Demyelinating diseases Flashcards
(10 cards)
Gullain Barre syndrome
Usually a post infection ascending paralysis. 30% by campylobacter jejuni, 10% by CMV. Can cause/result respiratory failure.
Multiple Sclerosis
Auto immune demyelination of cns.
Characterized by white matter lesions.
frequently presents with optic neuritis internuclear opthalmoplegia, hemiparesis/sensory sx, and bowel bladder incontinence.
A single episode of demyelination is not diagnostic for MS; it is called:
Clinically isolated syndrome
Progressive Multifocal Leukoencephalopathy:
Cause?
Commonly found in?
Increased risk with what drug?
A rapidly progressive often fatal CNS demyelination of oligodendrocytes.
Caused by reactivation of JC virus.
Seen in 2-4% of AIDS pts
Higher risk associated with natalizumab, a second line MS drug.
Acute Disseminated Enecephalomyelitis:
Cause?
Multifocal, periVENULAR inflammation and demyelination.
Cause: a post infections syndrome. Often follows measels, varicella zoster, or vaccines (Rabies or smallpox).
Metachromatic leukodystrophy:
Cause?
Findings?
An autosomal recesive lysosomal storage disorder. (arylsulfatase A deficiency). Build up of sulfatides leads to impaired myelin production -> central AND peripheral deymelination.
Pts have ataxia and dementia.
Krabbe disease
Cause?
Findings?
AKA Globoid cell leukodystrophy.
An autosomal recessive lysosomal storage disease due to deficiency in galactocerebrosidase. -> buildup of galactocerebroside destroys myelin sheath.
Pts have peripheral neuropathy, developmental delay, optic atrophy, and GLOBOID CELLS - large mphage with eosinophilic cytoplasm.
Acute inflammatory demyelinating polyradiculopathy
The most common variant of guillan barre. Auto immune destruction of schwan cells ->demyelination of peripheral nerves. Ascendding paalysis, facial paralysis in 50%.. Autonomic function affected.
Associated with Campylobacter jejuni and CMV.
Adrenoleukodystrophy
cause?
X linked genetic disease (males only) where long chain fatty acid metabolism is impaired and they build up in CNS, adrenals and testes. Leads to coma and death.
Internuclear Opthalmoplegia, often seen in MS, is due to:
In a ‘right INO’, what would you see?
Lesion of the Medial Longitudinal Fasciculus. The MLF connect the CNIII and VI nuclei so that they can coordinate horizontal gaze.
In a right INO, you have a right lesion of the MLF, the right eye will not b able to look left using the medial rectus (CNIII) and the abducted eye (the LEFT) will show nystagmus.
