Thalamic degeneration in X‐chromosome–linked copper malabsorption

Makoto Iwata, Asao Hirano, Joseph H. French

Research output: Contribution to journalArticle

10 Scopus citations

Abstract

Thalamic degeneration was present in 5 autopsied cases of X‐chromosome–linked copper malabsorption (X‐cLCM), Menkes' kinky hair disease. Among the thalamic nuclei, those in the formatio paraventricularis, intralamellaris, and extralamellaris were spared. The nuclei projecting to the granular cortices had severe neuronal depopulation. The thalamic nuclei that send axons to the agranular cortices were less often and less severely involved. The thalamic afferent system was intact except for degeneration of the red nucleus. Cerebral cortical lesions varied from case to case and usually were less marked than thalamic neuronal changes.

Original languageEnglish (US)
Pages (from-to)359-366
Number of pages8
JournalAnnals of Neurology
Volume5
Issue number4
DOIs
StatePublished - Apr 1979

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Fingerprint Dive into the research topics of 'Thalamic degeneration in X‐chromosome–linked copper malabsorption'. Together they form a unique fingerprint.

  • Cite this