Volume 56, Issue 6 (9 1998)
Tehran Univ Med J 1998, 56(6): 67-70 |
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Ghelichkhani H, Ghaffarpoor M. Multifocal dystonia, Clinical feature of Hallervorden-Spatz. Tehran Univ Med J 1998; 56 (6) :67-70
URL: http://tumj.tums.ac.ir/article-1-1510-en.html
URL: http://tumj.tums.ac.ir/article-1-1510-en.html
Abstract: (7694 Views)
Hallervorden-spatz disease is an inherited metabolic disorder with autosomal recessive trait. Onset is in late childhood or early adolescence. Clinical manifestation is variable but pyramidal and extrapyramidal signs are often prominent. Many of patients show progressive dementia and extrapyramidal symptoms. Ataxia or myoclonus is reported in the course of the disease in individual cases. Focal dystonias including tongue, eyelids (blepharospasm) and optic atrophy, retinitis pigmentosa, rarely familial parkinsonism are also reported. Pathologically pigmentary degeneration of globus pallidus, substantia nigra (pars reticular) and red nucleus is characteristic. In our case the main clinical feature was multifocal dystonia without obvious pyramidal or other extrapyramidal symptoms, and diagnosis was based on clinical and MRI findings.
Keywords: Hallervorden-Spatz, Myoclonus
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