Clinicopathological differences between juvenile and late infantile metachromatic leukodystrophy |
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Authors: | S Takashima A Matsui Y Fujii H Nakamura |
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Institution: | 1. Division of Child Neurology, Institute of Neurological Sciences, Tottori University School of Medicine, Yonago;2. Division of Neuropathology, Institute of Neurological Sciences, Tottori University School of Medicine, Yonago |
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Abstract: | The autopsy report of the juvenile type of metachromatic leukodystrophy is rare. The clinical and pathological difference between the juvenile and the late infantile type of metachromatic leukodystrophy was described. Loss of myelin sheaths was much less in the brain stem and spinal cord in the juvenile type than in the late infantile type, although that of the roots or cranial and spinal nerves was marked similarly in both types. The relationship of these pathological findings to clinical signs and laboratory data was discussed. |
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Keywords: | Metachromatic leukodystrophy lipidosis demyelination neuropathy |
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