Familial tremulous and myoclonic dystonia with white matter changes in brain magnetic resonance imaging
β Scribed by Dr. S. Bohlega; B. Stigsby; M. Z. Al-Kawi; D. R. McLean; P. Ozand; S. Omer; P. Coates
- Publisher
- John Wiley and Sons
- Year
- 1995
- Tongue
- English
- Weight
- 496 KB
- Volume
- 10
- Category
- Article
- ISSN
- 0885-3185
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β¦ Synopsis
Abstract
We report two families with a disorder, probably autosomal recessive, characterized by tremor of juvenile onset, dystonia, and myoclonus with preserved cognitive, cerebellar, and peripheral nervous system functions. During 4 years' followβup, mild spasticity appeared. Magnetic resonance imaging (MRI) revealed mild diffuse changes in the white matter. Central conduction times for visual, motor, and sensory systems were all prolonged. Extensive metabolic workβup failed to reveal lysosomal, peroxisomal, mitochondrial, or other metabolic abnormalities.
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