Sunday, May 1, 2016

Every seizure are nor simple seizure neither epilepsy -DrSudeepKC

A 3-month-old boy exhibits nystagmus and limb tremors unassociated with seizures. Over the next few years, he develops optic atrophy, choreoathetotic limb movements, seizures, and gait ataxia. 

picture of 3 month boy having nystagmus and limb moments

He dies during status epilepticus and at autopsy is found to have widespread myelin breakdown with myelin preservation in islands about the blood vessels.

The pathologist diagnoses a sudanophilic leukodystrophy to describe the pattern of staining observed on slides prepared to look for myelin breakdown products.

a. Neuromyelitis optica (Devic’s disease)
b. Central pontine myelinolysis
c. Marchiafava-Bignami disease
d. Acute disseminated encephalomyelitis
e. Pelizaeus-Merzbacher disease

The answer is e. Pelizaeus-Merzbacher disease is a demyelinating disorder that belongs to a group of degenerative diseases known as sudanophilic leukodystrophies. 

Leukodystrophy refers to the disturbance of white matter, and sudanophilic refers to the Sudan staining
characteristics of the involved white matter. Children with PelizaeusMerzbacher disease typically become symptomatic during the first months of life, but survival may extend into the third decade of life. Most affected
persons are male.


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