CNS Revision 1 21 157

CNS REVISION 1 ----- Active space -----

Headache 00:01:34

2 types :
Primary headache (90-95%) Secondary headache Pain insensitive
Recurrent. Causes : structures
No structural cause. • Infection (M/c : Meningitis). • Choroid plexus.
• Tumors : Rare. • Ependyma.

Dangerous headache
• First episode >55 years (Suspicion of
giant cell arteritis).
• Increasing severity over weeks.
• Early morning headache → Disturbed ↑ ICT : Perform fundoscopy
sleep associated with vomiting.
to rule out papilledema.
• Blurring of vision.
• ↑ on bending/lifting/coughing
(Meningeal inflammation).

IOC for ↑ ICT headache → MRI + MRA/MRV.

D/ds for ICT headache :
1. Space occupying lesions (M/c) :
• Tumor/tuberculoma/neurocysticercosis.
• Lesions affecting stalk : Tumor, infiltrative lesions (Sarcoidosis, Langerhans
Cell Histiocytosis (LCH), hemochromatosis, lymphocytic hypophysitis).
2. Cerebral venous thrombosis :
• Inherited cause : Factor V Leiden mutation.
• Acquired cause : Postpartum, Antiphospholipid Antibody Syndrome (APS).

Benign intracranial hypertension :

Previously called as pseudotumor cerebri.
Seen in middle aged obese females.
Slow ↑ in ICT over 3-6 months.
Sixth nerve palsy : False localising sign.
Imaging : Normal.
Opening pressure of CSF : ↑↑.
Rx : Repeated lumbar puncture to relieve pressure.
Topiramate (or) Acetazolamide.

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----- Active space -----

Primary headaches :
Tension type headache (TTH) > Migraine > SUNCT.
1. TTH :
• M/c cause of primary headache. • Female > male.
• No ↑ ICT signs. • 30-50 years.
• Does not affect activities of daily living. • 1/3rd cases are a/w depression.
• Perceived as a band/tightness/fullness. • No vomiting/photophobia/phonophobia.
Most effective in preventing daily attacks : TCA (Amitriptyline).
2. Migraine :
00:14:14
Female >> male.
Teenage.
↑ intensity with age.
Periodic in nature.
2 types : Classical Common
20% 80%
A/w aura : Visual aura (15 mins to 1 hour). No aura.
(Bright zig zag lines on periphery of visual field called as fortification spectra).

1-2 days prior, patient has low mood & irritability.

Properties of headache :
• Throbbing or pounding or pulsatile headache. • Duration : 4-72 hours.
• U/L (B/L in due course of time). • Location : Temporal.
• Vomiting ±.
• A/w photophobia, phonophobia (Patient prefers to sit quietly in a dark room).
• Post headache lethargy : Lasts for 1-2 days.
• Usually involves a trigger (Sleep deprivation : M/C).
Pathogenesis : Intracerebral vasoconstriction followed by vasodilatation.
Prophylaxis → Combat vasoconstriction.
Treatment → Combat vasodilatation.
DOC for :
• Mild to moderate attacks : NSAID.
• Moderate to severe attacks : 5 HT1B/1D agonists → Triptans.
• Severe to very severe attacks : 6 mg S/c Sumatriptan.
Triptans are contraindicated in ischemic heart disease.
Prophylaxis : Class I drugs → Propranolol, Valproate, Topiramate.
Trigeminal autonomic cephalalgia (TAC) :
Headache Any one of the I/L ANS symptoms :
• U/L. • Conjunctival congestion.
• Very severe. • Eyelid edema.
• Stabbing (or) + • Nasal congestion.
boring. • Rhinorrhea.
• Short lasting. • Lacrimation

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TAC includes : ----- Active space -----

• Cluster headache (M/C).
• Paroxysmal hemicrania.
• SUNCT : Short lasting unilateral neuralgic headache
with conjunctival injection & tearing.
• Hemicrania continua.

Cluster headache : 00:23:04

Young males.
Periodicity +.
Properties of headache :
• 1-8 attacks/day.
• Duration : 15 min to 3 hours.
• Location : Periorbital.
• Alcohol is a trigger.
• Can have migrainous features.
Aborted by : 6 mg s/c Sumatriptan (or) 100% O2 at 12-15 mL/min for 15-20 mins.
Prophylaxis :
• Short term : Steroid (or) Verapamil.
• Long term : Verapamil.
Other TAC :
Paroxysmal hemicrania SUNCT Hemicrania continua
• Female = male. • Male > female. • Female > male.
• Less severe attacks. • Cutaneous trigger. • Elderly.
• Less periodicity. • No refractory period • U/L continuous head-
• Nocturnal headache absent. following the cutane- ache.
• Alcohol is not a trigger. ous trigger. • Responds to
• 1-20 attacks/day. • Prevention : Lamo- Indomethacin.
• Duration : 2-30 mins. trigine. • ANS symptoms ++.
• Responds to Indomethacin.

Trigeminal neuralgia :
Middle aged to elderly.
Neuralgic pain along the distribution of V2 & V3.
Episodic pain.
If U/L : Compression of superior cerebellar artery.
If B/L : Demyelination (Multiple sclerosis).
Cutaneous trigger → refractory period → Tic douloureux (Severe pain).
Rx : Medical → Carbamazepine > Lamotrigine.
Not responding to medical Rx : Decompression Sx.

Note : Carbamazepine is C/I in HLA B1502.

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----- Active space -----
Lower motor neuron lesions
Lower motor neuron
structure
*Anterior horn cell (α and γ
motor neurons in the grey
matter of the spinal cord)
Dorsal root ganglion
Spinal nerve root/radicle
Plexus
Nerve
Neuromuscular junction
Muscle
Cranial nerve nuclei in
the brainstem + muscles
supplied :
• Midbrain : CN 3, 4.
• Pons : CN 5, 6, 7, 8.
• Medulla : CN 9, 10, 11, 12.
*Note : In AHC lesions → Asymmetric muscle wasting, fasciculations, DTR ++.
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CNS REVISION 2
00:00:34
Symptoms Diseases
• Degenerative motor neuron disease → Amyotrophic lateral
Purely motor sclerosis (UMN +LMN).
• Syringomyelia.
• Sjogren’s syndrome (Asymmetrical truncal + UL sensory
ataxia).
Purely sensory
• Para-neoplastic syndrome (Anti Hu antibody +).
• Cisplatin/taxanes.
• Radiculopathy → Disc compression → Inter-vertebral
disc prolapse : M/c (Brief electric shock-like pain, motor
weakness, loss of reflexes along the distribution of the
root).
• B/L polyradiculoneuropathy → Guillain Barre syndrome.
• Tumours.
• Mononeuropathy → Carpal tunnel syndrome (D/t
amyloidosis).
Sensory > • Mononeuritis multiplex :
motor • Leprosy (M/c).
• Polyarteritis nodosa.
• Cryoglobulinemia.
• Polyneuropathy :
• Acquired :
a. Large fibre → Vit B12 (SACD)/Vit E/Copper
deficiency.
b. Small fibre → Diabetes.
• Inherited → Charcot-Marie-Tooth disease.
• Myasthenia gravis (Diurnal variation of fatiguability, ptosis).
• Lambert-Eaton myasthenic syndrome.
• Intermittent weakness :
• Channelopathies.
• Mitochondrial myopathies .
Purely motor • Persistent weakness :
• Inherited → Dystrophies.
• Acquired :
a. Polymyositis, dermatomyositis.
b. Drugs/toxins.
c. Endocrine → Thyrotoxicosis, Cushing’s disease.
 CNS Revision 2 22 161

Large fibre V/S small fibre neuropathy : ----- Active space -----

Large fibre neuropathy Small fibre neuropathy

Tract/
Posterior column/Aα fibres. Spinothalamic tract/Aδ & C fibres.
fibres
Acquired : Inherited :
• Diabetes mellitus. • Fabry’s disease.
• Guillain-Barre syndrome (B/L
• Drugs. • Tangier’s disease.
polyradiculoneuropathy).
• Porphyria.
• Vit B12/vit E/copper deficiency.
Causes • Amyloidosis.
• Pyridoxine toxicity.
• Vasculitis.
• Taxanes.
• Uremia.
• Frederich’s ataxia.
• HIV.
• Leprosy.
Mostly axonal.
Mostly demyelinating.
Chronic axonal involvement.
Acute demyelinating
Sensory >> motor symptoms.
polyradiculopathy.
Distal → proximal progression.
Sensory + motor symptoms.
Motor weakness minimal.
Features Distal + proximal involvement.
Reflexes preserved (Except ankle jerk).
Motor weakness prominent.
NCS : Amplitude affected.
Reflexes lost.
Stocking and glove pattern.
NCS : Conduction velocity
In DM, amyloid, porphyria : ANS involvement
affected.
(M/c : Erectile dysfunction).
Tingling.
Positive Burning pain.
Cotton wool sensations.
symptoms
Paresthesia/dysesthesia.
Loss of pain/temperature/crude touch/
Sensory ataxia. pressure :
Negative
Numbness. • Non-healing ulcers.
symptoms
• Charcot joints.
• Burns.

Note : Vit B12/vit E/copper deficiency → Posterolateral cord syndrome + nerve

involvement.

Causes of combined neuropathies :

Combined large and small fibre neuropathy : Motor predominant neuropathy :
• Vincristine. • GBS.
• Carcinomatosis. • Porphyria.

All chronic neuropathies are axonal except Chronic Inflammatory Demyelinating

Polyneuropathy (CIDP).

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----- Active space -----

Guillain Barre syndrome :
Types :
1. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (M/c) :
• Males > females.
• Demyelinating fibres :
• Aα → Large/sensory-motor fibres.
• B → Preganglionic ANS fibres → ANS symptoms (Postural hypotension/arrhythmias).
• Mean age : 40 years.
• Characteristics :
• Acute monophasic : Lasts < 4 weeks .
• Inflammatory autoimmune : Anti-GM1 antibodies +nt. (Trigger → C. jejuni infection)
• Ascending polyradiculoneuropathy : LL → UL → brainstem (B/L LMN
7th CN palsy).

Symptoms :
Rubbery legs + tingling/cotton wool sensations/dysesthesia + transient blad-
der dysfunction → Rapidly progressive proximal to distal LL weakness → B/L
areflexic flaccid paraparesis.
2. Acute motor axonal neuropathy (AMAN) : Anti-GD1a antibody +nt
3. Acute motor sensory axonal neuropathy (AMSAN).
4. Miller Fisher syndrome :
• Demyelinating > axonal.
• Triad :
1. Ophthalmoplegia.
2. Arreflexia.
3. Ataxia.
Anti-GQ1b antibody +nt.
Treatment : Diagnostic tests :
• IVIG : 0.4g/kg for 5 days. • CSF : Albuminocytological dissociation.
• PLEX (Plasma exchange). • NCS : Demyelination.

Chronic inflammatory demyelinating polyneuropathy :

Duration : > 8 weeks.
Onset : Insidious.
Antecedent infection : 30% cases.
Presentation : Relapsing neuropathy with motor > sensory features.
• Motor : Proximal + distal LL predominant weakness.
• Sensory : Prominent symptoms + signs.
• ANS : Not involved.
Associated with :
• Cranial nerves : Not involved.
• HIV.
Treatment : • Myeloma.
• Steroids. • POEMS syndrome.
• PLEX.
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CNS REVISION 3 ----- Active space -----

Myasthenia gravis 00:00:17

Ocular myasthenia (15-20%) Generalised myasthenia (80-85%)

Fatiguability on repeated activity. Features of ocular myasthenia.
Diurnal variation. Pharyngeal muscle involvement.
Ophthalmoplegia + ptosis. Proximal muscles of lower limb involvement.
Autoimmune disease.
Age group affected : 20-40 years.
Female > male.
Males have a severe phenotype.
Associated diseases : Type-1 DM, vitiligo, pernicious anemia, Addison’s disease.

Pathogenesis :
Blocking antibodies against postsynaptic acetylcholine receptors (AChR).
AChR antibodies Anti musk antibodies
• Most specific. • Seen in 50% patients who are -ve for
• Seen in 80%. antibody against AchR.
• 70% have thymic hyperplasia, • Females without thymus involvement.
10% have thymoma. • Absent in ocular myasthenia.
• Tongue fasciculations.
• Unresponsive to pyridostigmine.

Double negative (AChR antibody -ve & anti musk antibody -ve) : Have anti LRP4 antibody.

Symptoms :
• 2/3rd patients start with ocular symptoms :
a. Asymmetric ptosis with B/L pupil sparing diplopia.
b. M/c muscle involved : Medial rectus.
• 10% have limb symptoms.
• Rest of the patients have pharyngeal muscle involvement : Nasal twang of
voice, dysarthria, dysphonia, dysphagia.
Note : Chronic progressive external ophthalmoplegia (CPEO) → B/L symmetrical
ptosis with no diplopia.
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----- Active space -----

Workup :
Icepack test.
Edrophonium test. Improvement
Neostigmine test.
Repetitive nerve stimulation test (RNST) :
• Baseline → Compound muscle action potential (CMAP) → Normal.
• Low frequency (3 Hz) repetitive stimulation → Decremental response.

Most sensitive test : Single nerve fibre electromyography.

Most specific test : AChR antibody.

Lambert Eaton myasthenic syndrome :

Antibodies against presynaptic V/Q Ca2+ channels.
Paraneoplastic syndrome : A/w small cell lung cancer.
Proximal lower limb weakness ++.
Diplopia, ptosis : Mild.
ANS symptoms ++.

RNST :
• Baseline CMAP : ↓↓.
• High frequency : Very high incremental response.

Treatment of myasthenia gravis :

DOC : Pyridostigmine 30-60 mg QID.
Followed by steroid and azathioprine to prevent relapse.
For myasthenic crisis : I/v steroid + IVIG.

Motor neuron disease 00:11:40

3 types :
1. Pure UMN : Primary lateral sclerosis.
2. UMN + LMN : Amyotrophic lateral sclerosis (ALS).
3. Pure LMN : Spinal muscular atrophy. Degenerating structures in ALS
ALS : • Anterior horn cells (AHC).
90% : Sporadic. • Cranial nerve nuclei in brainstem.
10% : Cu-Zn superoxide dismutase. • Corticospinal fibres upto AHC.
Seen in > 60 years. • Corticobulbar fibres upto cranial
Degenerative disease. nerve nuclei.
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Clinical features : ----- Active space -----

• Higher mental functions : Normal.
• Sensory symptoms : Nil.
• ANS : Normal.
• Cerebellum : Normal.
• Asymmetric involvement of upper limb → Wasting & atrophy → fascicula-
tions & cramps → exaggerated DTR.
• Distal + proximal involvement (Distal > proximal).
• Weakness.
• Can have either bulbar (40%) or pseudobulbar palsy (25%).

Bulbar palsy vs pseudobulbar palsy :

Bulbar palsy Pseudobulbar palsy

LMN involvement of cranial nerve nuclei UMN findings
in brainstem (9, 10, 11, 12) Involvement of corticobulbar fibres.
• Nasal twang. • Gag reflex +++.
• Nasal regurgitation. • Dysphonia.
• Flaccid tongue. • Small & spastic tongue.
• Flaccid dysarthria (Bulbar • Spastic dysarthria (Pseudobulbar
dysarthria). dysarthria).
• Jaw jerk ++.
• Emotional incontinence.

Spinal cord 00:18:43

Anatomy :
45 cm long & extends upto L1-L2. Origin of spinal cord segments
31 segments (C8, T12, L5, S5, C1). L4 to S2 From epiconus
Note : Cauda equina → L2 to C1. S3 to C1 From the conus (Bilaterally)

Arrangement of tracts in spinal cord :

Spinal cord tracts Arrangement of segments
(Medial to lateral)
Corticospinal tract (In lateral funiculus)
Spinothalamic tract (In lateral funiculus) C→T→L→S
All extrapyramidal tracts (In anterior funiculus,
except rubrospinal : Lateral funiculus)
Posterior column (In posterior funiculus) S→L→T→C
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----- Active space -----

Compressive myelopathy :

Intramedullary Extramedullary
Causes Ependymoma. Intradural : Neurofibroma, meningioma.
Syringomyelia. Extradural : Vertebral causes (Fracture,
mets, TB, etc), Vascular aneurysms.
Pain Funicular dull aching Root pain.
pain Tightness/band like (If posterior compres-
sion).
Sensory Spinothalamic tract +. Radiculopathy/Brown Sequard syndrome.
Posterior column spared. Posterior column symptoms (Upper limb >
Aka Dissociated sensory lower limb).
loss.
Bowel & ++ -
bladder
Motor Minimal motor involve- +++
ment (Lower limb > upper limb).
Sacral sparing.
AHC LMN + LMN -

Glove & stocking neuropathy : Sensory Brown Sequard syndrome

involvement in upper limb, motor • I/L pyramidal tract involvement.
involvement in lower limb. • I/L posterior column involvement.
• C/L spinothalamic tract involvement.

Conus medullaris v/s cauda equina :

Conus medullaris Cauda equina syndrome

S3 to C1
B/L symmetrical involvement.
No motor symptoms.
Anal/bulbocavernous reflexes lost.
LMN bladder.
Saddle anaesthesia.
L2 to C1
Asymmetrical : Root pain.
Variable motor symptoms.
Variable reflex loss.
Bladder involvement : Absent.
Sensory : Saddle anaesthesia or other patterns.

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Non compressive myelopathy : 00:35:34 ----- Active space -----

Causes :
Mneminc : “ VITAMIN ”
• Vascular (Anterior spinal artery infarction).
• Inherited (Fredrich’s ataxia).
• Toxins (Arsenic).
• Autoimmune (Sarcoidosis).
• Metabolic (B12, Vit E, Cu deficiency).
• Infections (TB, HIV).
• Neoplasia.

Acute transverse myelitis :

• Infectious trigger.
• Clear level : Band like sensation.
• Bowel/bladder : +++.
• At level → Hyperaesthesia & below level → UMN findings.
• Rx : Steroid.

Note : In GBS, there is no clear level & bowel/bladder involvement is absent.

Vitamin B12, vitamin E & Cu deficiency :

• Involvement of posterolateral cord + nerve + cerebrum (Sometimes).
• Posterior column → lateral spinothalamic tract → corticospinal tract →
large fibre neuropathy.
• Reflexes are lost late in the disease.
• Ankle jerk & knee jerk reflexes are absent (D/t neuropathy).
• Plantar reflex : Extensor.

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----- Active space ----- CNS REVISION 4

Dementia 00:00:48

Major cognitive impairment→ Daily activities affected.

Diagnostic criteria : More than one of the following functions are affected :
1. Memory → Medial temporal lobe / Hippocampus.
2. Language → Brocas (inferior frontal) & Wernicke (superior temporal).
3. Executive function → Dorsolateral prefrontal cortex.
4. Social cognition → Orbital prefrontal cortex.
5. Perceptual motor (praxicon) → Superior parietal lobule.
6. Complex attention → Orbital prefrontal cortex.

Functions of Prefrontal cortex

Prefrontal cortex

Medial Orbital Dorsolateral

Energisation and motivation Associated with : Executive function
Note : Lack of energisation • J : Judgement
and motivation → Apathy • I : Insight
• P : Personality
• F : Fluency
• A : Abstract thinking,
Attention

Causes :
Alzheimer’s Dementia (M/C) > Diffuse Lewy Body Dementia > Vascular dementia.
Other causes :
• Fronto temporal dementia (Picks disease)
• Normal Pressure hydrocephalus.
• Prion disease.
Note : 10 % of the dementia causes are reversible → Drugs , Vitamin B 12
deficiency, Chronic Meningoencephalitis, Normal pressure hydrocephalus,
Autoimmune conditions.

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Rapidly progressive dementia : ----- Active space -----

• Frontotemporal dementia
• Prion disease Also cause young onset dementia
• Diffuse Lewy body dementia
• HIV
• Whipples disease.
Note : In every young patients always rule out Chronic Meningoencephalitis &
Autoimmune pathology.

Alzheimer’s dementia 00:08:23

Also called → Tauopathy.

Pathogenesis :
APP gene on Chromosome 21 mutated
Amyloidogenic pathway

Aβ Amyloid

Aβ 40 → Cerebral amyloid angiopathy Aβ 42 → Alzheimer’s disease

Amyloid deposition :
Outside neuron →
Both composed of
Amyloid plaque
Aβ Amyloid Hyperphosphorylated tau
Inside neuron → (cause neuronal toxicity)
Neurofibrillary tangle
Note : No proven risk factors are present for Alzheimers disease.

Stages of Alzheimers disease :

1. Stage of amnesia.
2. Stage of anomia :
3. Stage of visuospatial disorientation (Non dominant parietal lobe affected)
4. Executive dysfunction (Prefrontal cortex affected)

Note :
• Lesions of angular gyrus → Gerstmann syndrome :
a. Acalculia
b. Finger anomia
c. Right to Left disorientation
d. Alexia with agraphia

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----- Active space -----

• Alexia without agraphia → Splenium of corpus callosum is involved.
• Visuospatial disorientation, constructional apraxia, dressing apraxia, hemispa-
tial neglect → Involvement of non dominant parietal lobe.
Treatment : Rivastigmine/ Donazepil/ Tacrine ( Cholinesterase inhibitors) +
Memantine (NMDA Antagonist).

Other causes of demetia 00:18:02

Frontotemporal dementia :
Frontotemporal dementia
Taupathy
MAPT gene on Chromosome 17
(loss of function)
Young onset (40-50 yrs)
30 % genetic
Rapidly progressive
Prefrontal cortex functions lost. Prefrontal cortex involved in the later
Alzheimers disease
Taupathy
• Presenilin 1 (Chr 14)
Early onset
• Presenilin 2 (Chr 1)
• Apo E4 → Late onset.
Late onset (>60 yrs)
5 % genetic
Slow progression
stages only.

Diffuse Lewy Body Dementia : 00:19:44

AKA Parkinson plus syndrome.
Subcortical dementia.
Characterized by Lewy bodies (α synuclein) in the neuronal cytoplasm.
Note : α synucleinopathies.
• Parkinsons disease
• Diffuse Lewy Body Dementia
• Multisystem Atrophy
Clinical features :
• REM Sleep disorders and ANS dysfunction (earliest symptom).
• Dementia > Rigidity > Tremors.
• Fluctuating alertness/ consciousness.
• Visual hallucination.
• Worsening of symptoms on starting antipsychotics.
• No response to L- Dopa.
Note : Cortical dementia v/s Subcortical dementia.

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Cortical Dementia Subcortical Dementia ----- Active space -----

Diseases • Alzheimers disease • Diffuse Lewy body dementia

• Frontotemporal • Progressive supranuclear palsy
Dementia • Normal Pressure hydrocephalus
Severity Severe Less severe
Apraxia/ Aphasia/ Agnosia (++) (-)
Extra pyramidal symptoms (-) (+++)
Corticobulbar involvement (-) (++)
Emotional involvement (-) (+)

Creutzfeldt jakobs disease : 00:26:58

It is a prion disease.
• Young onset dementia.
• β pleated sheet of prion.
• PrPsc → Protein involved.
• Rapidly progressive.
Clinical presentation :
Dementia + Myoclonus + High voltage sharp waves in a low slow background on EEG.
Pathological changes → Ribboning / spongiform changes in the cortex.

Vascular Dementia : 00:28:38

1. Post stroke Vascular dementia : Multi infarct state.

• Stepwise decline in cognitive function
2. Small vessel stroke : Lacunar stroke
• Risk factor : Systemic Hypertension.
• Pathogenesis : Lipohyelinosis.
• White matter disease → Binswangers disease( subcortical involvement).
• Characterised by :
1. Emotional fibres affected (corticobulbar)
2. Gait issues.
3. Urinary incontinence.

Normal Pressure Hydrocephalus : 00:31:19

Defective CSF absorption. G : Gait issues
( Apraxia → Ignition foot phenomenon)
Characterized by ventriculomegaly. U : Urinary incontinence
Clinical features : Hakim’s triad. D : Dementia ( subcortical)
Rx : Surgery.
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----- Active space -----

Movement Disorder 00:34:13

Controlled by Extrapyramidal system.

Hyperkinetic Hypokinetic
• Chorea • Parkinson’s disease
• Athetosis • Atypical Parkinson’s disease
• Hemiballismus
• Dystonia

Hypokinesia : Slowness or Paucity of movement + No evidence of weakness/ spasticity.

Parkinson’s Disease : 00:37:40

Usually present >60 yrs.
PARK 1 → Autosomal Dominant ( Chrom 4)
Young onset Parkinsons
PARK 2 → Autosomal Recessive ( Chrom 6)
80 % patients → Tremor predominant Parkinsons.
20 % patients → Akinetic rigid syndrome ( poor outcome).

Cardinal features :
• Tremor :
a. Asymmetric, distal pill rolling tremor
b. 4-6 Hz tremor
c. Micrographia(+)
d. Brought on by concentration.
e. Resting tremors.
• Akinesia/bradykinesia.
• Rigidity.

Other features :
• Glabellar tap sign (+)
• Lead pipe (elbow)/ Cog wheel rigidity (wrist)
• Hypomimia.
• Hypophonia.

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Atypical Parkinsonism : 00:42:45 ----- Active space -----

α Synuclenopathies Tauopathy
• Diffuse Lewy body Dementia • Progressive supranuclear palsy
• Multisystem atrophy • Corticobasal degeneration
Common symptoms :
• Rapidity of progression.
• No response of L- Dopa.
• Absence of tremors.

Multi system Atrophy ( MSA) vs Progressive Supranuclear palsy (PSP)

Multi system atrophy
Pathogenesis α Synnuclenopathy.
Clinical features • ANS symptoms → (+++)
• REM Sleep disorders → ( ++)
• Bilateral akinetic rigid Sym- • Wide based gait
metrical symptoms (axial)
MRI findings Hot cross bun sign
Progressive supranuclear palsy ( Steel
Richardsons syndrome)
Taupathy
• Max Bilateral Symmetrical axial
rigidity.
• Postural instability
• Supranuclear palsy → down gaze
palsy.
• Procerus sign : Wrinkling/ Furrowing
of the forehead
• Applause sign.
Humming bird sign/Morning glory sign

Types of MSA :
• MSA-P → Parkinson type
• MSA-A → Autonomic (Shydrager syndrome)
• MSA-C → Cerebellum

Corticobasal degeneration : 00:52:10

Tauopathy.
seen in elderly.
Clinical features :
• Asymmetrical
• Triad of → Apraxia + Myoclonus + Cortical dementia
• Hemiballismus /Parkinson features → ( Basal ganglia involvement).
Alien Limb phenomenon : Not using 1 limb and using the other limb.

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----- Active space ----- CNS REVISION 5

Approach to hemiplegia 00:00:44

Upper Motor Neurons (UMN) : Descending motor pathways.

1. Corticospinal tract.
Travel from cortex to the anterior horn cell.
2. Extrapyramidal tracts.
3. Corticobulbar tracts → upto cranial nerve nuclei in brainstem.

Causes of hemiplegia related to

Cortex Subcortex Brainstem

Cortex :
Corticospinal tract in cortex arises from :
• Motor cortex (Area 4) : 30 % fibres.
• Premotor cortex : 30% fibres.
• Sensory cortex (Area 3, 1, 2) : 40% fibres.
Frontal & parietal lobe Temporal lobe Occipital lobe
Supplied by : Supplied by : PCA.
M2 inferior division
Incongruent C/L homonymous
C/L hemiplegia Wernicke’s aphasia. macular sparing hemianopia.
Note : For motor weakness → Vessels involved :
• M1 (In sylvian fissure).
• M2 superior division.
• ACA (occlusion).

Features of cerebral lobes :

Frontal lobe Features of ACA occlusion :
Contains : • Lower limb > upper limb weakness.
• Prefrontal cortex. • Gait issues.
• Frontal eye field : • Bowel/bladder involvement.
To look to opposite side.
Note : B/L ACA occlusion → Severe apathy,
• Broca’s area.
akinetic rigidity, abulia.

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Parietal lobe : ----- Active space -----

Dominant Non-dominant
Areas : Features :
• Angular gyrus → Gerstman syndrome : • Visuospatial disorientation.
a. Acalculia, anomia. • Constructional apraxia.
b. Left to right disorientation. • Dressing apraxia.
c. Alexia with agraphia. • Hemispatial neglect (Severe
• Superior parietal lobule → Apraxia. form is c/a anosognosia).
• Supramarginal gyrus with association
areas : Visual agnosia.
Inferior quadrantanopia is seen in parietal lobe lesions.

Temporal lobe :

Medial Lateral
Functions : Areas :
• Memory. • Auditory cortex.
• Epileptogenic. • Auditory association areas → Auditory agnosia.
• Wernicke’s area.
Superior quadrantanopia is seen in temporal lobe lesions.
Limbic cortex (In temporal lobe) → Kluver Bucy syndrome.

Aphasia : 00:14:20

Fluency
No Yes
Motor aphasia Sensory aphasia

Comprehension + Comprehension - Comprehension + Comprehension -

Rep + Rep - Rep + Rep - Rep + Rep - Rep + Rep -

Transcortical Broca’s Isolation Global Nominal Conduction Transcortical Wernicke’s
motor aphasia aphasia/ aphasia aphasia aphasia sensory aphasia
aphasia echolalia aphasia

Note : Naming is affected in all aphasias.

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----- Active space -----

Subcortex :
Corticospinal tract passes through anterior 2/3rd of posterior limb of internal
capsule in close association with the corticobulbar fibres.

Features of internal capsule lesions :

• C/L dense hemiplegia.
• C/L UMN 7th nerve palsy.
• Hemisensory loss.
• Homonymous hemianopia.

Brainstem :
Crossed hemiplegia is seen in brainstem lesions : C/L hemiplegia + I/L cranial
nerve palsy.

Midbrain syndromes : 00:20:34

Corticospinal tract passes through middle 3/5 of crus cerebri.

th

Vessel involved : P2 segment of PCA.

Ventral midbrain syndromes
Weber syndrome Claude syndrome Benedict syndrome
Lesion : Base of mid- Lesion : Tegmentum. Features :
brain. Features : • Weber +
Features : • I/L 3 nerve palsy.
rd
Claude.
• I/L 3rd nerve palsy. • C/L ataxia (Dentato rubro • C/L
• C/L UMN hemiplegia. thalamo cortical fibres). hemiballismus.

Dorsal midbrain syndromes (Cause : Pinealoma)

Parinaud syndrome Nothnagel syndrome
Lesion : Tectum. Features :
Features : • I/L LMN 3rd nerve palsy.
• Vertical upgaze palsy → sunsetting • Gaze palsy.
sign. • C/L ataxia (Superior
• Overactive levator palpebrae superioris cerebellar peduncle).
→ lid retraction (Collier’s sign).
• Pretectal nucleus → Accommodation
reflex present, pupillary reflex absent.
• Convergence retraction nystagmus.
• Skew deviation of eyes.

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Pontine syndromes : 00:26:45 ----- Active space -----

Ventral pontine syndrome Dorsal pontine Lateral pontine syndrome

(Millard Gubler syndrome) syndrome (Foville (Marie fox syndrome)
syndrome)
F : I/L facial nerve palsy. F : I/L facial nerve A : I/L ataxia.
S : I/L sixth nerve palsy. palsy. S : C/L pain & temperature
H : C/L hemiplegia. G : Gaze palsy. loss (Spinothalamic tract).
H : C/L hemiplegia. H : C/L hemiplegia.

Medullary syndromes : 00:28:42

Lateral medullary syndrome Medial medullary syndrome

(Wallenburg syndrome) (Dejerine’s syndrome)
Vessel involved : V4 segment of vertebral artery 4 M’s :
> PICA • Motor nucleus of 12th nerve → I/L
Cranial nerves : LMN 12th nerve palsy.
• 5 : Spinal nucleus → I/L loss of sensation
th
• Motor fibres of pyramidal tract →
of face. C/L hemiplegia.
• 7th : NTS → Taste sensation. • Medial lemniscus → C/L posterior
• 8th : Vestibulocerebellar fibres → ataxia, column findings.
dizziness, vertigo. • Medial longitudinal fasciculus →
• 9th, 10th : Nucleus ambiguus → Dysarthria, Internuclear ophthalmoplegia.
dysphagia.
• 10th : Dorsal nucleus of vagus → Autonomic
features.
• 13th : Horner’s syndrome.
Sensory : C/L pain & temperature loss
(Spinothalamic tract).
Cerebellum : I/L ataxia (Spinocerebellar fibres).
No motor deficit.

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----- Active space -----

Stroke 00:34:50

Blood supply of brain :

> 22 mL/100 g/min : Normal.
< 10 mL/100 g/min : Infarct.
10 - 22 mL/100 g/min : Penumbra.

Transient ischemic attacks (TIA) :

The neurological deficit comes back to normal within 24 hours, generally within
an hour.
ABCD2 score : Predictor of risk of stroke.
Rx : Dual antiplatelet for 21 days.

Management of stroke :
In clinical practice :
High risk of stroke → Admit → CT brain (to detect bleed) + CT angio (to localise
large vessel involved).

Candidate for thrombolysis Contraindications for thrombolysis

• > 18 years. • Bleeding episode.
• < 4.5 hours from onset of stroke. • ICH.
• No bleed. • Surgery in last 14 days.
• No edema which is >1/3rd of the • GI bleed in last 21 days.
brain territory. • BP >185/110 mm Hg despite treatment.

If available at centre, mechanical thrombectomy is preferred over thrombolysis.

Thrombolysis → Alteplase (aka TPA) 0.9 mg/kg given :
• 10% as bolus.
• 90% as infusion over one hour.
Thrombectomy :
• Done if M1 (or) PCA are the vessels involved.
• Can be done upto 24 hours.

Most ideal line of Mx of stroke : Diffusion weighted MRI + CT brain.

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Diffusion weighted MRI (DW MRI) : ----- Active space -----

• Shows changes in form of hyperintensities from 30 mins to 6 hours after
onset of stroke.
• Apparent diffusion co-efficient sequencing shows hypointensities in stroke.
• Normal DW MRI means stroke is less likely.

DW MRI flair :
• It shows changes after 6 hours.
• Used in early morning stroke, where the exact time of onset of stroke can
not be determined.

MR angio : Detects main vessel occlusion.

Perfusion MRI : Detects penumbra.

Summary :
Assessment of risk of stroke
CT brain + DW MRI with ADC sequence If ADC sequence is
not available, add
MR angio to locate site of lesion perfusion MRI

First order lesion Unaccessible lesion

Mechanical
thrombectomy Presents within Presents after
4.5 hours 4.5 hours

Thrombolysis Medical Mx
Medical management :
• Aspirin (75 mg) within 48 hours → continued lifelong.
• No role of statins & dual antiplatelet agents.
• Anticoagulation : Only for cardioembolic stroke.
Warfarin : In valvular heart diseases, AF, mechanical heart valve.
Dabigatran : In other conditions.
• If BP > 220/110 mm Hg → Treat hypertension

Carotid endarterectomy :
• If carotid atherosclerosis > 70% (or)
• If carotid atherosclerosis is 50-70% with symptoms.

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