About The Medical Condition Pelizaeus-Merzbacher Disease, Transitional

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Find the definition of the medical term Pelizaeus-Merzbacher Disease, Transitional. Pelizaeus-Merzbacher Disease, Transitional defined and explained for easy understanding at the Medical Conditions from ClusterMed.info.

Pelizaeus-Merzbacher Disease, Transitional

Pelizaeus-Merzbacher Disease, Transitional Medical Condition Defined & Explained
Pelizaeus-Merzbacher Disease, Transitional Medical Condition

What's The Definition Of The Medical Condition Pelizaeus-Merzbacher Disease, Transitional?

A rare, slowly progressive disorder of myelin formation. Subtypes are referred to as classic, congenital, transitional, and adult forms of this disease. The classic form is X-chromosome linked, has its onset in infancy and is associated with a mutation of the proteolipid protein gene. Clinical manifestations include TREMOR, spasmus nutans, roving eye movements, ATAXIA, spasticity, and NYSTAGMUS. Death occurs by the third decade of life. The congenital form has similar characteristics but presents early in infancy and features rapid disease progression. Transitional and adult subtypes have a later onset and less severe symptomatology. Pathologic features include patchy areas of demyelination with preservation of perivascular islands (trigoid appearance). (From Menkes, Textbook of Child Neurology, 5th ed, p190)

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