๐ง Demyelinating diseases are neurological disorders caused by damage to the myelin sheath โ the protective covering of nerve fibers in the central and peripheral nervous systems.
Myelin enables rapid, efficient nerve conduction, and its loss disrupts neuronal communication, leading to a wide range of neurological symptoms.
๐ Common Demyelinating Diseases
- Multiple Sclerosis (MS): The most common demyelinating disease. An autoimmune condition where the immune system attacks the CNS, causing plaques of scar tissue in the brain and spinal cord.
- Neuromyelitis Optica (NMO / Devicโs disease): Primarily affects the optic nerves and spinal cord, causing recurrent visual loss and paralysis. Now recognised as distinct from MS (anti-AQP4 antibody mediated).
- Acute Disseminated Encephalomyelitis (ADEM): Rare, usually post-infectious or post-vaccination; causes a sudden widespread attack on myelin, especially in children.
- Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): A peripheral nervous system disorder with gradual limb weakness and sensory loss due to immune attack on peripheral myelin.
- Progressive Multifocal Leukoencephalopathy (PML): A rare, often fatal CNS infection with JC virus, seen in immunocompromised patients (e.g. HIV, transplant, natalizumab therapy).
โ ๏ธ Causes & Pathogenesis
- Immune system dysfunction โ myelin misidentified as foreign.
- Genetic susceptibility โ e.g. HLA associations in MS.
- Infections โ molecular mimicry may trigger autoimmunity.
- Environmental factors โ low vitamin D, smoking, migration risk patterns.
๐ฉบ Symptoms
- Weakness & fatigue
- Numbness, tingling, or sensory loss
- Balance & coordination problems (ataxia)
- Visual symptoms โ blurred vision, diplopia, optic neuritis
- Cognitive impairment โ poor concentration, memory loss
- Spasticity, stiffness, painful spasms
- Neuropathic pain (burning, shooting sensations)
- Bladder & bowel dysfunction
๐ฌ Diagnosis
- Clinical assessment: Pattern of relapsing-remitting or progressive neurological deficits.
- MRI: White matter lesions (periventricular, juxtacortical, infratentorial, spinal cord).
- CSF: Oligoclonal bands (esp. MS).
- Blood tests: To exclude mimics (B12 deficiency, vasculitis, infections).
- Electrophysiology: Nerve conduction studies for CIDP.
- Use of diagnostic frameworks: e.g. McDonald criteria for MS.
๐ Treatment
- Corticosteroids: High-dose IV methylprednisolone for acute relapses.
- Disease-modifying therapies: e.g. Interferon-ฮฒ, glatiramer acetate, natalizumab, fingolimod, ocrelizumab (MS).
- Immunosuppressives: Rituximab, azathioprine, mycophenolate for NMO/CIDP.
- Plasma exchange (plasmapheresis): For severe, steroid-refractory attacks.
- Symptomatic therapies: Baclofen for spasticity, gabapentin for neuropathic pain, bladder management, physio/OT.
- Supportive care: Rehabilitation, counselling, lifestyle modifications (exercise, vitamin D, smoking cessation).
๐ Prognosis
The outlook depends on the disease and its aggressiveness.
๐ฟ MS often follows a relapsing-remitting course with variable disability.
โก NMO tends to be more aggressive without immunotherapy.
ADEM usually resolves, while PML carries a poor prognosis.
๐ Early diagnosis & disease-modifying therapy are key to slowing progression and preserving quality of life.