Cells of the CNS😊

Imp

• Demyelination:
• Conduction velocity reduced
• Distal latencies

• Axonal:
• Low amplitude

Multiple Sclerosis:

• CNS demyelinating disease.

• Away from equator, Low Vit D → ↑ incidence

• There is plaque formation in the brain.

• Revised McDonald criteria
• Dissemination in time and space

• Charcots triad (SIN)
• SCANNING SPEECH
• INTENTION TREMOR
• NYSTAGMUS
• Due to MLF → Internuclear Ophthalmoplegia

• Lhermitte sign
• Shock like sensation down the spinal cord

• Uthoff sign
• Increases with temp

• Also called Dawson finger.

• U/L Optic nerve is involved.

• Presentation:
• Female patient.
• Mainly of Caucasian origin.
• Northern part of Europe origin.
• British women.
• One sided decreased visual acuity.
• Also has relative efferent pupillary defect.
• Optic neuritis.
• Bladder/bowel involvement.

• Autoimmune: 
• Antibodies against 
• Myelin basic protein 
• Oligodendrocyte glycoprotein.
• Oligodendrocytes attacked/killed by T-cells → Demyelination.

• Effect: 
• Decrement in nerve conduction velocity.

• Mnemonic:
• Myru nn pryunnath basic (Myelin basic protein) anu
• Olipich olipich (Oligodendrocytes) nadakkum

MRI head

Treatment:

• Acute episode/First presentation:
• Visual blurring, decreased colour vision:
• Methylprednisolone

• Mitoxantrone
• Most effective

Condition	DOC
Acute Episode	IV Steroids
Primary Progressive	Ocrelizumab
Relapsing Remitting	β-IFN

Prevention:

• β-Interferon

• Glatiramer

• Dimethyl Fumarate

• Monoclonal antibodies
• In worst case scenario
• Natalizumab
• S/E → Progressive Multifocal Leucoencephalopathy
• Aw JC virus
• Mnemonic: Nattelilu lesions varathirikan

PPMS (Primary Progressive Multiple Sclerosis):

• Ocrelizumab.

• Mnemonic: To progress → First Ocred (Ocrelizumab)

SPMS (Secondary Progressive Multiple Sclerosis):

• Siponimod.

• Mnemonic: Second sip

NOTE

• NMOSD (Neuromyelitis Optica Spectrum Disorder).
• Young female patient
• Sudden onset bilateral optic neuritis
• Recurrent

• In multiple sclerosis:
• Unilateral involvement.
• Gradual slow process.
• Secondary progressive multiple sclerosis.

ADEM - Acute Disseminated Encephalomyelitis

• <20yrs

• Monophasic

• Antecedent infection

• ON : B/L

• Spinal cord: Long segment

Optic Neuritis

• Definition: Inflammation of the optic nerve.

• Cause: Most common cause is Multiple Sclerosis.

Clinical Features:

• Mnemonic: 2 CUP mar

• Central scotoma.

• Color blindness (red & green).

• Unilateral loss of vision.

• Pain in eye on ocular movements.

• Marcus Gunn pupil (RAPD).

• Uhthoff sign:
• Worsening of symptoms with increased body temperature (e.g., exercise).
• Mnemonic: Uhthoff → Utto → Up → Temperature

• Pulfrich sign:
• Tachtokinetic dissociation.
• Illusion of depth perception
• Mnemonic: Pullil thotta ariyulla

• Lhermitten’s sign
• Passive neck flexion causing an electric shock-like sensation radiating to the spine and shoulders
• Due to increased sensitivity of the myelin to stretch

Investigation

• MRI

• LP
• NMO (Neuromyelitis optica antibodies → Anti Aquaporin 4 antibody)
• Nemo (NMO) fish → aquatic (Anti Aquaporin 4)
• MOG (Myelin Oligocyte Glycoprotein) antibodies

Treatment:

• Steroids (IV →f/b oral)

• f/b β Interferons

• Glatiramer acetate : 30 mg s/c injection.

Mnemonic:

• Vinnu → calls myre (myelin basic protein), pulle (pulfrich), then spits - thphuuu (uhthoff)

• Olipich (oligodendrocytes) nadakkunavan vannu

• she took gun (marcus gunn) → fire to his eyes

• he got eye pain (pain on movement)

• Kandavar scoot ayi (scotoma)

NEUROMYELITIS OPTICA / DEVIC'S DISEASE

• https://www.notion.so/Neuro-3-2350a7eb0c778023ac7dc562d86f9426?source=copy_link#2350a7eb0c778068b7efe6f96af88a61

NMOSD (Neuromyelitis Optica Spectrum Disorder)
DEVIC'S DISEASE

• DEVIC'S DISEASE

• 20 -40 yrs

• Female: Male ratio = 3:1.

• Demyelinating disease.

• Disease is Astrocytopathy
• Damage to astrocyte foot processes causes cerebral edema and death.
• Astrocyte foot processes: 
• Do not form blood brain barrier.
• BBB → Formed by endothelium of Brain capillaries

Cause

• Can be secondary (2°) to Multiple Sclerosis

Death

• Spinal cord involvement at C3, C4, C5

• Phrenic nerve supply to diaphragm is affected.
• Causes respiratory failure.

• NOTE: In many neurological diseases
• parkinsonism, Alzheimer’s, VCJD
• death is often due to pneumonia from being bedridden and aspirating.

Antibodies:

• NMO IgG antibodies / Anti-Aquaporin-4 antibody
• Damages water channel,
• Causes cerebral edema

• Anti-MOG
• Myelin oligodendrocyte glycoprotein antibody.

Manifestation:

1. Optic neuritis
• Sudden onset blindness
• bilateral
• Increased (↑) chance of relapse
• More frequent than in Multiple Sclerosis alone
• History of recurrent Optic Neuritis
• most important to differentiate from MS

2. Longitudinal extensive transverse myelitis:
• >3 consecutive spinal segments.
• Symptoms:
• Pin, needle sensations,
• Root pain/radicular pain below level.
• Acid or boiling pain

3. Spectrum disorder manifestations:
• A: Area postrema syndrome (protracted vomiting).
• B: Brainstem syndrome (3rd cranial nerve or any other nerve palsy).
• C: Cerebral syndrome (seizures, encephalopathy).
• D: Diencephalic syndrome (hypothalamus affected)
• Hunger affected → Anorexia, Weightless
• N: Narcolepsy (damage to Reticular activating system).

NOTE

• Multiple sclerosis:
• Relapsing/remitting course,
• mostly unilateral ocular deficit.

Diagnosis criteria for NMOSD

• At least
• 1 clinical core feature + Anti aquaporin 4 antibody.

• If antibody not identified:
• 2 clinical core features + Gadolinium enhanced MRI.

• MRI:
• Optic neuritis.
• MRI spine: >3 segments involved.
• Cloud like brain lesions (parenchyma or brain stem).

Treatment:

• β-Interferon, Glatiramer: Contraindicated.

• Methylprednisolone.

• No improvement: Plasmapheresis.

Prevention:

• Immunomodulators +
• Mycophenolate, Azathioprine

• Steroids

Important Information

• Therapeutic Plasmapheresis indicated in:
• HUS.
• TTP.
• NMOSD.
• GBS