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Clinical Symptoms of Adult Metachromatic Leukodystrophy and Arylsulfatase A Pseudodeficiency
Arch Neurol 52:408-413, Hageman,A.T.M.,et al, 1995
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Article Abstract
In 13 patients,a diagnosis of adult metachromatic leukodystrophy was made. The main symptoms were dementia,behavioral abnormalities,ataxia,and polyneuropathy.In 12 patients,a diagnosis of arylsulfatase A pseudodeficiency was made.No characteristic clinical syndrome could be detected in these patients.Adult metachromatic leukodystrophy is a progressive metabolic disease with symptoms of demyelination of the central and peripheral nervous systems.Diagnosis must be confirmed by determination of arylsulfatase A activity and accumulation of sulfatides. Pseudodeficiency for arylsulfatase A can be confirmed or excluded by means of DNA analysis.
 
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arylsulfatase A
arylsulfatase A pseudodeficiency
metachromatic leukodystrophy
metachromatic leukodystrophy,adult onset
MRI
MRI,abnormal

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