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Hereditary Adult-Onset Alexander's Disease with Palatal Myoclonus, Spastic Paraparesis and Cerebellar Ataxia
Neurol 45:2266-2271, Schwankhaus,J.D.,et al, 1995
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Article Abstract
We describe a progressive neurologic disorder in three sisters characterized clinically by palatal myoclonus,spastic weakness, hyperreflexia,mild cerebellar dysfunction,and ocular motor abnormalities. Postmortem examination of one patient demonstrated widespread Rosenthal fiber deposition associated with myelination.The father previously was reported to have similar pathologic findings and carried a clinical diagnosis of multiple sclerosis.These clinical and pathologic findings describe a rare familial leukodystrophy that corresponds most closely to cases reported as adult Alexander's disease.Although similar pathologically to the well-characterized infantile variant of Alexander's disease,it is not known whether this adult variant represents the same disease process.
 
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Alexanders disease
Alexanders disease,adult onset
ataxia
ataxia,cerebellar
familial
hyperreflexia
leukodystrophy
leukoencephalopathy,differential diagnosis
MRI
MRI,abnormal
multiple sclerosis,misdiagnosis
palatal myoclonus
paraparesis
paraparesis,spastic
Rosenthal fibers
white matter disease

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