| PMD | Pelizaeus-Merzbacher disease; posterior mandibular depth; primary myocardial disease; private medici... |
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| PMD | Pelizaeus Merzbacher Disease |
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| Pelizaeus, Friedrich | <person> German neurologist, 1850-1917. See: Merzbacher-Pelizaeus disease, Pelizaeus-Merzbacher disease. (05 Mar 2000) |
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| Pelizaeus-Merzbacher disease | A sudanophilic leukodystrophy with a tigroid appearance of the myelin resulting from patchy demyelination. Type 1-classic, nystagmus and tremor appearing in the first few months of life, followed by slow motor development sometimes with choreoathetosis, spasticity, optic atrophy and seizures, with death in early adulthood, X-linked recessive inheritance; type 2-contralateral form with death in months to years after birth, X-linked recessive inheritance; type 3-transitional, with death in the first decade; type 4-adult form associated with involuntary movements, ataxia and hyperreflexia, but without nystagmus; type 5-variant forms. Cockayne is sometimes included as a sixth form. Synonym: Merzbacher-Pelizaeus disease. (05 Mar 2000) |
| pelizaeus-merzbacher syndrome | <radiology> Slowly progressive dysmyelinating disease, diffuse, symmetrical demyelination, cerebrum, cerebellum, brainstem, spinal cord, islands of partially demyelinated fibres, CT may be normal, two types: X-linked recessive (presents in infancy), autosomal dominant (preadulthood) (12 Dec 1998) |
| Merzbacher-Pelizaeus disease | A sudanophilic leukodystrophy with a tigroid appearance of the myelin resulting from patchy demyelination. Type 1-classic, nystagmus and tremor appearing in the first few months of life, followed by slow motor development sometimes with choreoathetosis, spasticity, optic atrophy and seizures, with death in early adulthood, X-linked recessive inheritance; type 2-contralateral form with death in months to years after birth, X-linked recessive inheritance; type 3-transitional, with death in the first decade; type 4-adult form associated with involuntary movements, ataxia and hyperreflexia, but without nystagmus; type 5-variant forms. Cockayne is sometimes included as a sixth form. Synonym: Merzbacher-Pelizaeus disease. (05 Mar 2000) |
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Synonyms : Adult Pelizaeus-Merzbacher Disease, Atypical Pelizaeus-Merzbacher Disease, Classic Pelizaeus-Merzbacher Disease, Pelizaeus-Merzbacher Disease, Adult, Pelizaeus-Merzbacher Disease, Atypical, Pelizaeus-Merzbacher Disease, Classic, Pelizaeus Merzbacher Disease
| Pelizaeus-Merzbacher disease |
Pelizaeus-Merzbacher disease (PMD) is a rare central nervous system disorder in which coordination, motor abilities, and intellectual function are delayed to variable extents. The disease is one of a group of genetic disorders called the leukodystrophies that affect growth of the myelin sheath, the fatty covering--which acts as an insulator--on nerve fibers in the brain. ...
Ãâó: en.wikipedia.org/wiki/Pelizaeus-Merzbacher_disease
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| Pelizaeus-Merzbacher d. |
an X-linked leukoencephalopathy, occurring in early life and running a slowly progressive course into adolescence or adulthood. It is marked by nystagmus, ataxia, tremor, choreoathetoid movements, parkinsonian facies, dysarthria, and mental deterioration. Pathologically, there is diffuse demyelination in the white substance of the brain, which may involve the brain stem, cerebellum, and spinal cord. The cause is mutation of the gene on the long arm of the X chromosome that codes for proteolipid protein. Called also Merzbacher-Pelizaeus d., familial centrolobar sclerosis, and Pelizaeus-Merzbacher sclerosis.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| Pelizaeus-Merzbacher s. |
see under disease.
Ãâó: www.mercksource.com/pp/us/cns/cns_health_library.j...
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