[Neuropathology of trichopoliodystrophy (Menkes' disease). Anatomoclinical observation]

The authors describe an anatomo-clinical case of trichopoliodystrophy in many ways similar to cases already published. The original features of the case are as follows: the ultrastructure of the duodenal mucosa is normal; the endoplasmatic reticulum of the putaminal neurons contains many-layered lamellar structures probably composed of protein; type II fibres predominate in the striated skeletal muscle suggesting abnormal neuromuscular maturation.