cord compression/ hemisection of spinal cord
At the level of compression:
1. Ipsilateral LMN sign (due to damage to AHC)
2. Impairment of all sensations in the dermatome of compressed segment:
Ipsilateral (damage to dorsal root).
Below the level of compression:
1. Ipsilateral LMN sign (due to damage to ipsilateral CST)
2. Impairment of deep sensations of ipsilateral side
3. Impaired pain and temperature sensation of the contralateral side
below the level (due to damage of contralateral LSTT),
Signs just below the level of compression:
A zone of hyperalgesia may be present due to irritation of lowest viable
segment by compressive lesion.
40
Localization of lesion
A probable localization of the level of spinal cord lesion can be assessed by noting
some of the following features:
1. Distribution of weakness:
High cervical lesion (C1-C5): Quadriparesis
Low cervical lesion (C6-C8): Quadriparesis with proximal part of the
upper limb spared
Thoracic/ lumber lesion: Paraplegia.
2. Distribution of LMN signs:
Seen in the same segment.
3. Upper level of sensory loss
4. Vertebral tenderness/ deformity:
Level of vertebral tenderness/ deformity
Related spinal cord segment
Cervical
+1
T1-T6
+2
T7-T9
+3
T10
L1
T11
L2-L3
T12
L4-L5
L1
Sacral segments
5. Signs and symptoms at different spinal cord segments:
41
Affected nerve root
Signs and symptoms
C1-C4
Vasomotor and respiratory dysfunction
Diaphragmatic palsy (as phrenic nerve emerges from
C3-C5 spinal segments)
If bilateral: Paradoxical respiration*.
C5-C6
Normal shoulder movement
Weakness of elbow flexion
Biceps jerk/ Supinator jerk: Lost
Inverted (Biceps + Supinator) jerk^ may be present
Brisk triceps jerk.
C6-C7
Weakness of (elbow + wrist) extension
Triceps jerk: Lost
Brisk finger flexion jerk.
C7-C8
Weakness of (wrist + finger) flexion jerk.
Thoracic cord (T1-
Paraparesis
T8)
Usually the upper level of sensory loss lies
somewhere on the trunk. Ex:
Nipple line: T4 dermatome
Umbilical line: T10 dermatome.
T10-T12
Umbilicus is pooled upwards on attempting to raise the
head as upper abdominal muscle contracts normally but
lo er a do i al us le a t. It is alled Bee or s sig ~.
L2-L4
Weakness of hip flexion and knee extension
Loss of knee jerk (L2-L4)
Plantar: Bilateral extensor.
L4-L5
Weakness of hip extension, knee and ankle flexion
Loss of ankle jerk (L5-S1).
[*Paradoxical respiration: It is a life-threatening medical condition that
occurs when a segment of the rib cage breaks under extreme stress and
becomes detached from the rest of the chest wall. It occurs when multiple
adjacent ribs are broken in multiple places, separating a segment, so a part
of the chest wall moves independently.
^Inverted biceps jerk: In some cases of compressive myelopathy of C3-C6
segment, a combination of biceps and supinator jerk elicits an elbow
extension (C5-C6) and finger flexion response (C7-C8), respectively.
42
Compression of C5-C6 leads to exaggerated reflex response of C6-C7 and
C7-C8 due to UMN lesion. However, in some cases, these segments become
so hyperexcitable that attempted biceps and supinator jerk will lead to a
response which is normally mediated by these hyperactive segments.
~ Bee or s sig :
The rectus abdominis muscle at the level of the umbilicus is supplied by the
T10 nerve roots. Lesions of the spinal cord or roots between T10 and T12
will cause weakness of the lower part of the muscle, and thus a positive
Bee or s sig .]
Difference between extramedullary & intramedullary lesions of spinal cord
Features
Extramedullary lesion
Intramedullary lesion
Root pain
Prominent an early
Often not prominent/ late
(Dorsal root compression)
manifestation
manifestation
Pyramidal/ UMN sign
Early manifestation
Late manifestation, often spared
(CST damage)
Sacral sensory loss
Early manifestation
Late manifestation
Progression of symptoms
Often rapid
Often slow
Differe e et ee paraplegia i e te sio & paraplegia i fle io
Features
Paraplegia in extension
Paraplegia in flexion
Attitude of lower limb
Extended
Flexed
43
Plantar
Extensor
Extensor but may be accompanied
by withdrawal response
Pathophysiology
Lesion of pyramidal tract Lesion of pyramidal tract as well as
extrapyramidal fibres
Some important etiology of chronic cord compression
1. Vertebral metastasis:
Common primary cancers:
Breast
Bronchogenic
Bowel
Gynaecological
Prostate.
Common site of metastasis: Thoraco-lumber vertebrae.
Symptoms:
Low back pain is a prominent symptom
Symptoms of primary malignancy may be present.
2. Caries spine/ TB spine/ vertebral TB:
It is due to hematogenous spread of bacilli
Symptoms of primary TB may/ may not be present
Causes of cord compression:
Collapse of the vertebra
Compression by paravertebral abscess
Tubercular myelitis
Tubercular vasculitis (of spinal cord blood vessels).
Vertebral tenderness may be present
Local deformity may be present:
Knuckle: prominence of 1 spinous process
Gibbus: prominence of >1 spinous processes.
Investigation of compressive myelopathy
1. Imaging:
a. X Ray spine (to detect vertebral abnormality)
44
b. MRI confirms any cord compression, often tell about the underlying
cause.
2. Investigations to assess the underlying cause:
a. Preliminary investigations:
H , TC, DC, ESR i i fe tio
Na+ K+ Urea creatinine
Li er fu tio tests Alkali e phosphatase le el i etastasis
Seru al iu i etastasis
Chest X Ray.
b. Special investigations:
Nature of it depends on the definitive clinical diagnosis.
Ex: Vertebral metastasis: Histopathology.
Treatment
Supportive
1. Immobilization of the spine (particularly in case of trauma)
2. Absolute bed rest
3. Bed sore prevention (in appropriate cases)
4. Catheterization (if required)
5. Dexamethasone (to reduce spinal cord edema)
6. DVT prophylaxis
7. Exercise and physiotherapy (when safe to start).
Definitive
1. Pharmacological:
a. TB: Anti-tubercular drug
b. Spinal abscess: IV antibiotic.
2. Surgical:
a. Neurosurgical decompression
b. Orthopedic fixation
c. Tumor excision.
3. Radiotherapy:
In case of metastasis.
45
Dx of cord compression
1. Acute cord compression (in spinal shock stage):
a. Spinal shock stage of acute transverse myelitis
No H/O trauma
b. Polio
No sensory disturbance and no
c. GB syndrome
bowel-bladder disturbance
2. Acute cord compression (spasticity has developed):
Acute transverse myelitis
Do a MRI to confirm
3. Chronic cord compression:
Other diseases causing paraparesis/ quadriparesis with spasticity may
mimic chronic cord compression. These include non-compressive
myelopathies:
a. Motor neurone disease
b. Multiple sclerosis
c. Subacute combined degeneration of spinal cord.
- All these diseases have no upper border of sensory loss and bowel-bladder
disturbance is not prominent in these diseases.
46
Non-compressive myelopathy
Acute transverse myelitis
Introduction
It is a sudden onset, rapidly progressive inflammation of the spinal cord, where
the inflammatory process can extend transversely as well as longitudinally for 1-2
segments.
Etiology
1. Most likely, the inflammation is an autoimmune response likely to be
triggered off by different viruses (HSV. Measles etc.)
2. As a complication of old generation rabies vaccine.
Pathophysiology
As the
Rapid
inflammation
Inflammation of
accumulation of
Strangulation of
subsides, usually
spinal cord
inflammatory
the spinal cord
there is complete
exudate
recovery
Symptoms
1. Prodromal symptoms:
Precedes the paralysis and characterized by fever, malaise, bodyache etc.
2. Rapidly progressive weakness:
a. Usually starts with both lower limbs
47
b. Upper limbs are often spared as the inflammation commonly occurs in
the thoracic segment
c. Rapidly progressive sensory impairment with a definite upper level
above which sensations are normal.
3. Prominent sphincteric disturbance:
a. Acute retention of urine
b. Fecal incontinence.
- With proper treatment, symptoms start to improve after few days, leading
to complete recovery, in some patients, a degree of residual weakness may
persist.
Signs
1. Spinal shock stage:
Po er:
To e: Fla id
Deep te do refle DTR : /-
Pla tar: Bilateral e te sor Up goi g:
Wasting/ fasciculation: Absent
Sensory: Impairment/ loss of all sensation with a definite upper level.
2. Stage of paralysis:
Power:
To e: Spasti
DTR: lo us a e prese t
Pla tar:
Sensory: Impaired.
3. Stage of recovery:
Power and sensation gradually start to return
UMN signs and sensory signs gradually disappears.
Investigations
1. MRI of spinal cord
2. Lumber puncture:
a. WBC cou t slightl
b. CSF protei Nor al/ slightl
48
c. NO classical albuminocytological dissociation.
3. Routine investigations:
Hb, TC, DC, CRP, Na+, K+, Urea, Creatinine.
Treatment
1. Supportive:
A. Absolute bed rest
B. Bed sore prevention
C. Catheterization
D. DVT prophylaxis
E. Exercise, physiotherapy and walking aids.
2. Definitive:
Systemic corticosteroid.
Dx of ATM
1. Acute compressive myelopathy in shock stage (H/O trauma is present)
2. GB/ Polio: No H/O sensory loss/ bowel-bladder disturbance
3. Compressive myelopathy (with spastic signs): Shows exactly same feature
4. Acute lesion in spinal cord (non-compressive): Ant. Spinal artery occlusion/
thrombosis.
Motor neuron disease (MND)
Introduction
It is a chronic degenerative disease affecting motor neurons.
Affected motor neurons
Corticospinal fibres
UMN
*Predominantly bulbar
Corticonuclear fibres
Motor
cranial nerve nuclei are
neuron
affected in MND.
Anterior horn cell
LMN
Cranial nerve nuclei*
49
Spectrum of motor neuron diseases
Motor neuron
disease
Combined (UMN +
UMN disease
LMN disease
LMN) type
Spinal (affecting
Spinal (affecting
CSF)
AHC)~
Bulbar (affecting
Bulbar (affecting
CBF)*
BCNN)^
Spinobulbar
Spinobulbar
[CSF: Corticospinal fibres, CBF: Corticobulbar fibres, AHC: Anterior horn cell,
BCNN: Bulbar cranial nerve nuclei]
Examples:
*Pseudobulbar palsy, ^True/ progressive bulbar palsy, ~Spinal muscular atrophy.
Note (incorporate with the picture below in signs and symptoms of AML):
In early stages of MND, CSF are damaged first in the upper limbs, leading to
predominance of UMN signs. When it progresses, it gradually involves AHC,
leading to mixed (UMN + LMN) signs. At the late stages, it progresses to
predominance of LMN signs.
When we try to incorporate lower limbs in this picture, they show predominance
of UMN signs until the late stages, when they also begin to show LMN signs.
Amyotrophic lateral sclerosis (ALS)
Introduction
It is the commonest variety of motor neuron disease characterized by combined
(UMN + LMN) degeneration.
50
Etiopathogenesis
Exact cause is unclear, however there are few hypothesis:
1. Mutation of superoxide dismutase 1 (SOD1) gene leading to accumulation
of abnormal proteins causing cytotoxic cell damage
2. Abnormal accumulation of excitatory neurotransmitter glutamate leading
to cell apoptosis
3. Mutation of VEGF gene also plays some role.
Symptoms
1. Bulbar:
Dysphagia
Dysarthria
Dysphonia
Nasal intonation of voice
Nasal regurgitation of food
Recurrent episodes of chocking/ aspiration leading to aspiration
pneumonia.
Tongue:
Tone: Spastic
Wasting and fasciculation: May be present
Pharyngeal reflex: Lost
Gag reflex: May/ may not be brisk
51
Patient may be emotionally labile, leading to spontaneous spells of
laughing/ crying (due to pseudobulbar palsy).
2. Spine:
Slowly progressive para/quadri-paresis
Wasting: Particularly of small muscles of hand, often very prominent
No sensory disturbances
No sphincteric disturbances.
Signs
1. Upper limb:
Combined (UMN + LMN) signs
Po er:
Tone: Spastic (due to UMN type of damage), but some muscles may
be flaccid (due to LMN type of damage)
Wasting: Some muscles may be wasted
Fasciculation: May/ may not be present
Reflexes: Brisk/ absent in varying combinations.
2. Lower limb:
Usually spastic weakness (due to predominance of UMN signs)
Jerk: Brisk
52
Wasting and fasciculation: Absent
Plantar: Bilateral extensor
As the disease progresses, lower limb will also show superadded
LMN signs.
Functions which will remain unaffected in ALS:
1. Higher function
2. Function of extraocular muscles
3. Sensory function
4. Sphincteric function normal (as nucleus of Onuf is usually spared).
Complications of ALS
1. Respiratory paralysis, leading to type respiratory failure
2. Recurrent episodes of chocking/ aspiration, leading to recurrent episodes
of aspiration pneumonia.
Investigations
1. Nerve conduction velocity (NCV)/ Electromyography (EMG)
2. MRI spine: To rule out any structural compression of spinal cord.
Treatment
Supportive
B. Bed sore prevention
C. Catheterization (if required)
D. Diet:
Maintenance of nutrition may be difficult due to swallowing difficulty, often
patient requires feeding jejunostomy or PEG (Percutaneous endoscopic
gastrostomy) tube.
E. Exercise and physiotherapy
F. Long term ventilatory support.
Definitive
Riluzole (Glutamate synthase inhibitor)
53
Dx of ALS
Patient with quadriparesis:
Cervical compressive myelopathy:
Here (sensory + sphincteric) disturbances are present
Patient with wasting of small muscles of hand:
1. Lower cord compression (C7-C8)
2. Plexopathy
3. Peripheral neuropathy
4. Pa oast s tu or C8-T1 compression).
54
Cerebrovascular accident (CVA)
Part 1: Transient ischemic attack (TIA)
Introduction
TIA is characterized by reversible focal neurodeficit lasting usually <24 hours and
is due to reversible occlusion of cerebral artery.
Risk factors
Thrombosis (artery to artery embolism):
The main cause is cerebral atherosclerosis resulting from:
A. Abdominal obesity
B. BP
C. Cigarette smoking
D. Diabetes/ Dyslipidemia
E. E er ise.
Cardioembolism:
The causes are:
1. Atrial fibrillation
2. Mitral stenosis ? AF
3. Infective endocarditis
4. AMI (Mural thrombus)
5. Paradoxical embolism (Dislodged deep vein thrombosis in a patient of
patent foramen ovale, VSD, ASD etc.)
Clinical features of TIA
Clinically, TIA may be classified into 2 groups, each of which has some distinct
clinical features, which may appear in varying combinations involving the
particular areas affected:
Carotid TIA
Vertebro-basilar TIA
Contralateral hemiplegia (CST)
Diplopia (CN 3,4,6)
Contralateral hemisensory loss (LSTT)
Dysarthria/ Dysphagia (Bulbar nuclei)
Contralateral homonymous
Dys-equilibrium (Cerebellum)
hemianopia (Optic radiation)
55
Aphasia (Speech area)
Drop attack (sudden fall due to
weakness and loss of tone of lower
limb)
Amaurosis fugans (Transient loss of
Vertigo and vomiting
vision when the patient feels as if a
Contralateral hemiparesis (CST)
curtain is coming down in front of the
Contralateral hemisensory loss (LSTT)
eye; cause: retinal artery occlusion)
All the patients of TIA should be assessed for:
1. Atherosclerotic risk stratification
2. Atrial fibrillation
3. Cardiac murmur
4. Carotid bruit.
Investigation
Investigation of TIA
Carotid Doppler
Brain imaging
Risk stratification
ultrasound
CT head
Lipid profile
Fasting/ postprandial
MRI brain
blood sugar
ECG
24 hour ECG in selected
cases
Echocardiogram
Treatment
1. Risk factor modification (write as per ABCDE in risk factor)
2. Pharmacotherapy:
o Antiplatelet (Aspirin + Dipyridamol or Clopidogrel)
o Anticoagulant (Long term Warfarin in case of cardiac embolism)
56
o Antilipidemic drug
o Antihypertensive drug
o Antidiabetic drug.
3. Surgical treatment:
Carotid end-arterectomy
Indication:
>70% stenosis of internal carotid artery.
ABCD2 scoring and its application
This scoring system acts as a future stroke risk predictor in a patient of TIA:
A. Age> 60 years (1)
B. SBP> 140 mm Hg OR DBP> 90 mm Hg (1)
C. Clinical features:
Weakness ? Speech disturbance (2)
Only speech disturbance (1)
D1. Duration:
>1 hour (2)
10-59 min (1)
D2. Diabetic (1).
Scoring
Implication
0-2
Low risk (does not require hospitalization)
3-5
Moderate risk (Hospitalization may be considered)
6-7
High risk (Hospitalization recommended)
PART 2: Stroke
Introduction
Abrupt onset focal neurodeficit lasting for >24 hours due to a vascular event.
Type
1. Ischemic stroke:
a. Thrombotic stroke
b. Embolic stroke
57
2. Hemorrhagic stroke
Most common risk factors
1. Thrombotic stroke: Atherosclerosis
2. Embolic stroke: AF
3. Hemorrhagic stroke: Hypertension
Hemorrhagic stroke
Risk factors:
Arterial hypertension
Aneurysm
Arterio-venous malformation
Side effects of antiplatelet/ anticoagulant therapy
Accidental (head injury).
Locations of hemorrhage:
1. Epidural
2. Subdural
3. Subarachnoid
4. Intracerebral/ intraparenchymal.
58
Mechanism of clinical manifestations:
1. Due to damage to corticospinal and corticonuclear fibres innervating lower
? of 7th cranial nerve nucleus
2. Damage to other structures: depending upon the site of lesion
These manifestations help us to localize the site of lesion
3. Due to underlying etiology/ risk factors
4. Due to raised ICT (resulting from compressive effect of blood/ intracerebral
edema).
Case presentation of CVA
Note that, in long case of CVA, the type and localization of lesion has to be made
roughly, and points have to be presented supporting your estimation.
History and examination should include thorough cerebrovascular risk
assessment.
Clinical features which may help us to get an idea about the underlying type of
stroke are as follows:
Features
Thrombotic stroke
Embolic stroke
Hemorrhagic stroke
Onset and
Sudden. At times,
evaluation
it progresses over
Sudden in onset
few hours
Simultaneously affects all the parts
affecting one part
after another
59
Preceding
H/O a preceding TIA may be present
-
symptoms
Associated
Rare
May be present
symptoms
(due to sudden rise
of ICT)
History
H/O angina/
H/O palpitation/
H/O hypertension
claudication may
syncope may be
(poorly controlled)
be present
present
may be present
Pulse
-
Irregular pulse (if AF is
-
present)
Murmur
-
Murmur of MS may
-
be present
Clinical manifestations and localization of site of lesion
Site of lesion
Clinical features
Cortex
1. Contralateral hemiplegia (CST damage)
2. Contralateral lower facial weakness (CN7 palsy):
Asymmetrical weakness/ monoparesis/ facial
weakness only
3. Contralateral sensory loss
4. Contralateral homonymous hemianopia (Optic
radiation damage)
5. Aphasia:
a. Fluent (Wernicke's
b. Non-fluent (Bro a's)
Usually seen in right sided lesion (as speech
area is usually right sided)
6. Visuospatial neglect (non-dominant parietal lobe
lesion): As the right lobe is dominant in majority of
people, there is unawareness of the left side.
Internal capsule
1. Contralateral hemiplegia
2. Contralateral lower facial weakness.
Midbrain
1. Contralateral hemiplegia
2. Contralateral lower facial weakness
3. Ipsilateral CN3 palsy
4. Ipsilateral CN pals 1+ = We er's s dro e
60
Pons
1. Contralateral hemiplegia
2. Ipsilateral LMN type of CN7 palsy
3. Ipsilateral CN6 palsy
4. Loss of touch sensation from ipsilateral ? of face
(Principal sensory nucleus of CN5 damage)
5. Ipsilateral Hor er's s dro e S
patheti tru k
damage)
6. Loss of pain and temperature sensation from
contralateral ? of body (LSTT damage)
Medulla
Medial medullary
1. Contralateral hemiplegia
syndrome
2. Ipsilateral LMN type of CN12 palsy
Lateral medullary
1. Ipsilateral LMN type of CN 9, 10, 11 palsy
syndrome
2. Loss of pain and temperature sensation from
ipsilateral ? of face (spinal nucleus of CN5 damage)
3. Ipsilateral Hor er's s dro e S
patheti tru k
damage)
4. Loss of pain and temperature sensation from
contralateral ? of body (LSTT damage)
5. Vertigo, vomiting, nystagmus (vestibular nucleus
damage)
Common sites of hyperetnsive hemorrhage:
Site
Clinical features
Basal ganglia
Contralateral hemiplegia ? Contralateral lower facial weakness
Thalamus
Contralateral hemiplegia ? Contralateral lower facial weakness
Contralateral sensory loss
Contralateral homonymous hemianopia
Conjugate movement abnormality
Chronic pain of the affected limbs.
Cerebellum
Nystagmus
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Ataxia
Incoordination of movement
Vertigo/ vomiting.
ICT
Dropping level of consciousness (low GCS score)
Investigation of stroke
1. Imaging
2. Risk factor diagnosis
3. Routine investigation
4. Special investigation
Imaging
1. CT head
2. MRI (in selected cases)
3. Cerebral angiography (in selected cases):
a. MR angiogram
b. CT angiogram
Risk factor diagnosis
1. Blood sugar (Fasting + Post-prandial)
2. Fasting lipid profile
3. ECG
4. Echocardiogram
5. 24 hour ECG monitoring (in selected cases: suspected paroxysmal
arrhythmia)
6. Carotid Doppler ultrasound (in selected cases).
Routine investigation
1. Blood: Hb, TC, DC, ESR
2. Renal function: Na, K, Urea, Creatinine
3. Coagulation profile: BT, CT, PT, aPTT
4. Chest X Ray
62
Special investigation
1. If vasculitis is suspected: Vasculitis screen (for detection of autoantibodies)
2. If hypercoagulable disorder is suspected: Tests to rule out hypercoagulable
disorders
3. Id cerebral venous thrombosis is suspected: MR venogram.
Treatment
1. Immediate treatment
2. Risk factor modification
3. Supportive treatment.
Immediate treatment
Suspected stroke
Airway protection,
breathing and
circulatory support
CT head
Ischemic stroke
Hemorrhagic stroke
If thrombolysis
Neurosurgical
indicated (onset of
referral
symptoms <3 hours)
Thrombolysis
(Alteplase)
63
Risk factor modification
1. Lifestyle modification:
Control of ABCDE.
2. Pharmacotherapy:
Antiplatelet drugs (to prevent thrombus formation):
Aspirin 300 mg for 14 days
Clopidogrel for rest of the life.
Anticoagulant agents (in case of cardio-embolism):
Warfarin
Antihypertensive agents (during acute period):
During acute period of stroke, BP should not be quickly reduced
because rapid decline of BP may jeopardize cerebral autoregulation
and may cause irreversible damage. So, this period is alled ischemic
penumbral zone . During this period, the target BP should be (140-
150) / (80-90).
Long term target BP should be <140/80.
Antidiabetic drugs
Antilipidemic drugs:
Atorvastatin.
Supportive treatment
A. Airway protection (? oropharyngeal suction ? intubation, if required)
Absolute bed rest
B. Breathing support (oxygen ? invasive ventilation)
Bed sore prevention (frequent change of posture/ air or water mattress)
C. Circulatory support (IV fluid, if required)
Catheterization, if required
Cerebroprotective agents (Piracetam/ Citicoline): Have doubtful role
D. Diet:
Oral feeding (if safe)
R le's tu e feedi g
Long term feeding:
Feeding jejunostomy
Percutaneous endoscopic gastrostomy tube
64
Drugs:
Anti-edema measures (IV mannitol followed by oral glycerol)
Anticonvulsant (if there is post stroke seizure)
Antibiotic (if any focal infection is suspected)
E. Exercise (Physiotherapy)
Differential diagnosis of stroke
1. Dx of acute hemiparesis:
a. CVA/TIA
b. Post-seizure he iparesis Todd's paral sis : Te porar a ifestatio
c. Cerebral venous sinus thrombosis
d. Multiple sclerosis.
2. Dx of causes of stroke:
a. Ischemic
b. Hemorrhagic.
Oxford classification of stroke
There are 4 categories in Oxford classification of stroke, which depends upon the
presence of 1 or more of the following parameters:
1. Contralateral hemiplegia/ Contralateral hemisensory loss involving:
Upper limb ? Lower limb ? Lower half of face
2. Contralateral homonymous hemianopia
3. Higher cortex dysfunction: Aphasia/ Visuospatial neglect
4. Cerebellar signs/ brainstem sign/ loss of consciousness/ isolated
homonymous hemianopia
1+2+3: Total anterior circulation stroke (TACS)
Any 2 of (1+2+3): Partial anterior circulation stroke (PACS)
Only 1: Lacunar anterior circulation stroke (LACS)
Any 1 component of 4: Posterior circulation stroke (POCS)
SN: Lacunar infarct
It is a small vessel stroke typically due to occlusion of a deep penetrating branch
of a cerebral artery.
65
Risk factor: Athero-thrombotic occlusion
Clinical features:
1. Contralateral weakness: Upper limb/ lower limb/ lower half of face
2. Contralateral hemisensory loss.
Investigation:
1. Imaging
2. Risk factor stratification.
Treatment:
1. Immediate
2. Risk factor stratification
3. Supportive treatment
Same as general management of stroke.
Autopsy findings:
On autopsy, the affected region of the brain looks like a lake (small area filled with
fluid), therefore the a e la u ar is gi e .
SN: Brainstem signs
1. Contralateral weakness and contralateral hemisensory loss (LSTT damage)
2. Motor cranial nerve palsy
3. Vital center dysfunction:
a. Respiration:
Respiratory depression
Che e Stoke's reathi g (apnea followed by hyperpnoea)
66
b. BP: Erratic
c. Pulse: Erratic
4. Loss of consciousness (Low GCS):
Ocular abnormality:
I. Loss of Doll's e e/ O ulo ephai refle
II. Persistent conjugate deviation towards one side
III. Pupillary abnormality:
o Bilateral dilated non-reactive pupils (in brainstem death)
o Pin point pupil (in pontine hemorrhage).
Subarachnoid hemorrhage (SAH)
Bleeding inside subarachnoid space.
Etiology
1. Rupture of a sa ular a eur s Berr 's a eur s
2. Intraventricular extension of intraparenchymal bleed.
67
Risk factors
A. Arterial hypertension
AV malformation
Anticoagulant/ antiplatelet therapy
Aneurysm rupture
B. Bleeding disorder
Clinical features
1. Headache
o Onset: Sudden
o Site: Often occipital (due to vertebro-basillar artery aneurysm rupture)/
neck pain but may be generalized
o Nature: Usually severe, ofte des ri ed
the patie t as orst
heada he of life
o Some extra manifestations may be present due to meningeal irritation:
a. Neck stiffness
b. Photophobia.
2. Focal neurodeficit:
o Falling level of consciousness (low GCS): Due to increased ICT secondary
to obstructive hydrocephalus
o Hemiparesis/ Aphasia etc. due to cerebral infarction resulting from
severe vasospasm: Leaked blood irritates blood vessels
o Neck rigidity may be present: Leaked blood irritates meninges
o 3rd cranial nerve palsy: Due to an expanding aneurysm of posterior
communicating artery
o Homonymous hemianopia: Due to an aneurysm of anterior cerebral
artery (compression over optic tract).
Investigation
Supportive/ routine:
1. Blood: Hb, TC, DC, CRP/ESR
2. Renal function: Na+ K+ Urea creatinine
3. Coagulation profile: PT, aPTT
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Definitive:
1. CT Head (sensitive to detect hemorrhage): It will show bleeding within
sulcus/ cistern
2. If CT is inconclusive: Do a Lumbar puncture: It will reveal blood stained
CSF, which may be of 2 origins and can be differentiated by following:
o Continuous leak of blood stained CSF: Due to SAH
o Gradually the blood staining gets clear: Due to traumatic tap.
CSF RBC count:
o In case of traumatic tap, there will be a significant drop in RBC
count between sample 1 and sample 4
o In case of SAH, there will be no such drop.
LP should be avoided in case of suspected raised ICT.
Xanthochromia:
Yellowish discoloration of CSF due to presence of bilirubin/ other
pigment.
It usually takes 6-12 hours before Xanthochromia develops. Therefore,
ideally LP should be performed before 12 hours of onset of symptoms.
3. Cerebral angiogram: 4 vessel DSA (Digital subtraction angiography).
Treatment
It has 2 components: supportive and interventional.
Supportive treatment:
A. Absolute bed rest for 48-72 hours (if patient prefers than in a dark room)
Analgesic
B. Bowel: Laxatives (to avoid any straining during defecation)
C. Circulatory support: IV fluid (to maintain adequate hydration)
D. Drugs: Nimodipine (to counteract cerebral vasospasm).
69
Interventional treatment:
1. Neurosurgical: Clipping of the aneurysm
2. Radiological: Coiling
3. Ventriculostomy (if significant hydrocephalus develops).
Complications
1. Obstructive hydrocephalus
2. Vasospasm
3. Rebleeding
4. Cerebral salt wasting syndrome (hyponatremia).
SN: Saccular aneurysm
Outpouching of an arterial wall of cerebral blood vessels usually due to congenital
weakness of the wall.
Site:
Can occur anywhere in the Circle of Willis but common sites are:
1. Distal of ICA
2. Bifurcation of MCA
3. Top of Basillar artery.
70
Associations:
1. Coarctation of aorta
2. Polycystic kidney disease
Description:
A saccular aneurysm has two parts: a neck and an apex.
Rupture usually occurs at the apex.
Size of the aneurysm correlates poorly with risk of rupture.
Clinical features:
CN3 palsy
Before rupture
Homonymous
Clinical features of
hemianopia
Saccular aneurysm
SAH and its
After rupture
complications
71
Investigations:
1. CT
2. LP
3. DSA
Treatment:
It has 2 components: supportive and interventional (same as SAH).
72
Neuropathies
They are of 2 types:
1. Acute (Ex: Guillain-Barr? Syndrome)
2. Chronic (Ex: Diabetes)
Guillain-Barr? Syndrome (GB syndrome)/ Acute inflammatory demyelinating
polyneuropathy (AIDP)
Introduction:
It is a disease characterized by rapidly progressing inflammatory demyelination of
multiple, predominantly motor peripheral nerves (roots/ radicles may also be
damaged).
Etiopathogenesis:
Most likely inflammatory reaction is triggered off by a recent infection: URTI/ GI
infection.
Clinical features:
Preceding history of an URTI (fever, sore throat, cough) or GI infection
(diarrhea, abdominal pain etc.) may be present.
Symptoms:
1. Rapidly progressive weakness: Usually starts with lower limb, then
ascends.
2. Often proximal weakness is more than weakness in distal parts.
3. Sensory symptoms: Patient often complains of a deep seated severe
pain in the limbs.
4. Respiratory distress and drowsiness due to type 2 respiratory failure
(hypoxemia with hypercapnia).
5. Sphincteric disturbance is very rare.
Signs:
Motor:
1. Paraparesis/ Quadriparesis: Flaccid.
2. Initially proximal weakness> distal weakness.
3. Symmetrical weakness usually present.
4. Deep te do refle es/ Jerk: / -
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5. Planter: Lost/ unresponsive/ flexion (But never extensor)
Wasting and fasciculation is absent in most of the cases due to rapidity
of the progression of disease.
Sensory:
No objective sensory signs.
Cranial nerves:
Bilateral 7th cranial nerve LMN type of palsy may be present.
Autonomic:
1. Tachy/brady-arrhythmia: may be present.
2. Hyper/hypo-tension: may be present.
Paralysis of respiratory muscles:
Mo itor Si gle reath: Nu
er ou ti g test .
1. Respiratory distress
2. Type 2 respiratory failure (hypoxemia with hypercapnia)
3. Tachypnea
4. Low GCS (Glasgow coma score): Due to drowsiness resulting from
retention of CO2.
Investigation
1. NCV:
Confirms the diagnosis. In NCV, two types of lesion are seen:
a. Demyelinating type
b. Axonal damage type (bad recovery).
2. CSF/ Lumber puncture:
a. Protein: Markedly increased
b. WBC: Mildly increased
- Therefore, al u i o ytologi al disso iatio is present.
c. Bedside spirometry:
FVC
3. Special test: Detection of anti GM1 antibody.
Treatment
1. Supportive treatment:
Bed rest
Bed sore prevention
74
Catheterization
DVT prophylaxis
Exercise and physiotherapy.
2. Definitive treatment:
IV IgG for 5 days
Plasmapheresis.
Differential diagnoses
1. Polio
2. Shock stage of acute transverse myelitis
3. Shock stage of acute compressive myelopathy.
Miller Fisher Syndrome
It is an atypical variant of GB.
Clinical features:
All clinical features + ophthalmoplegia + ataxia.
Investigation:
1. NCV
2. LP
3. Detection of Anti Gq1b antibody.
Treatment:
As in GB syndrome.
Polio
Introduction
It is a disease characterized by rapid degeneration of anterior horn cell (AHC).
Causative agent
Poliovirus type 1, 2, 3.
Clinical features
There are 4 types of outcome in a polio patient:
75
a. Asymptomatic
b. Abortive
c. Non-paralytic polio (neck stiffness + meningeal signs)
d. Paralytic polio.
Course of paralytic polio (in comparison to GB)
1. Prodromal illness:
Fever
GB: Usually symptoms of a
Malaise
preceding URTI/ GI
Neck pain
infection is present
Neck stiffness
Constipation.
2. Asymptomatic latent period: Absent in polio
GB: May be present
3. Weakness:
Rapidly progressive
Para/quadri-paresis
Proximal weakness is more prominent throughout the course of illness
Bilaterally asymmetrical involvement GB: Usually symmetrical
To e of affe ted li
:
Refle : /-
GB: Same features are seen
Plantar: Unresponsive/ flexor
Fasciculation: Usually present
GB: Absent
Wasting: Prominent
Recovery: Very poor
GB: Often good recovery
Signs of LMN type bulbar palsy
GB: Facial palsy more common
Autonomic dysfunction: May occur
Respiratory paralysis with failure: May occur
(Sensory + Sphincteric) disturbance: Absent.
Investigation
Detection of virus in stool sample
Treatment
Established case: Supportive
Primary prevention: Vaccination.
76
Peripheral neuropathy
Introduction
It is a condition characterized by:
Mono-neuropathy or
Poly-neuropathy.
Causes
A
AIDP*
Alcoholic neuropathy
B
Vitamin B deficiency (B1, B6, B12)
Vitamin B6 deficiency is commonly seen in INH therapy.
C
CIDP^
Chronic renal failure
Carcinomatous neuropathy (Paraneoplastic syndromes)
Connective tissue disease (vasculitis)
D
Diabetes
Drug induced:
Amiodarone
Anticancer drugs
Anti-retroviral drugs.
E
Endocrinopathy (Hypothyroidism)
Exogenous toxins (Pb, As etc.)
F
Familial: Hereditary sensory motor neuropathy/ Charcot
Merry Tooth disease
G
GB*
H
HIV
Ha se 's disease (Leprosy)
[* Both are same, ^IDP: Inflammatory demyelinating poly-neuropathy]
Clinical features
1. Due to the underlying disease
2. Due to neuropathy itself:
Motor, sensory and autonomic manifestations may occur in an isolated
manner/ in varying combinations.
77
A. Motor:
Weakness:
Uni/bi-lateral
Asymmetrical
Patchy in distribution: Involving only that part of the limb
which is innervated by the affected nerve
Often starts with distal weakness.
LMN signs present in the affected part of the limbs:
Flaccidity
Refle : /-
Wasting: Present
Fasciculation: Absent
Plantar: Unresponsive/ flexor.
B. Sensory:
Sensory impairment:
Paresthesia: Tingling/ pins and needle sensation
In some cases (ex. diabetes): severe burning pain
Unilateral/ bilateral (asymmetrical).
Sensory loss:
Patchy in distribution: From the dermatome of the affected
nerve
Distal part of limb is affected first (Glav and stocking
distribution).
Neuropathic ulcer: Often present.
C. Autonomic:
Gastro-intestinal:
Constipation due to hypomotility
Genito-urinary:
Sphincteric disturbance
Impotence
Postural hypotension.
78
Investigations
Confirm neuropathy
Diagnosis of underlying disease
NCV
Hb, TC, DC, ESR/CRP
Na+ K+ Urea creatinine
Fasting and postprandial blood glucose
Serum B12
Serum TSH
Liver function test
Vasculitis screen
Management
Treatment of
Supportive treatment
underlying cause
for neuropathy
Motor
Sensory
Autonomic
Prevent/
Drugs for
Symptomatic
Physiotherapy
Foot/ limb care
Avoid trauma
urgently treat
neuropathic pain
treatment
any infection
Mobility aid
Amitryptiline
Protective
Gabapentin
splints
Pregabalin
79
Myasthenia Gravis
Introduction
It is an autoimmune disease characterized by fatigable weakness and is usually
due to autoantibody mediated blocking of post-synaptic acetylcholine receptor.
Clinical association
1. Thymoma
2. Grave's disease
3. Addiso 's disease
4. Pernicious anemia.
Pathophysiology
1. There is auto-antibody mediated blocking of Ach receptor in the post-
synaptic membrane resulting in too few functional Ach receptor.
2. Simultaneously, there is exaggeration of physiological rundown of Ach in
the pre-synaptic membrane.
- Both of these are responsible for impaired N-M transmission
which gets further compromised on repeated contraction of
muscle leading to fatigable weakness.
Clinical features
The type of weakness is fatigable; so signs and symptoms become more
prominent as the muscle continues to work continuously.
1. Ocular features:
a. Drooping
b. Extra-ocular muscle fatigue: Diplopia
c. Extra-ocular muscle weakness: External ophthalmoplegia (distribution of
weakness does not follow any specific cranial nerve pattern)
d. Ptosis: Usually fatigable
e. No pupillary abnormality.
2. Facial weakness
3. Weakness of bulbar muscles
4. Limbs:
a. Fatigable weakness
80
b. Tone, reflex, jerk, plantar: Usually normal
c. Wasting, fasciculation: Absent.
5. Respiratory paralysis and type 2 respiratory failure may occur
- Therefore, the patient should be regularly monitored for the following
signs:
Unexplained shortness of breath (SOB)
Tachypnea
Signs of carbon di oxide retention
Single number breath counting test
Bedside spirometry.
Investigation
1. Edrophonium challenge test/ Tensilon test:
Dramatic improvement of weakness
2. NCV (tests the nerve)/EMG (tests the muscle):
Detrimental response on repetitive stimulation of muscle
3. Detection of Ach-R antibody
4. Antibody against muscle tyrosine kinase: Predictor of respiratory paralysis
5. CT chest: To look for thymoma.
Treatment
1. Stable stage:
a. Neostigmine/ Pyridostigmine (anti-cholinesterase)
b. Corticosteroid
c. Immunomodulators (Micophenolate mofetil)
2. Myasthenia crisis (acute respiratory failure/ acute limb weakness):
a. IV IgG/ Plasmapheresis
b. Ventilatory support
c. Thymectomy.
SN: Lambert Eaten Myasthenia Syndrome
Introduction:
It is a Paraneoplastic syndrome of small cell lung carcinoma. It is due to anti Ca++
channel antibodies in pre-synaptic membranes.
81
Clinical features:
Same as myasthenia gravis except:
1. Weakness improves with repeated muscle contraction
2. Areflexia occurs
3. Autonomic disturbances occur.
Diagnosis:
EMG: Incremental response on repeated stimulation
Treatment:
Di-amino-pyridine
Note:
It may manifest even before lung CA appears radiologically. So a patient with
LEMS must be periodically screened by X-Ray to detect any obvious mass on the
earliest opportunity.
82
Miscellaneous topics
Subacute combined degeneration of spinal cord
Introduction:
It is one of the neurological complications of vitamin B12 deficiency.
Clinical features:
1. Pyramidal tract damage:
Spastic weakness (UMN type).
2. Dorsal column damage:
Loss of joint, position and vibration sense (deep sensation).
Ro berg's sig (Sowing to one side on eye closure): Positive.
Stamping gait: Patient does not know where his foot is and so, while
walking raises the foot high up and brings down on the ground
forcefully like stamping.
3. Peripheral neuropathy:
Sensory: Impaired superficial sensation, patchy in distribution.
Motor: LMN signs.
4. Cognitive impairment/ dementia.
Investigation:
1. Nerve conduction velocity (NCV) study
2. MRI: To rule out any structural damage.
3. Blood: H , MCV, a o tes p ese t; pa
tope ia a e p ese t.
4. Estimation of serum vitamin B12 ? Folic acid.
Treatment:
Parenteral supplementation of vitamin B12 + Oral folic acid.
F ed i k s Ata ia
Inheritance: Autosomal recessive (AR).
It shows t i u leotide epeat se ue e : a ifestatio s o u ea lie i
successive generations.
83
Clinical features:
1. Pyramidal tract damage:
Spastic weakness (UMN type).
2. Spino-cerebellar fibre damage:
Ataxia
Nystagmus
Past pointing (pointing beyond the finger in the finger-nose test)
Dysdiadochokinesia (impaired ability to perform rapid, alternating
movements).
3. Peripheral neuropathy:
Sensory: Sensory loss
Motor: LMN type of weakness.
4. Optic atrophy
5. Cardiomyopathy
6. Skeletal:
Pes cavus
Pes equinovarus.
Investigation:
1. NCV
2. MRI
3. Genetic studies.
Treatment:
1. Supportive
2. Genetic counselling.
Tabes Dorsalis
Introduction:
It is a neurological complication of syphilis.
Clinical features:
D
Dorsal column lesion: Loss of deep sensation
O
Ocular manifestation: Argyll Robertson pupil*
84
R
Root damage: Severe electric shock like pain sensation along the
distribution of affected root
S
Stamping gait
A
Autonomic disturbance: Acute abdominal pain (Tabetic crisis)
L
Loss of sphincteric control
I
Intact power of muscles (Pyramidal tract not affected)
S
Skeletal deformity: Charcot joint~
* Argyll Robertson pupils are bilateral small pupils that reduce in size when the
patient focuses on a near object, but do not constrict when exposed to bright
light. It is a highly specific sign of neurosyphilis.
~ Charcot joint is a progressive degenerative/ destructive joint disorder in patients
with abnormal pain sensation and proprioception.
Investigation:
1. VDRL test
2. TPHA test.
Treatment:
Benzyl penicillin (IM)/ 3rd generation cephalosporins.
Multiple sclerosis (MS)
Introduction:
It is an autoimmune inflammatory demyelinating disease of the nervous system.
Etiopathogenesis:
In genetically predisposed individuals, an autoimmune response triggers off the
demyelinating process. Environmental factors probably play some role.
Clinical course:
Depending on the clinical course, MS is classified into 3 types:
1. Relapsing-remitting type
2. Primary progressive type
3. Secondary progressive type.
85
Clinical features:
1. Higher function:
Cognitive dysfunction
Depression.
2. Cranial nerves:
Optic neuritis
Trigeminal neuralgia
Internuclear ophthalmoplegia (On attempted lateral gaze, there is a
failure of adduction and nystagmus of the abducting eye, cause:
damage of median longitudinal fasciculus)
3. Motor:
Weakness:
May affect one/ multiple limbs. There is marked spasticity with UMN
signs.
4. Sensory:
Patient may complain of numbness, tingling, paresthesia of different
parts of body.
Sensory impairment
86
Lhermitte's sign: Electric shock like sensation passes down the spine
on attempted neck flexion.
5. Cerebellum:
Cerebellar signs may be present.
6. Sphincter:
Sphincteric disturbances are common (retention and incontinence).
7. Trophic changes:
As many of these patients are completely bed ridden, they may develop
bed sore, trophic ulcer etc.
Investigation:
1. MRI of brain and spine:
Visualizes the demyelinating lesion (Plaque).
2. Lumber puncture:
CSF shows markedly elevated IgG (Oligoclonal band of IgG).
Treatment:
1. Supportive:
a. Bed sore prevention
b. Catheterization
c. Exercise and physiotherapy
d. Daclofen: For spasticity.
e. Rehabilitation
f. Mobility aid.
2. Drugs:
a. Corticosteroid:
In acute flare: IV Methylprednisolone
Chronic phase: Oral corticosteroid.
b. Immunomodulators:
Glatiramer acetate
Natalizumab
IV IgG/ Plasmapheresis may be effective during acute relapse.
87
Parkinsonism
Introduction
It is a disease of extrapyramidal tract characterized by imbalance between
dopamine and acetylcholine, leading to the triad of:
Bradykinesia
Rigidity and
Tremor.
Etiology:
Parkisonism
Primary
Secondary
Post-encephalitic
Atherosclerotic
Wilson's disease
Drug induced
Exogenous toxins
parkinsonism
Old generation
Manganese
antipsychotic
Metoclopramide
MPTP
Reserpine
Pathogenesis:
Initiation:
1. Genetic mutation: Parkin and synuclein genes are affected
88
2. Atherosclerosis
3. Toxins.
- I
ala e etwee dopa i e a d a etyl- holi e i igrastriatal
pathway leads to clinical manifestations.
Clinical manifestations
1. Higher functions:
Cognitive dysfunction
Depression
Gambling tendency.
2. Movements:
a. Voluntary:
Slow to initiate and carry out any activity (bradykinesia)
Finer movements (which requires precision) are impaired
Ex: Handwriting becomes progressively illegible and small in size
(micrographia)
Activities requiring postural control get impaired: there is a tendency
to fall.
b. Autonomic functions:
Impaired/ lost.
Ex: Loss of spontaneous arm swinging during walking.
c. Involuntary movement: Tremor:
Rest tremor:
Rest tremor in the hand is often described as pil rolli g / dru
eati g , the tremor is usually marked in the wrist and fingers, which
display motion made in the act of rolling a pill.
Rest tremor often also affects ankle and may be seen in the tongue
and lower jaw.
Aggravates when the patient is emotionally upset/ excited
Decreases in relaxed state/ sleep/ when the affected part is thrown
into action.
3. Power:
Normal, provided that sufficient time is given to build it up.
89
4. Tone:
Hypertonia/ rigidity
Types:
a. Lead pipe rigidity (rigidity> tremor)
b. Cogwheel rigidity (tremor> rigidity).
Usually the flexors are more hypertonic than the opposite group: this
is responsible for the typical attitude/ posture of the patient
Rigidity of pharyngeal muscle:
a. Swallowing difficulty
b. Dribbling of saliva.
Rigidity of laryngeal muscle:
Typically monotonous, slow voice without any modulation.
5. Wasting: Absent
6. Reflex: Usually normal
7. Sensory function: Usually normal
8. Sphincter function: Usually normal
9. Gait:
Difficult to initiate
Slow velocity: short shuffling gait
Impaired/ lost arm swing in time of walking
Tendency to fall and at times, to prevent this fall, patient tries to run
towards the desti atio ; leadi g to Festinating gait
Difficulty to stop
Difficulty to turn around (the whole body turn around like a statue).
10. Facies:
Due to rigidity and bradykinesia of facial muscles, face becomes
expressionless with infrequent blinking and staring look, often called
Parkinsonian mask facies .
Investigation
Clinical diagnosis, however a CT/ MRI of brain is often carried out to rule out any
structural lesion.
90
Treatment
L-DOPA+ CARBI-DOPA
Dopamine releasing agent
(Amantadine)
Dopaminergic agonist
Drugs
(Ropinirole/ Pramipexol)
MAO-B inhibitor (Seleziline)
Dopamine metabolism
inhibitor
COMY inhibitor
(Entacapone/ Tolcapone)
Central anticholinergic
(Trihexyphenydyl,
Benztropine): Usually used
for tremor
Deep brain stimulation
Treatment of a parkinsonian
Surgical
Thalamotomy
patient
Pallidotomy
Physiotherapy
Supportive treatment
Walking aids
Gene therapy
Speech therapy
91
Differential diagnosis
1. Diseases causing tremor:
a. Parkinsonism
b. Cerebellar disorders
c. Benign familial tremor.
2. Parkinsonism plus syndrome:
a. Progressive supranuclear palsy (PSP)
b. Multisystem atrophy/ Shy-Drager syndrome
c. Olivo-ponto-cerebellar degeneration.
3. Causes of parkinsonism (See in etiology).
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Conus medullaris and Cauda equina lesions
Points
Conus medullaris lesion
Cauda equina lesion
Definition
Conus medullaris is the terminal
Cauda equina is the lower spinal
tapering end of spinal cord
nerves containing lower lumber,
containing lower sacral and single
sacral and coccygeal nerves: all
coccygeal segment.
of which descend towards their
exit through intervertebral
foramina.
Graphical
representation
Clinical features
Lower limb
Absent
Present, usually variable and
weakness
asymmetrical
Lower limb jerk
Normal
Asymmetrical hypo-reflexia/ a-
reflexia
Sensory loss
From perianal and perineal region
Asymmetrical sensory loss in
(Saddle back anesthesia)
both lower limbs
Sphincteric
Very prominent
Not common
disturbance
Etiology
Lumbosacral vertebral diseases
Surgery
Often considered
Not commonly considered
Investigation
MRI spine
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Myopathy
Intrinsic diseases of muscles characterized by muscle weakness ? wasting.
Causes/ types
Myopathy
Inherited
Acquired
Duchenne's
muscular dystrophy
Drug induced
Endocrinopathy
Becher's muscular
Long term
Cushing's
dystrophy
corticosteroid
syndrome
Myotonic
dystrophy
Statins*
Thyrotoxicosis
Alcohol
Acromegaly
*Statins are more liable to cause myositis.
Symptoms and signs
1. Weakness: Proximal weakness
2. In some cases, there may be severe muscle pain due to myositis
3. Muscle wasting: Often present
4. Fasciculation: Absent
5. Tone/ jerks/ plantar: Usually normal.
Investigation
1. Serum CPK: May be elevated if there is myositis
2. EMG: Confirms the diagnosis
3. In selected cases, muscle biopsy may be required.
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Treatment
1. Treat the underlying cause
2. Stop the offending drug
3. Rehabilitation.
Myotonic dystrophy
Clinical features
Organ/ area
Defect
Face
Frontal boldness
Eye
Bilateral ptosis
Premature cataract
Hollowing of the face (due to wasting of facial muscles)
Thyroid
Goitre
Heart
Conduction disturbance
Breast
Gynecomastia
Limbs
Myotonia (inability to relax muscles after sustained
voluntary contraction, i.e. handshake)
Muscles
May be wasted, but no gross weakness
Testes
Testicular atrophy
Systemic
Diabetes
Investigation
Genetic testing
Treatment
Genetic counseling and support.
Often responds to:
Phenytoin
Procainamide
Quinine.
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Du he e's us ular dystrophy
General description:
X linked recessive myopathy
Usually occurs in male.
Clinical features:
1. Weakness: Involves proximal muscles; so, more prominent in shoulder and
pelvic girdle muscles
2. Wasting: Affected muscles may get wasted
3. Pseudo-hypertrophy: Particularly affecting Gastrocnemius muscle due to
abnormal accumulation of fibro-fatty tissue
4. Wadling gait: During walking, body slightly bend backwards and kid walks
with wide based gait
5. Gower's sign: If baby is asked to stand up from a lying down position, he
first rolls on to one side then sits up supporting his elbow on the ground
and then gradually stands up by climbing up his knees
6. Intellectual impairment
7. Cardiomyopathy.
Investigation:
Ge eti testi g: Mutatio of Dystrophi ge e.
Treatment:
Supportive. Usually these babies become completely bedbound by the age of 10-
12 years.
Be ker's us ular dystrophy
Cli i al feature like Du he e's us ular dystrophy ut:
May remain mobile upto the age of 15 years
Intellectual impairment more common
Cardiomyopathy less common.
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Infective diseases
Meningitis
Inflammation of meninges.
Causes
1. Pyogenic organism: N.meningitidis, Pneumococcus, Staph.aureus (post
neuro-surgical patients), Listeria
2. Tubercular
3. Viral: HSV, Mumps
4. Fungal (in HIV patients): Cryptococcus, Histoplasma
5. No cause is found in 50% of cases.
Pyogenic meningitis
Clinical features
1. Constitutional symptoms:
Acute febrile illness
Weakness, malaise, loss of appetite.
2. CNS symptoms:
Headache: Severe, usually gradual in onset and progressively
increasing in nature
Neck pain ? neck stiffness
Photophobia: The patient often prefers to lie with eyes closed/ in a
dark room
3. Features of increased ICT:
A. Altered consciousness
B. Behavioral abnormality
C. Confusion, coma, convulsion
D. Delirium
Headache increases and vomiting may occur.
4. Cranial nerve palsy:
CN 3, 4 and 6 palsy may occur.
5. Cutaneous manifestations:
Meningococcemia: Meningococcal rash:
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Typically starts as purpuric/ pin head spots which may enlarge. These
rashes hara teristi ally do t lea h o pressure Glass test due to small
vessel vasculitis/ leakage.
Signs
1. Temperature: High
2. GCS: May be low
3. Meningeal signs:
Neck stiffness/ neck rigidity
Kernig sign
Brudzinski sign
4. Signs due to underlying focus of infection (ENT areas/ danger area of face).
Investigation
1. Blood: Hb, TC, DC, ESR/ CRP
2. Renal function: Na+ K+ Urea Creatinine
3. Blood culture and sensitivity
4. CT head: To rule out any radiological evidence of raised ICT (which is a
relative contraindication of lumber puncture)
5. Lumber puncture:
Description of CSF sample:
I. Physical appearance: May be turbid
II. Biochemistry:
Glucose:
Normal: 40-85 mg/dL
In pyoge i e i gitis:
Normally
>0.6
In pyogenic meningitis, this ratio often goes <0.4
Note:
Paired CSF and random blood glucose is checked to rule out
masking of CSF hypoglycemia due to hyperglycemic condition. (If
a person is diabetic, his blood glucose level will be high and
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consequently, his CSF glucose level will also be high. Even in the
presence of pyogenic meningitis, his CSF glucose will be within the
normal range. To avoid this fallacy, the ratio is taken.)
Protein:
Normal: 15-45 mg/dL
I pyoge i e i gitis:
Cytology:
Normal WBC: 0-5/L
In pyogenic meningitis: Leukocytosis is seen, which is neutrophilic
i ature. Ofte WBC le el il go a o e >
/L.
Microbiological:
Gram stain + culture sensitivity.
Treatment
Supportive treatment:
A. Absolute bed rest
B. Bed sore prevention
C. Catheterization
Circulation by IV fluid
D. Drugs (supportive):
Antipyretic
Antiemetic
Anticonvulsant
Anti-edema (mannitol).
DVT prophylaxis
Diet.
Definitive treatment:
Empirical antibiotic (IV Ceftriaxone): Usually given for 10-14 days
If Staph. infection is suspected, then add Vancomycin.
99
Complications
Mnemonic
Complications
S
Septicemia
Subdural empyema
Seizure
A
Abscess of brain
Acute adrenocortical failure/ Waterhouse Friderichsen syndrome (due to
adrenal insufficiency resulting from meningococcal vasculitis: patient
suddenly goes into shock)
H
Hydrocephalus
Hemiparesis (stroke due to cerebral vasculitis)
Tubercular meningitis
Clinical features
1. Constitutional symptoms:
Gradual in onset, often may persist for few days to weeks before meningitis
symptoms appear:
Low grade fever
Weight loss
Loss of appetite
Low grade headache.
2. CNS symptoms:
Headache: May increase
Vomiting
Photophobia.
3. Symptoms of raised ICT:
ABCD as previous one
4. Symptoms of pulmonary TB may be present.
Signs
1. Temperature: Raised
2. GCS: May be low
3. Meningeal signs: Neck rigidity may be present
4. Cranial nerve palsy: CN 3, 4, 6 palsy (most common: CN6) may be present
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5. Fundoscopy: Choroid tubercles may be present
6. Signs of active pulmonary TB may be present.
Investigations
1. Blood: Hb, TC, DC, CRP/ ESR
2. Renal function: Na+ K+ Urea Creatinine
3. Blood culture
4. Sputum AFB and mycobacterial culture (if active pulmonary TB is
suspected)
5. Chest X Ray
6. CT head:
To rule out raised ICT
Tuberculoma may be present.
7. Lumbar puncture (LP):
I. Physical appearance:
Co e oagulu ay e prese t
II. Biochemistry:
Protei :
Glu ose:
= 0.4-0.6
III. Cytology:
WBC ou t: Usually 5 -
/L
Predominant cells: Lymphocyte
IV. Microbiology:
AFB: May be present
Mycobacterial culture (BACTEC MGIT method): Often positive
Rapid detection method:
X-PERT TB (Nucleic acid amplification)/ RIF (Rifampicin assay):
Promoted by WHO
Detect M.tuberculosis
Can detect rifampicin resistance which is a sensitive
predictor of MDR-TB.
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Treatment
Definitive treatment:
Anti-tubercular drug:
Duration: HREZ for 2 months and HR for at least 7-10 months
Dosage: H (5 mg/kg), R (10 mg/kg), E (15 mg/kg), Z (25 mg/kg)
Vitamin B6 supplementation with H
Corticosteroid:
Duration: 4-6 weeks
Rationale: To prevent tubercular arachnoiditis and meningeal thickening so
that chance of obstructive hydrocephalus and permanent neurological
deficit is minimized.
Complications
1. Obstructive hydrocephalus
2. Stroke/ hemiparesis (resulting from tubercular arteritis).
Brain abscess
A localized area of suppurative infection within the brain.
Risk factor
Although infection may spread to brain from any primary focus of infection in the
body, the common risk factors are:
Ear infection
Dangerous area of face
Scalp laceration/ skull injury
Post neurosurgical patients.
Common organism
Streptococcus/ staphylococcus/ anaerobes.
102
Clinical features
1. Systemic: Fever, malaise, weight loss
2. Features due to increased ICT
3. Primary focus of infection often present.
Investigation
CE-CT of brain
Routine: Hb, TC, DC, CRP, Blood culture
Treatment
Empirical antibiotic:
Ceftriaxone IV + Metronidazole
(+ Vancomycin if Staph. infection suspected)
Often IV antibiotics may be continued for 4-6 weeks after which they are
changed to appropriate oral forms.
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Brain tumor
Classification
Glioma
Astrocytoma
Primary
Ependymoma
Glioblastoma multiforme
Brain tumor
Breast CA
Secondary (metastasis)
Bronchogenic CA
Lymphoma
Mechanisms of clinical manifestations
1. Raised ICT
2. Localizing symptoms and signs (focal manifestations)
3. False localizing symptoms
4. Herniation syndrome.
Clinical manifestations
Manifestations due to raised ICT
A. Altered consciousness
B. Behavioral disturbances
C. Confusion, coma, convulsion
D. Delirium
E. Etiology: Features due to underlying etiology
F. Focal neurodeficit
Fundoscopy: Papilledema
G. GI manifestations: Nausea, vomiting
GCS may be low
H. Headache
Herniation syndrome.
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Localizing symptoms and signs (focal manifestations)
Area involved
Signs
Frontal lobe
Contralateral pyramidal signs
(Motor area)
Focal seizures (due to seizures) with motor manifestations
Aphasia Broca's
Anosmia (CN1)
Behavior and personality disturbances.
Parietal lobe
Contralateral hemisensory loss
(Sensory area)
Astereognosis (inability to recognize an object by its size,
shape and texture)
Sensory inattention/ neglect (visual/ spatial)
Contralateral homonymous hemianopia (inf.quadrantic).
Temporal lobe
Focal seizure with somatosensory manifestations
Psychiatric manifestations
Automatism
Aphasia (Wer icke's)
Contralateral homonymous hemianopia (sup.quadrantic).
Occipital lobe
Contralateral homonymous hemianopia
Cortical blindness (in case of bilateral compression).
False localizing symptoms
A group of clinical manifestations occur in patients with raised ICT (particularly
brain tumor) which gives an erroneous impression about the site of the lesion:
CN6 palsy (as it has the longest intracranial course)
CN3 palsy
Bilateral extensor plantar (resulting from brainstem compression)
Ipsilateral upgoing plantar (resulting from compression of cerebral
peduncle against tentorium).
Herniation syndrome
If there is raised ICT, a part of the brain sometimes escape through a foramen
towards a low pressure compartment. This commonly occurs with cerebellum,
105
which herniates through foramen magnum; leading to brainstem (particularly
medullary) compression- causing:
1. Respiratory arrest
2. Circulatory collapse
3. Deep coma.
Investigation
1. CE-CT Brain
2. If CT is abnormal: MRI brain.
Treatment
Supportive treatment:
1. Propped up positioning
2. Anti-edema drugs:
IV mannitol (for 3-5 days) followed by oral glycerol
Steroid
3. Analgesic
4. Antiemetic
5. Anticonvulsant (if seizure occurs)
6. Surgical:
Ventriculo-peritoneal shunt (in case of obstructive hydrocephalus)
Craniotomy flap (in a stroke patient with severe edema).
7. Elective ventilation with permissive hyperventilation (hypocapnia will
induce cerebral vasoconstriction and therefore ICT will be reduced).
Definitive treatment:
1. Surgical excision of brain tumor
2. Radiotherapy
3. Chemotherapy.
106
Pseudotumor cerebri/ Benign intracranial hypertension (BIH)
Introduction
It is a condition characterized by raised ICT where the underlying cause is
innocuous.
Causes
1. Idiopathic (common in young obese females)
2. Drugs:
a. Long term vitamin A derivatives (Ex: Retinoid)
b. Tetracycline
c. OCP.
Clinical features
Often patients are obese
H/O offending drug intake
Signs and symptoms of raised ICT (ABCDEFGH as describe above)
Investigations
1. CE-CT Brain:
Findings:
Signs of raised ICT
No structural lesion
Ventricular system not dilated.
2. LP (do very cautiously):
High CSF pressure; other findings are normal.
Treatment
1. Encourage to lose weight
2. Stop offending drugs
3. Acetazolamide tablet (Carbonic anhydrase inhibitor)
4. Therapeutic lumbar puncture.
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Seizure
Introduction
Definition of seizure:
Temporary cerebral dysfunction due to abnormal paroxysmal neuronal discharge.
Definition of epilepsy:
It is a clinical syndrome characterized by recurrent unprovoked seizures.
Causes of seizure
A. Alcohol (intoxication/ withdrawal)
Accident (Post traumatic seizures)
B. Biochemical abnormality:
Hypoglycemia/ hyperglycemia
Hyponatremia/ hypernatremia
Hypercalcemia
Metabolic encephalopathy (uremic/ hepatic/CO2 narcosis)
Hypoxic/ ischemic encephalopathy.
C. Cerebral causes:
Vascular causes:
Ischemic stroke
Hemorrhagic stroke
Intracranial space occupying lesions (IC-SOL)
Infections:
Meningitis
Encephalitis
Tuberculoma
Neurocysticercosis
Brain abscess.
D. Drugs (side effect/ intoxication):
Fluoroquinolone
Carbapenem.
E. Exogenous toxins (Ex: Cocaine).
I. Idiopathic.
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Types of seizure
Seizure
Focal/ partial (abnormal electrical
Generalized (abnormal electrical
activity arises from a discrete region
activity arises from a diffuse area of
of one cerebral sphere)
brain)
Simple partial (without loss/
Absence (Petit Mal)
impairment of consciousness)
Complex partial (with loss/
Atonic
impairment of consciousness)
Focal seizure with secondary
Myotonic
generalization
Tonic-clonic (GTCS/ Grand Mal)
Clinical features
Focal seizure:
Clinical features of this type of seizure depends on the location of focus of
abnormal electrical activity.
Simple partial seizure
1. Motor:
Twitching/ jerky movement of a part of the body
This may spread to other parts of boy (Jacksonian movement).
2. Somatosensory:
Paresthesia (tingling/ pins and needle sensation)
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This can spread to other parts of the body.
3. Special sensory manifestations:
Visual: Flashes of light
Auditory: Buzzing noise
Olfactory: Odd smell
Gustatory: Odd taste.
4. Autonomic:
Epigastric fullness
Palpitation
Flushing/ sweating
Psychiatric manifestations:
o Illusion
o Hallucination
o Deja-vu.
The above manifestations usually occur for few seconds to minutes but
there is no impairment of consciousness.
Complex partial seizure
1. Aura:
Motor/ somatosensory/ special sensory/ autonomic/ psychiatric
manifestations occur before loss of consciousness.
However, these manifestations may accompany/ follow the loss of
consciousness.
2. Loss of consciousness:
Typically lasts for few seconds to minutes, when the patient develops
impairment/ loss of consciousness.
3. Automatism:
Abnormal voluntary activity like lip smacking, repeated swallowing effort,
sudden running etc.
4. Recovery:
Usually, after recovery there is amnesia about impaired consciousness
episode.
Focal seizure with secondary generalizations
This may be of 2 types:
110
1. Simple partial seizure followed by GTCS
2. Complex partial seizure followed by GTCS.
Generalized seizures:
Absence seizure
Typically occurs in children (4-8 years) and usually ceases by the age of 20.
Clinical features:
Sudden/ abrupt loss of external awareness: this spell typically lasts for
few seconds to minutes and followed by complete recovery.
Often occurs in the middle of a conversation when the patient misses a
few words/ breaks off in missed sentence and restart the conversation
from where he/she left off.
In some patients, it is accompanied by a tonic/ tonic-clonic movement.
Patients are not aware about these spells.
Atonic seizure
Abrupt loss of postural tone leading to dropping of head/ sudden collapse/
fall. These patients are at risk of getting injured.
Myoclonic seizure/ Juvenile myoclonic seizures
Sudden abnormal forceful contraction of muscles leading to violently
disobedient limb.
Generalized tonic clonic seizure (GTCS)
Clinical stages:
1. Tonic stage:
Characterized by generalized rigidity/ spasm of different muscles leading
to:
An abnormal posture (hyper-extended)
Eyes rolled upwards (ocular spasm)
Pooling of saliva and frothing (pharyngeal spasm)
Ictal cry/ a moaning (low pitch) sound (laryngeal spasm)
Tongue bite (Spasm of jaw)
111
A brief spell of respiratory arrest (spasm of respiratory muscles).
- Usually this tonic stage lasts for few seconds to minutes and
followed by clonic stage.
2. Clonic stage:
Abnormal jerky movement of different parts of the body which may be
short lasting (seconds to minutes) or may go on for a while.
If this continues for a while without any intervening period of recovery
of consciousness, it is called status epilepticus .
If this continues for a while with intervening periods of recovery of
consciousness, it is called serial seizures .
- During tonic-clonic spells, patient usually becomes unconscious.
3. Post-ictal period:
May last for few minutes to few hours and typically characterized by:
A state of confusion/ disorientation
Significant headache
Significant muscle pain
Patient may develop urinary/fecal incontinence. This is a usually a non-
specific symptom.
Investigation
(Correlate with the causes of seizure)
1. Blood: Hb, TC, DC, ESR
2. Renal function: Na+ K+ Urea Creatinine
3. Liver function test
4. Serum Ca++
5. Blood glucose
6. CE-CT brain (to rule out a structural lesion); if CT is inconclusive, then an
MRI may be required.
7. EEG: May be normal/ abnormal.
Treatment
General advice:
Ensure enough sleep for at least 8 hours a day
Avoid driving/ swimming/ working with heavy machinery
112
Seek attention in case of any infection
Monitor the side effects of your own medication(s).
Drug treatment:
1st line drug of choice in seizures
Focal seizure
Generalized seizure
Atonic/ myoclonic/
CLT
Absence
tonic/ tonic-clonic
Etx
VLT
C= Carbamazepine, L: Lamotrigine, T: Topiramate, V: Valproate, Etx: Ethosuximide
Intervention for medically refractory epilepsy:
1. Partial lobectomy
2. Lesionectomy
3. Vagus mediated stimulation.
Principles of drug treatment of seizures
1. When to start?
a. Idiopathic/ unprovoked seizures:
Wait and watch policy
Long term antiepileptic drugs (AED).
b. Secondary/ provoked seizures:
Treating the underlying cause is enough
AED on temporary basis
AED on long term basis.
113
2. What are the general principles of treatment?
a. Start on monotherapy, then gradually increase the dose till the
maximum dose is reached/ patient is seizure free
b. Monitor for side effects/ toxicity
c. If patient is not seizure free even after taking maximum tolerable dose,
then consider starting another 1st line drug
d. Gradually increase the dose of the 2nd drug and gradually taper off the
dose of 1st drug
e. If an add-on drug is required, then adjunctive drugs which are currently
used are:
Gabapentin
Pregabalin
Levetiracetam
Lacosamide.
3. When to stop?
AED can be stopped if the patient remains completely seizure free for
consecutive 2 years (or 5 years).
Dx of epilepsy
1. Non-epileptic seizure/ pseudoseizure/ functional seizure:
Points to differentiate:
a. More common in young female
b. Often witnessed
c. Aura absent
d. Convulsive movement often looks unusual
e. Injury rare
f. Tongue bite rare
g. Incontinence rare
h. Hyperprolactinemia absent.
2. Syncope:
Points to differentiate:
a. Pre-monitory/ warning symptoms are different from aura
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b. T itchi g/ jerky o e e t, if occurs, does 't last for more than few
seconds
c. No post-ictal symptoms
d. Tongue bite rare.
3. TIA:
Points to differentiate:
Negative symptoms predominant:
a. Weakness (loss of power)
b. Loss of sensation
c. Loss of special sense.
Status Epilepticus
Introduction
Repeated tonic-clonic seizures for a while without any intervening period of
recovery of consciousness.
Investigations
Once the seizure is controlled, relevant investigations are to be done to rule out
any provocable cause of seizure.
Management
A. Airway: Must be secured. If required apply oropharyngeal suction and
intubation.
B. Breathing: Free flow oxygen and ventilation, if required.
C. Circulation: Maintain circulatory volume by IV fluid
D. Drugs:
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IV Lorazepam or IV/Per rectal Diazepam
If fails
IV phenytoin (Loading dose followed by maintenance dose)
If fails
IV phenobarbitone
If fails
Intubation + Ventilation + General anesthesia / Deep sedation
- Once seizure is under control, long term AED treatment should be
considered.
116
Syncope
Introduction
Transient loss of consciousness (LOC) due to global cerebral hypoperfusion, where
LOC is abrupt in onset, of short duration and followed by spontaneous complete
recovery.
Causes
1. Neurally mediated/ reflex syncope:
A. Vasovagal attack:
Pathogenesis:
I. Vasodepressor response (loss of vasoconstrictor tone, particularly
in upright position)
II. Cardio-inhibitory response/ asystole.
B. Situational syncope:
I. Forced micturition
II. Forced defecation
III. Cough syncope.
C. Carotid sinus hypersensitivity.
2. Cardiogenic causes:
A. Arrhythmia:
I. Brady-arrhythmia:
o Sinus node disease
o AV junction block (Heart block)
II. Tachy-arrhythmia:
o Supraventricular tachycardia (SVT)
o Ventricular tachycardia (VT).
B. Structural heart disease:
I. Aortic stenosis (AS)
II. Hyperobstructive cardiomyopathy (HOCM).
3. Postural/ orthostatic hypotension:
Causes:
A. Drugs: Vasodilators (antihypertensives)
B. Autonomic neuropathy
C. Volume depletion: Bleeding/ dehydration.
117
Clinical features
A. Prodromal/ warning/ pre-syncopal symptoms:
Autonomic symptoms (suggestive of vasovagal attack):
Sweating
Nausea
Palpitation
Paleness of patient
Blurred vision.
Postural symptoms (suggestive of orthostatic hypotension):
Dizziness
Light headacheness
Blurred vision.
Usually no pre-syncopal symptoms (suggestive of transient
arrhythmia).
- Some patients with warning symptoms can actually prevent the loss of
consciousness by taking appropriate defensive mechanisms. Therefore,
these patients often come with pre-syncopal symptoms.
B. Syncopal episode:
Precipitating factors:
Prolonged standing (vasovagal)
Emotional stress:
Bad news/ severe fear/ anxiety/ horrible sight/ smell etc.
Overcrowded place
Sudden head turning/ tight collar shirt/ shaving (carotid sinus
hypersensitivity)
Sudden standing (postural hypotension).
Actual episode:
Transient loss of consciousness usually lasting never for a
period > (10-15) sec.
Often there is a gap in the memory
Jerky movements of the limbs (seizure) may occur but is
usually very brief and usually occurs after TLOC
Momentary urinary incontinence may occur
Tongue bite extremely rare.
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C. Recovery phase:
Usually recovery is complete and there is no post-syncopal
confusion/ drowsiness.
Signs
(Lying + standing) BP must be recorded if postural hypotension is suspected
Ask the patient to stand for 2-3 minutes: A fall of SBP/DBP /
Hg +
postural symptoms is suggestive of postural hypotension
Proper cardiovascular examination should be carried out to elicit any
cardiac abnormality which may cause syncope (Arrhythmia/AS/HOCM).
Investigation
1. ECG:
1. Resting ECG
2. 24 hours continuous ECG (if transient arrhythmia is suspected and
resting ECG is normal)
2. Echocardiogram (to rule out structural heart disease)
3. Carotid sinus massage (to confirm carotid sinus hypersensitivity)
- To be avoided in patients with H/O carotid TIA/ if carotid Doppler shows
atherosclerotic plaque.
4. Head tilt test/ Tilt table test (to diagnose neurally mediated syncope)
5. Electrophysiological study: Focus of tachyarrhythmia can be induced,
located and ablated, if required.
6. CT head (to rule out any internal bleeding as a consequence of fall).
Treatment
1. Neurally mediated syncope:
Advice the patient:
a. To avoid any triggering/ precipitating factor
b. To take a defensive mechanism during a pre-syncopal symptom
c. Physical counter pressure maneuvers:
I. Continuous limb movement while prolonged standing
II. To sit with crossed legs
III. Simple isometric exercise of the hand (hand-gripping)
d. To drink plenty of fluid
119
e. Midodri e (-agonist).
2. Carotid sinus hypersensitivity:
Permanent pacemaker
3. Cardiogenic syncope:
a. Treat the underlying cause
b. Implantable defibrillator in selected cases.
4. Orthostatic hypotension:
a. Treat the underlying cause
b. Avoid sudden standing.
Differential diagnosis
1. TLOC, but not due to global hypoperfusion:
a. Hypoglycemia
b. Primary seizure disorder
c. Vertebro-basillar TIA
2. No TLOC, but transient impaired consciousness:
a. Carotid TIA
b. Cataplexy.
Headache
Causes
Primary
Secondary
Migraine
Accidental (post-traumatic)
Tension headache
Acute angle closure glaucoma
Cluster headache
Bacterial sinusitis
Primary cough headache
Raised ICT
Primary exertional headache
CVA (causing SAH and ICH)
Primary sex headache
Meningitis
*Red colored causes should be ruled out first.
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Migraine
It is a condition characterized by recurrent severe headache resulting from
inappropriate vasodilation of cerebral blood vessels.
Pathogenesis
There is intermittent vasodilation of cerebral blood vessels which is mediated by
different neurotransmitters.
Clinical features
1. Triggering factors:
Bright light
Loud sound
Chocolate/ Caffeine
Oral contraceptive pills
2. Aura:
These are the symptoms which precede/ accompany the headache:
Visual symptoms:
o Flashes of light
o Zigzag lines
o Tunnel vision.
3. Headache:
Site: Classically the hemicranium, but may be generalized
Character: Throbbing
Intensity: Moderate to severe
Duration: For several hours
Accompanied by: Nausea, vomiting
Patient prefers to lie in closed eye/ in a dark quiet room.
Atypical varieties of migraine
1. Basillar artery migraine (posterior circulation migraine):
Headache + focal neurodeficit (Ex: Dys-equilibrium, Dysarthria,
Temporary drowsiness)
2. Ophthalmoplegic migraine:
Diplopia + Weakness of extraocular muscles.
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Investigation
It is essentially a clinical diagnosis. In severe recurrent headache, a CT head is
required to rule out any structural lesion.
Treatment
1. Avoid any triggering factor
2. Acute attack: Analgesic (Paracetamol/ NSAIDS) + Antiemetic
Drug of choice is Sumatriptan.
3. Prophylactic/ preventive:
A. Antiepileptic: Topiramate
Amitriptyline
- These 2 drugs are drug of choice.
B. -blocker: Propranolol
C. Calcium channel blockers: Flunarizine.
Tension headache
Severe recurrent headache often precipitated by stress.
Pattern of headache:
Site: generalized
Nature: Band like sensation/ constricting sensation
Severity: Moderate to severe
Usually no aura.
Investigation:
CT head, if required
Treatment:
Acute: Analgesic
Prophylactic: ABC (as above).
Cluster headache
Typically occurs in middle aged males.
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Pattern of headache:
Site: Orbital, retro-orbital and temple regions
Character: Moderate to severe
Nasal symptoms:
Running/ blocked nose
Ocular symptoms:
Wateri g fro eyes/ ptosis/ te porary Hor er's sy dro e
Why the a e cluster ?
It occurs in spells when the patient develops severe headache which may
vary in severity in different parts of the day and such spells recur almost
every day for few days to weeks; then there is a symptom free period of
few weeks after which the next cluster comes.
Investigation:
Ct head to rule out any structural lesion.
Treatment:
Acute: Analgesic + High flow oxygen + Sumatriptan
Prophylactic: Verapamil + Lithium.
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Extras
Involuntary movements
Tremor
It is a rhythmic oscillatory movement of different parts of the body due to
repeated contractions and relaxations of a group of muscles.
Classification/ types
Tremor
Depending upon frequency
Depending upon activity
Fine
Medium
Coarse
Rest
Intention
Postural
Postural tremor: Usually occurs when tremulous part of the body is kept in a
sustained posture.
Intention tremor: Tremor increases while attempting to execute a voluntary
activity.
Causes
Type
Causes
Fine/ intention/ postural
A. Alcohol excess/ withdrawal (chronic)
B. Benign essential tremor
C. Cigarette smoking
D. Drugs: 2 ago ist
E. Endocrinopathy: Thyrotoxicosis.
Medium/ coarse/ rest
Parkinsonism
Cerebellar lesion.
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Clinical features
Affected part of the body shows tremor: type and aggravating factor of
which depends on the underlying disease
Specific features of the underlying disease.
Investigation
1. TSH
2. Relevant investigations to assess the underlying disease
3. Stopping of any triggering factor.
Special note: Benign essential tremor
History:
Family history of tremor usually present.
Clinical features:
Description of tremor:
Bilaterally symmetrical
Fine tremor
Usually affects upper limbs, head, tongue but usually spares trunk and
lower limbs
Aggravated by stress
Temporarily relieved by alcohol.
Treatment:
Drug of choice: Propranolol
If fails: Primidone.
Chorea
It is an abnormal involuntary movement characterized by non-repetitive jerky
movement particularly affecting the extremities.
Site of lesion:
Caudate nucleus
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Causes:
1. S de ha 's chorea
2. Hu ti gto 's disease
3. Wilso 's disease
4. Chorea gravidarum.
Athetosis
It is an abnormal movement characterized by slow sinuous, often fine movement
particularly affecting the extremities.
Site of lesion: Putamen
Causes:
1. Wilso 's disease
2. Perinatal hypoxia
3. Kernicterus.
Fasciculation
Abnormal twitching movement involving few muscle fibres.
Types
Fasciculation
Physiological
Pathological
Denervating lesion particularly
When the muscle is exhausted
affecting AHC/ CNN
Motor neurone disease
Polio
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Dementia
It is a state of cognitive impairment which is usually slowly progressive and often
causes social and occupational dysfunction.
Causes
Vitamin B12 deficiency
Reversible
Hypothyroidism
Alzheimer's disease
Causes of dementia
Vascular dementia
(Multi-infarct disease)
Fronto-temoral dementia
Irreversible
Lewy body dementia
Normal pressure hydrocephalus
Progressive supranuclear palsy
Clinical features
1. Short time memory loss
2. Inability to name an object (nominal aphasia)
3. Visuo-spatial dysfunction:
a. Inability to navigate
b. Gets lost in a known surrounding
4. Executive dysfunction:
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a. Easy distractibility
b. Lack of concentration
5. Apraxia (inability to execute a motor activity in spite of normal power)
6. Along with these manifestations, patients often become delirious
7. Patient often develops apathy (indifference to others)
8. Specific signs and symptoms of the underlying disease
9. Mi i- e tal state e a i atio score <20.
Investigation
1. Dementia screen:
a. Blood: Hb, TC, DC, CRP/ESR
b. Serum vitamin B12
c. Serum TSH.
2. CT head.
Treatment
1. Non-pharmacological management:
Family and social support
2. Pharmacological management:
a. Treatment of any reversible cause
b. For dementia:
Anticholinesterase:
Donepezil
Rivastigmine
NMDA receptor blocker:
Memantine.
This post was last modified on 01 September 2021