Hearing loss associated with progressive ataxia (Lichtenstein-Knorr disease?) . Report of a sporadic case with peculiar neuroradiological findings
Striano, S.; Barbieri, F.; Meo, R.; Bilo, L.; Cirillo, S.
Acta Neurologica 11(5): 351-359
1989
ISSN/ISBN: 0001-6276 PMID: 2603781 Document Number: 345752
A sporadic case of hearing loss with ataxia, clinically suggesting Lichtenstein-Knorr disease, is described. Magnetic Resonance (MR) shows an involvement of the white matter, which appears diffusely reduced and abnormal, together with a peculiar tickening of the grey matter. The possible relationships with leukodystrophies and with generalized cortical dysplasias are discussed. To our knowledge, MR imaging has never been performed in Lichtenstein-Knorr disease.