Degeneration of the cerebellar system in X-chromosome?linked copper malabsorption
| Autor: | Asao Hirano, Makoto Iwata, Joseph H. French |
|---|---|
| Rok vydání: | 1979 |
| Předmět: |
Adult
Cerebellum Pathology medicine.medical_specialty Adolescent Red nucleus Somatic cell Purkinje cell Biology Cerebellar Cortex Purkinje Cells medicine Humans Neurons Afferent Menkes Kinky Hair Syndrome Brain Diseases Metabolic Dorsal spinocerebellar tract Age Factors Anatomy Granule cell medicine.anatomical_structure Dentate nucleus Superior cerebellar peduncle nervous system Neurology Neurology (clinical) |
| Zdroj: | Annals of Neurology. 5:542-549 |
| ISSN: | 1531-8249 0364-5134 |
| DOI: | 10.1002/ana.410050609 |
| Popis: | Examination of the cerebellar system of 5 autopsied patients with X-chromosome-linked copper malabsorption led to the discovery that among cerebellar afferent systems, only the dorsal spinocerebellar tract showed consistent degenerative changes. Cerebellar cortical lesions comprised granule cell loss and Purkinje cell abnormalities that included nuclear and cytoplasmic degenerative changes with cell loss and deficient dendritic arborization as well as the presence of somatic sprouts. Perisomatic baskets were often absent in the area of sprouting Purkinje cells. Cerebellar cortical topographical analysis revealed relative sparing of the caudal portion of the cerebellum. The nodulus was intact in all patients. The dentate nucleus and the superior cerebellar peduncle were preserved. The red nucleus was consistently degenerated. |
| Databáze: | OpenAIRE |
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