Sudanophilic leucodystrophy: report of a case with tigroid demyelination of the centrum ovale

: A 17-year-old girl with negative familial and personal history developed a progressive neurologic disorder characterized by cortical amaurosis, metal deterioration and spastic tetraparesis. Death occurred one year after the onset of symptoms. Histopathologic examination of the C.N.S. showed the presence of extensive, discontinuous demyelination of the white matter of the centrum ovale, with preservation of perivascular myelin islets, associated with massive fat degradation, loss of axons and severe astroglial proliferation. This case belongs to the group of sudanophilic leucodystrophies, being an atypical case, a transition between pure sudanophilic leucodystrophy and the group of Pelizaeus-Merzbacher's disease.