Degeneration of the cerebellar system in X-chromosome-linked copper malabsorption

M. Iwata, A. Hirano, J. H. French

Research output: Contribution to journalArticle

39 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)542-549
Number of pages8
JournalAnnals of Neurology
Volume5
Issue number6
StatePublished - 1979

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Purkinje Cells
X Chromosome
Copper
Spinocerebellar Tracts
Red Nucleus
Cerebellar Nuclei
Neuronal Plasticity
Cerebellum

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Degeneration of the cerebellar system in X-chromosome-linked copper malabsorption. / Iwata, M.; Hirano, A.; French, J. H.

In: Annals of Neurology, Vol. 5, No. 6, 1979, p. 542-549.

Research output: Contribution to journalArticle

Iwata, M, Hirano, A & French, JH 1979, 'Degeneration of the cerebellar system in X-chromosome-linked copper malabsorption', Annals of Neurology, vol. 5, no. 6, pp. 542-549.
Iwata, M. ; Hirano, A. ; French, J. H. / Degeneration of the cerebellar system in X-chromosome-linked copper malabsorption. In: Annals of Neurology. 1979 ; Vol. 5, No. 6. pp. 542-549.
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