Thalamic degeneration in X‐chromosome–linked copper malabsorption

Makoto Iwata, Asao Hirano, Joseph H. French

Research output: Contribution to journalArticlepeer-review

10 Scopus citations


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
Issue number4
StatePublished - Apr 1979

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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