SOLITARY SPASTIC PARAPARESIA REVEALING KR ABBES DISEASE IN AN ADULT

A 25-year-old woman developed a slowly progressive spastic paraparesis a few weeks after delivering of her first child. A cerebral MRI showe d bilateral areas of hyperintensity in T-2 weighted images in the cent rum semi-ovale, infernal capsule, midbrain and occipital white matter. A profond deficiency of galactosylceramidase activity was demonstrate d in leukocytes (<5 p. 100 of controls) and in cultured fibroblasts. T he diagnosis of the adult form of Krabbe's disease was thus retained. We summarize the clinical symptoms and neuroradiological signs of this disorder and discuss the causative factors of its clinical heterogene ity.