Orthochromatic leukodystrophy with pigmented glial cells. An adult case with clinical-anatomical study

The case history is reported of a woman who died at the age of 36, at the conclusion of 11 years progressive neurological and psychiatric symptomatology. The anatomical and histological examination demonstrated an orthochromatic leukodystrophy with pigmented glial cells. Attention is drawn to the difficulty of finding these cells, which serve to differentiate between the unusual and the "simple" form of the disease. In the reported patient the pigmented cells were found around the vessels and only in specific cerebral locations. It is emphasized that the form is extremely rare (this is the tenth case so far reported). The significance of whether the iron content should be considered as an incidental or necessary finding is discussed. Systematic research for pigmented casts of this kind is taken to be important for all brains presenting a diffuse sclerosis after a protracted clinical course, mainly in adult patients.