LEUKODYSTROPHY INCIDENCE IN GERMANY

Through a survey of all departments of pediatrics, neurology and neuro pathology in Germany, we calculated the incidence of all major forms o f leukodystrophy. Only diagnoses based on specific biochemical tests i n association with typical findings and/or neuroradiologically proven white matter involvement were accepted, In accordance with these stric t criteria, 617 cases of leuko-dystrophy were found (incidence of all forms: app. 2.0/100,000), Minimal incidence was estimated at 0.8/100,0 00 for adrenoleugodystrophy/adrenomyeloneuropathy (ALD/AMN), 0.6/100,0 00 for metachromatic leukodystrophy (MLD), and 0.6/100,000 for Krabbe disease. Thus ALD/AMN is apparently underdiagnosed in Germany. A consi derable proportion of leukodystrophies could not be classified in spit e of adequate diagnostic procedures in experienced centers. (C) 1997 W iley-Liss, Inc.