title: Hearing loss associated with progressive ataxia (Lichtenstein-Knorr disease?). Report of a sporadic case with peculiar neuroradiological findings.
abstract: 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.
authors.forename: S
authors.initials: S
authors.lastname: Striano
authors.forename: F
authors.initials: F
authors.lastname: Barbieri
authors.forename: R
authors.initials: R
authors.lastname: Meo
authors.forename: L
authors.initials: L
authors.lastname: Bilo
authors.forename: S
authors.initials: S
authors.lastname: Cirillo
mesh_terms: D002524:Cerebellar Ataxia / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D002648:Child; D004569:Electroencephalography; D005260:Female; D034381:Hearing Loss / Q000150:complications*; D006312:Hearing Loss, Bilateral / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D008279:Magnetic Resonance Imaging