Neurological manifestations of xeroderma pigmentosum.

Xeroderma pigmentosum is a genodermatosis with neurological manifestations in approximately 18p. 100 of cases. Polymorphous and variably associated si gns are observed, progressing with the clinical course. The etiology of the neurological breach remains unknown. We report two siblings who had xerode rma pigmentosum with intellectual deficiency, a pyramidal, cerebellar and c ordonal syndrome, ophthalmoplegia, and axonal peripheral neuropathy. We dis cuss the epidemiological, clinical and electrophysiological aspects of the neurological breach in xeroderma pigmentosum.