Medical Dictionary

Pelizaeus–Merzbacher disease

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noun Pel·i·zae·us–Merz·bach·er disease \ˌpel-ēt-ˈsā-əs-ˈmerts-ˌbäḵ-ər-\

Medical Definition of Pelizaeus–Merzbacher disease

  1. :  a progressive disease of the central nervous system that is marked by myelin degeneration resulting in deterioration of cognitive and motor functioning Pelizaeus-Merzbacher disease is a leukodystrophy with symptoms including involuntary eye movement, delayed development (as of motor skills and language), weak muscle tone, impaired coordination, and spasticity. It is inherited as an X-linked recessive trait requiring that both parents pass on a copy of the defective gene on the X chromosome.

Biographical Note for pelizaeus–merzbacher disease

Pelizaeus

,

Friedrich

(1850–1917), and

Merzbacher

,

Ludwig

(1875–1942), German neurologists. In 1885 Pelizaeus described a congenital and familial disease that is due to atrophy of the white matter of the brain and whose characteristics include speech disturbances, loss of coordination, and mental impairment. Merzbacher independently described the same disease in 1908.


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