Zur neurokutanen Melanose

Abstract

The authors report on a patient of 33 years of age suffering from neurocutaneous melanosis in whom two years after excision of a naevus pigmentosus et pilosus giganteus cellular proliferation of the melanoblasts in the area of the soft meninges was seen. This process led to the death of the patient within a few weeks' time despite intensive therapeutic measures, the manifestation being that of a generalised space-occupying growth in the CNS. Neuroradiological techniques can objectify the clinical pattern of signs of this very rare disease and may possibly enable an early diagnosis.

An unusual case of neurocutaneous melanosis

“…[3] reviewed the literature and found 39 cases with “definite” diagnosis based on pathological (biopsy, surgery, autopsy,) or MRI studies, the other reported cases being only “provisional”. We have found two other cases before 1991, one reported in German [4] the second in Portuguese [5], and, from 1991 onwards, at least 44 new cases [6‐30] of which ten were neurologically asymptomatic [11, 28]. Moreover, an MRI study of 43 neurologically asymptomatic patients demonstrated abnormalities in 14, of which 10 had T1 shortening indicative of melanotic rests within the brain parenchyma or the meninges [31].…”