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CENTRAL NERVOUS SYSTEM

DR.SRINATH.CHANDRAMANI
Asst.Prof & ICU in-charge
K.J.Somaiya Medical College,
Mumbai

Introduction

The human nervous system is the organ of


consciousness, intelligence and is the most
intricate structure known to exist.
One-third of the 35,000 genes encoded in
the human genome are expressed in the
nervous system.
Each mature brain is composed of 100 billion
neurons.

Methods of approach
Pathology based approach
Etiology based approach
Anatomical axis based approach

Pathology Based approach

Ion Transmission & Channelopathies


Neurotransmitter imbalance
Gene transcription defects
Degenerative
Hypoxic injury
Infection and Inflammation

Etiology Based approach

Infective
Vascular
Traumatic
Drug and toxin induced
Degenerative
Neoplastic
Miscellaneous

Anatomical Axis based approach


My preferred approach
Most methodical and organised
Easy to remember and recollect

CNS
Pyramidal System

Extra Pyramidal System

Basal Ganglia Complex


UMN

LMN
Cortex
Sub Cortex
Internal Capsules
Brainstem
Spinal Cord

Anterior Horn Cell


Root/Radicle
Plexus
Peripheral Nerve
N-M Junction
Muscle

CORTEX

Altered sensorium
Convulsion
Aphasia
Memory/calculation Impairment
Apraxia
Homonymous hemianopia
Cortical sensation loss

SUBCORTEX
Dementia
Homonymous Hemianopia

Brought in vogue by :
o HIV PML &
o Dementia syndromes

Internal Capsule
Complete Hemiplegia
Dense hemiplegia
Involvement of UMN facial on same side
of Hemiplegia

Brainstem
Crossed Hemiparesis

Cranial nerve involvement


PCA territory
Various syndromes

BRAINSTEM CIRCULATION
A.

Midbrain
Syndromes

B.
C. Pontine
Syndromes

D. Medullary
syndromes

MIDBRAIN SYNDROMES
SYNDROME

SITE

CRANIAL N.

OTHER FEATURES

IPSILATERAL 3RD
NERVE
IPSILATERAL 3RD
NERVE

CONTRALATERAL HEMIPLEGIA

WEBERS

CEREBRAL PEDUNCLE

BENEDICTS

RED NUCLEUS

NOTHNAGELS

SUPERIOR CEREBELLAR
PEDUNCLE

IPSILATERAL 3RD
NERVE

CONTRALATERAL CEREBELLAR
ATAXIA

CLAUDES

RED NUCLEUS +
SUPERIOR CEREBELLAR
PEDUNCLE

IPSILATERAL 3RD
NERVE

CONTRALATERAL TREMOR,
CHOREA, ATHETOSIS &
CEREBELLAR ATAXIA

CONTRALATERAL TREMOR,
CHOREA, ATHETOSIS

PONTINE SYNDROMES
SYNDROME

SITE

FOVILLES

DORSAL
PONS

MILLARD
GUBLER

VENTRAL
PONS

CRANIAL N.

OTHER FEATURES

IPSILATERAL
LATERAL GAZE PALSY,
6TH, 7TH NERVE CONTRALATERAL
HEMIPARESIS
IPSILATERAL
ONLY ABDUCENS PALSY,
7TH. 6TH ONLY
CONTRALATERAL
FASCICLE
HEMIPARESIS

MEDULLARY SYNDROMES
SYNDROME
MEDIAL
MEDULLARY
SYNDROME

LATERAL
MEDULLARY
SYNDROME

CRANIAL
NERVE
Ipsilateral
12TH nerve

OTHER FEATURES

Ipsilateral 5th
nerve

Ataxia of limbs, falling to side of lesion:


Horners syndrome (miosis, ptosis, decreased
sweating):
Loss of taste: Nucleus and tractus solitarius
Numbness of ipsilateral arm, trunk, or leg:
Contralateral loss of pain and thermal sense
due to Spinothalamic tract

Ipsilateral
fibers of
9th & 10th
nerves

Contralateral incomplete hemiparesis and


contralateral impairment of tactile and
proprioceptive sense (medial leminiscus)

BASILAR ARTERY SYNDROME


Also called Syndrome of the lone vertebral
artery:
Bilateral long tract signs (sensory and motor;
cerebellar and peripheral cranial nerve
abnormalities)
Paralysis or weakness of all extremities, plus all
bulbar musculature: Corticobulbar and
corticospinal tracts bilaterally

Misc syndromes
Compete Medullary syndrome due to
occlusion of Vertebral artery.

Occlusion of Internal Carotid artery


leads to blindliness due to opthalmic
artery Amaroux fugax

CRANIAL NERVE DISORDERS


Olfactory nerve : unilateral anosmia
helpful in detecting frontal lobe tumours.
Parosmia is altered smell. Present mainly
as Aura of temporal lobe epilepsy. Also in
atrophic rhinitis

CRANIAL NERVE DISORDERS


Optic nerve :
Vision worse than 6/60 is legally blind.
Field of vision is 90,75,75,60 from temporal to inferior in
counter-clockwise direction.
Homonymous hemianopia is due to cortical or subcortical
infarction.
Bitemporal hemianopia is due to optic chiasmal lesions.
When Visual examination is normal with loss of vision, it is
due to Cortical blindness.

CRANIAL NERVE DISORDERS

3rd/4th/6th are always discussed together.


Ptosis Unilateral & Bilateral.
Light and Accomodation reflex.
False localising sign of 6th nerve.
Isolated 3rd nerve lesions are common in DM.
Pupillary sparing differentiates from other
causes.

CRANIAL NERVE DISORDERS


Trigeminal Sensory and Motor
nerve.
Sensory loss distribution
Trigeminal neuralgia
Jaw Jerk reflex Pseudo-bulbar
palsy

CRANIAL NERVE DISORDERS


UMN & LMN facial palsy
LMN palsy Bells phenomenon & palsy.
Sparing of Emotional fibres
differentiates Bilateral UMN from
Bilateral LMN palsy.
Hyperacusis N. Stapedius involvement.

CRANIAL NERVE DISORDERS


Auditory nerve Vestibular &
Cochlear.
Vertigo
Hearing loss.
Caloric test Brain dead criteria.

CRANIAL NERVE DISORDERS


9th & 10th together
GAG reflex
11th Trapezius and
Sternocleidomastoid
12th tongue movement & protrusion.

SPINAL CORD

Symmetrical, Bilateral
Root pain, Cord pain, Funicular pain
Radicular pain
Motor + Sensory affection
Bladder/Bowel involvement
Flexor spasms
Clonus

ANTERIOR HORN CELL


Pure motor
Commonly unilateral
E.g. Poliomyelitis

Radicle / Plexus
Symptoms involving nerve root
distribution
Typical presentation
E.g. erbs palsy

Peripheral nerve
Motor /+ sensory
Sensory symptoms
positive/negative

Asymmetrical at onset

Neuro-Muscular junction
Fluctuating motor weakness
E.g. Myasthenia gravis

Muscle
Pure motor
Fasciculations
Atrophy / pseudo-hypertrophy
Involvement in groups

SUMMARY
UPPER MOTOR NEURON
FROM CORTEX TO SPINAL CORD

LOWER MOTOR NEURON


ANT HORN CELL TO MUSCLE

SPASTICITY / HYPERTONIA
FLACCIDITY/ HYPOTONIA
EXAGERATED DEEP TENDON JERKS DIMINISHED/ABSENT DTR
ABSENT ABDOMINAL REFLEX

PRESENT

EXTENSOR BABINSKIS
NO MUSCLE WASTING
NO FASCICULATION

ABSENT BABINSKIS
WASTING COMMON
FASCICULATION COMMON

Extra-pyramidal system
Accessory nervous system
Controls co-ordination, balance and
fine-tuning of motor movements.
Presents as involuntary movements

Summary of involuntary movements

MOVEMENT
INTENTION TREMOR

SITE
CEREBELLUM

DESCRIPTION
TREMOR ONLY ON ACTION

RESTING TREMOR

SUBSTANTIA NIGRA
RED NUCLEUS
CORTEX

AT REST ONLY.
ABOLISHED BY ACTION
INTERMITTENT MUSCLE
RELAXATION
HYPERTONIA OF BOTH AGONIST &
ANTAGONIST
SLOW, RHYTMIC MOVEMENTS

ASTEREXIS
RIGIDITY
CHOREA
HEMIBALLISMUS

SUBSTANTIA NIGRA
PUTAMEN
CAUDATE NUCLEUS

SUBTHALAMIC
NUCLEUS
DYSTONIA/ATHETOSIS PUTAMEN

VIOLENT, FLINGING MOVEMENT


WRITHING / FOCAL MOVEMENT

INVOLUNTARY MOVEMENTS CTD..


MOVEMENT
MYOCLONUS

DESCRIPTION
RAPID, RHYTHMIC, SHOCK
LIKE MUSCLE JERKS

CAUSES
IN SLEEP, MYOCLONIC EPILEPSY

FASCICULATION

NON-RHYTMIC MUSCLE
FASCICLE CONTRACTION.

SIGN OF ANT,HORN CELL


DEGENERATION., MND, SYRINX,
OPP, PRIMARY MUSCULAR
ATROPHY, THYROTOXIC/
CARCINOMATOUS MYOPATHY

BEST SEEN IN LARGE


MUSCLES DELTOID OR
CALVES.
FIBRILLATION

TWICTHING OF SINGLE
MUSCLE FIBRE

DENERVATION
HYPERSENSITIVITY. BEST TONGUE

INVOLUNTARY MOVEMENTS CTD..


MOVEMENT
MYOKYMIA

DESCRIPTION
CONTRACTION OF
BUNDLE OF MUSCLE
FASCICLES

CAUSE
MOST COMMON
INVOLUNTARY
MOVEMENT. EG.
ORBICULARIS OCULI
CHRONIC DENERVATION
WITH RENERVATION. MND.

MINIPOLYMYOCLONUS

TREMOR-LIKE IN SMALL
JOINTS

TITUBATION

INVOLUNTARY NODDING LESIONS OF VERMIS OF


OF HEAD
CEREBELLUM

TEST 1

CORTEX & INTERNAL CAPSULE


CEREBROVASCULAR ACCIDENTS
MULTIPLE SCLEROSIS
EPILEPSY
INFECTIONS

CEREBRO-VASCULAR ACCIDENTS
Thrombo-embolic stroke
Hemorrhagic stroke
Small-vessel disease
Intra-cranial hemorrhage
Cerebral venous sinus thrombosis
Sub-archnoid hemorrhage
Transient ischemic attack
Special types Todds paralysis.

CEREBRAL
CIRCULATION

Classification on Territory
involved

Anterior circulation stroke


Middle cerebral artery
circulation
Posterior circulation stroke

Anterior circulation stroke


Excellent collateral circulation thru circle of
Willis.
Hence only Bilateral infarction / unpaired ACA
lesions become clinically manifest.
Presents as Paraplegia with bladder/bowel
involvement.
Recurrent artery of Heubner Facio-branchial
monoplegia

Middle cerebral circulation stroke


Most common area of CVA
Lenticulo-striate branches
involved.
Clinical features as per area
involved.

Posterior circulation stroke


Affects brainstem and
cerebellum.
Already various syndromes
discussed

Parameter
Age
Onset
Progression
Convulsion

Differences in presentation

Raised ICT

Embolic
Young
Hyperacute
Max at onset
Most
common
Uncommon

Time of event
Treatment
Prognosis

Morning
Self resolving
Excellent

Thrombotic
Old
Step-ladder
Over hours
Rare

Hemorrhagic
Middle age
Acute
Over mins
Occasional

Uncommon exc. Malignant MCA


infarct
Night
Thrombolysis/aspirin
Moderate

Common
Evenings
Conservative
Poor

Thrombolysis in stroke

Role of Imaging in stroke


Indications
Contra-indications
Approved Drug
Routes and respective Window period
Complications

Small vessel disease/


Lacunar infarct syndrome
Occurs in old age
Due to occlusion of small perforating br.
Progressive natureover months to years
Most important risk factor - hypertension
Preventable disorder
Treatment
Prognosis

Cortical venous sinus thrombosis


Epidemiology
Causes
Clinical presentation : next slide
Treatment
Prognosis

CORTICAL VENOUS THROMBOSIS


SITE
CAVERNOUS
SINUS

CLINICAL FEATURES
PROPTOSIS, PTOSIS, OPTHALMOPLEGIA,
PAPILLEDEMA. OPT.div. 5TH NERVE

SUPERIOR
SAGGITAL SINUS

SIGNS OF BOTH HEMISPHERE INVOLVEMENT,


HEADACHE, PAPILLEDEMA AND CONVULSIONS

TRANSVERSE
SINUS

9TH,10TH,11TH CRANIAL NERVE INVOLVEMENT


WITH HEMIPARESIS, PAPILLEDEMA AND FITS

Radiological signs in CVST


Cord sign In Plain CT scan, hyper density of
the straight sinus and cortical vein due to
fresh blood clot within the vein or sinus.
Empty Delta sign seen in contrast CT, due to
enhancement of walls of superior sagittal
sinus with hypo dense area within it.

Intracerebral bleed
Hypertension most important risk
factor
Putamen most common site
Subthalamus rarest
Treatment

SAH

8% of all strokes
Rupture of aneurysym most common
cause.
Clinical features
Diagnosis
Treatment
Prognosis

Misc causes of Stroke


Moyamoya disease is an occlusive disease involving large intracranial
arteries, especially the distal internal carotid artery and the stem of
the middle and anterior cerebral arteries. Vascular inflammation is
absent.
The lenticulostriate arteries develop a rich collateral circulation
around the occlusive lesion, which gives the impression of a puff of
smoke (moyamoya in Japanese) on conventional x-rayangiography .
The disease occurs mainly in Asian children.
Because of the occurrence of intracranial hemorrhage,
anticoagulation is risky.

Misc causes of Stroke


Reversible posterior leukoencephalopathy can occur in head injury,
migraine, sympathomimetic drug use, eclampsia, and the postpartum
period.
The etiology is unclear but likely involves widespread cerebral
segmental vasoconstriction.
Patients complain of headache and manifest fluctuating neurologic
symptoms and signs, especially visual symptoms. Sometimes cerebral
infarction ensues.
Conventional x-ray angiography is the only means of establishing the
diagnosis, but because angiography itself can cause spasm of vessels,
even the existence of this vascular entity is debated.

Misc causes of Stroke


Binswangers disease (chronic progressive subcortical
encephalopathy) : is a rare condition.

CT or MRI scans detect periventricular white matter


infarcts and gliosis.
There is lipohyalinosis in the small arteries of the deep
white matter, as in hypertension. There are usually
associated lacunar infarcts.

Misc causes of Stroke


CADASIL
(cerebral autosomal dominant arteriopathywith subcortical infarcts and
leukoencephalopathy)
is an inherited disorder that presents as small-vessel strokes, progressive dementia,
and extensive symmetric white matter changes visualized by MRI.
Approximately 40% of patients have migraine with aura, often manifest as transient
motor or sensory deficits.
Onset is usually in the fourth or fifth decade of life.
This autosomal dominant condition is caused by one of several mutations in Notch3, CADASIL is the only monogenic ischemic stroke syndrome so far described.
Genetic testing is available.

MULTIPLE SCLEROSIS

CNS demyelinating disorder


Young onset, F > M.
Presenting complaints :

Optic neuritis, INO, Hemiparesis,


numbness,
Paraesthesia, Ataxia.
Uthoffs phenomenon
Lhermittes sign

Paraparesis, Tingling

Multiple Sclerosis
Diagnostic criteria
Types
Neuromyelitis Optica / Devics disease

Treatment
Prognosis

Epilepsy
Partial
Simple, Complex, Secondary generalisation

Generalised
GTC, Absence, Myoclonic epilepsy.

Unclassified
Status epilepticus
Todds Paralysis

Anti-Epileptic agents
Levotiracetam
Phenytoin / Fosphenytoin
Carbamazepine / Oxcarbamazepine
Sodium Valproate
Diazepam/Lorazepam
Phenobarbitone
Vigabratine/ lamotrigine Add-on only

Headache
Primary vs. Secondary headache

Primary Headache :
Migraine-type
Tension-type
Cluster headache

CNS INFECTIONS
Acute Pyogenic Meningitis.
Brain abscess.
TBM
HSV meningoencephalitis
PML JC virus
CJD
Cryptococcal meningitis

Dementia
Progressive memory impairment
Anterograde more common
Alzheimers : Gene defect APP gene,
Presenilin 1
Presenilin 2
Apo-E
Characterised by neurofibrillary tangles.
Most pathology in Nucleus basalis of Meynert.
Drug used is Tetrahydroacramine (Tacrine)

Neuro-regressive disorders

SSPE
Epilepsy
Wilsons disease
Glycogen storage disorders

Halloverden Spatz
Mitochondrial mutations (MELAS)

Treatable Dementia

Alcohol / Drug intoxication


Wernickes encephalopathy
Korsakoffs psychosis
Endocrine Hypothyroid & Addisons
Infections Viral, TBM, Neurosyphilis,
Whipples disease
Depression
Sarcoidosis
Normal pressure Hydrocephalus

Test 2

Spinal cord disorders

Brown Sequard syndrome


Paraplegia
Compressive
Non-compressive
Transverse myelitis
Subacute combined degeneration
Anterior Spinal artery syndrome

Brown Sequard syndrome

Hemi-section of the cord. Consists of :


ipsilateral weakness (corticospinal tract)
loss of joint position and vibratory sense (posterior column), with
contralateral loss of pain and temperature sense
(spinothalamic tract) one or two levels below the
lesion.
Segmental signs, such as radicular pain, muscle atrophy, or loss of a
deep tendon reflex, are unilateral.
This classical syndrome is rare, and partial forms
are more commonly encountered.

Compressive Myelopathy
All myelopathies are compressive till
otherwise proved !!!

Extradural lesions
Intradural lesions
- Extramedullary
- Intramedullary

Non-compressive myelopathy

Transverse Myelitis is the prototype


Neuromyelitis Optica
Subacute combined degeneration of
the cord
Anterior spinal artery syndrome
Hematomyelia

Transverse Myelitis

Post-infective phenomenon
Commonly demyelinating, self-limiting.
Campylobacter jejuni, Borrelia, viral ,etc
Progression Plateau Recovery
Treatment : IVIG, Steroids, Plasmapheresis
Prognosis

SCD of cord

Secondary to Vitamin B12 deficiency


No relation to severity of anaemia
Common in alcoholics
Suspicion must for diagnosis
Dissociate anaesthesia
Treatment & Prognosis

Anterior spinal artery syndrome

Single ASA as compared to paired PSA.


Dissociate anaesthesia
Iatrogenic
Artery of Adamkowicz
Prognosis

Syringomyelia

Commonly congenital
Progresses with growth, but rarely
symptomatic
Dissociate anaesthesia
Cape and Hood type of anaesthesia
Treatment
Prognosis

Anterior Horn cell

MND Both Genetic/Acquired


Mixture of UMN and LMN signs
Predominatly UMN ALS
Predominatly LMN SpinoMuscularAtropy
Small muscle wasting & Fasciculations are
hallmark of disease.
ALS-plus syndromes :
with FTD / Parkinsons

Peripheral neuropathy
AIDP / GBS
CIDP

SENSORY NEUROPATHY
PURE MOTOR NEUROPATHY
AUTONOMIC NEUROPATHY
MIXED

GUILLIAN BARRE SYNDROME


Epidemiology
Asburys criteria
Acute, Ascending, symmetrical, Flaccid Quadriparesis with
Areflexia.
Preceding febrile illness
Cranial nerve involvement
Sensory / bladder / Bowel rare.
Albumino-cytologic dissociation in CSF
Demyelinating neuropathy
Treatment IVIG/ Plasmapheresis.

Subtypes of
GBS

Neuro-muscular junction disorders


PARAMETER
Site of pathology
Epidemiology
Age
Clinical features
Antibody

Association
EMG
Treatment

MYASTHENIA
Post-synaptic
F >> M
Young
Diplopia, Ptosis
Anti-AcHr in 80%
Anti MuSK (muscle
specific kinase) in 40-50%
Thymoma
Decremental response

EATON-LAMBERT
Pre-synaptic
M >> F
Elderly
Proximal muscle weakness
Anti-P/Q type
calcium channels
in 85%
Small cell Ca Lung
Incremental response

Anti-Cholinergic,
ImmunoSupressio

Malignancy treatment. Symptomatic


Rx.

Muscle disorders
Muscular Dystrophy
Duchennes
Beckers
Limb Girdle
Facio-Scapulo-humeral
Oculopharyngeal

Muscle disorders
Myopathy
Endocrine, Toxin, etc

Myositis
Dermato/Polymyositis
Inclusion body myositis

Test 3

Parkinsonism
Parkinsonism vs. Parkinsons disease
Triad Bradykinesis
Rigidity
Resting tremor

Non Motor symptoms of Parkinsons disease

D/D for Parkinsonism


Idiopathic Parkinsons disease
Vascular Parkinsonism Binswangers
Drug induced parkinsonism
Normal pressure Hydrocephalus
Shy-Drager syndrome
Lewy body disease
Olivo-pontine degeneration
Striato-nigral degeneration
Multi-system atrophy

Treatment of Parkinsons disease


Dopamine
Dopamine Agonists
Anti-cholinergics
Seligeline
Amantidine

Misc topics yet important


Glasgow coma scale
Huntingtons Chorea
Neuro-cutaneous syndromes
Prion disease
Intracranial tumours

GLASGOW COMA SCALE


Most sensitive scale for Head injury patient
Includes following parameters :
Eye Opening 1 to 4
Best verbal response 1 to 5
3 if intubated)
Best motor response 1 to 6
Minimum score 3 , maximum score 15
Interpretation : < 8 indicates 85% mortality
> 11 indicates 85% survival

Huntingtons disease

Tri-Nucleoside (CAG) repeat disorder


Autosomal dominant
Phenomenon of anticipation
Starts in middle age and progresses
Absence of IgA
Associated with progressive dementia
No cure. Prognosis poor.

Other Tri-Nucleotidase diseases

Freidricks ataxia
Heriditary telengectasia
Sturge Weber syndrome
Histiocytosis

Neurocutaneous syndromes

Neurofibromatosis
Tuberous sclerosis
Sturge Weber syndrome
Spina bifida
Ataxia telengectasia

Neurofibromatosis
Genetic diseases with Autosomal transmission. Presents in 2 forms :
NF-1 (Von Recklinghausen/peripheral NF)
NF-2 (central NF) bilateral acoustic neur

Caf-au-lait spots
Lisch nodules pigmented nodules on iris
Cutaneous Neurofibroma
Skeletal and spinal deformity.

No cure. Family counselling must.

Tuberous Sclerosis
Adenoma sebaceum
Shagreen patch
Ash leaf macules
Manifests as Mental retardation, Seizures,
CNS hamartomas

Ataxia-Telengectasia

Osler-Rendu-Weber syndrome
Multiple AV malformations in the Liver, lung,
CNS and mucous membranes.
Manifests as Progressive ataxia.
Treatment is surgical and temporary.

Prion disease
Prion is the only non-nucleated
pathogen to cause human infections.
It is also called Transmissable
spongiform encephalopathy.
It is unique in the way that the etiology
maybe genetic, sporadic or infectious.

Prion disease

Creutzfeldt-Jacob disease, Bovine


spongiform encephalopath, familial
fatal insomnia, Kuru are the
examples.
Brain Biopsy at autopsy is diagnostic.
Prevention is the only treatment.

CNS tumours
Most common intracranial tumours in Metastasis.
Most Common tumour to metastasise to the brain Lung Ca
Most common glial tumour - Astrocytoma
Most common site In children - Posterior fossa
Most common CNS tumour to spread
Medulloblastoma

CNS tumours
Cerebellar Hemangioblastoma seen in VonHippel Lindau syndrome
Primary CNS lymphoma is characterised by
being diffuse histiocytic in type. Common in
AIDS patient, Since it is multicentric,
Chemotherapy is definitive treatment.

Critical Care Neurology


Coma
Brain death
Hypoxic-Ischemic encephalopathy
Osmotic Dysequilibrium syndrome
Neurogenic shock

THANK YOU !