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, CONGENITAL. 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)

Descriptor ID	D020371
MeSH Number(s)	C10.228.140.163.100.362.775 C10.228.140.695.625.775 C10.314.400.775 C16.320.322.906 C16.320.565.189.362.775 C18.452.132.100.362.775 C18.452.648.189.362.775
Concept/Terms	Pelizaeus-Merzbacher Disease Pelizaeus-Merzbacher Disease Pelizaeus Merzbacher Disease Sclerosis; Brain, Pelizaeus-Merzbacher Pelizaeus-Merzbacher Sclerosis; Brain Sclerosis; Brain, Pelizaeus Merzbacher Leukodystrophy, Hypomyelinating, 1 Pelizaeus-Merzbacher Disease, Transitional Pelizaeus-Merzbacher Disease, Transitional Pelizaeus Merzbacher Disease, Transitional Transitional Pelizaeus-Merzbacher Disease Transitional Pelizaeus Merzbacher Disease Cockayne-Pelizaeus-Merzbacher Disease Cockayne-Pelizaeus-Merzbacher Disease Cockayne Pelizaeus Merzbacher Disease Pelizaeus-Merzbacher Disease, Atypical Pelizaeus-Merzbacher Disease, Atypical Pelizaeus Merzbacher Disease, Atypical Atypical Pelizaeus-Merzbacher Disease Atypical Pelizaeus Merzbacher Disease Adult Pelizaeus-Merzbacher Disease Adult Pelizaeus-Merzbacher Disease Adult Pelizaeus Merzbacher Disease Pelizaeus-Merzbacher Disease, Adult Pelizaeus Merzbacher Disease, Adult Classic Pelizaeus-Merzbacher Disease Classic Pelizaeus-Merzbacher Disease Classic Pelizaeus Merzbacher Disease Pelizaeus-Merzbacher Disease, Classic Pelizaeus Merzbacher Disease, Classic

Below are MeSH descriptors whose meaning is more general than "Pelizaeus-Merzbacher Disease".

Below are MeSH descriptors whose meaning is related to "Pelizaeus-Merzbacher Disease".

Below are MeSH descriptors whose meaning is more specific than "Pelizaeus-Merzbacher Disease".