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  • Title: Hearing loss associated with progressive ataxia (Lichtenstein-Knorr disease?). Report of a sporadic case with peculiar neuroradiological findings.
    Author: Striano S, Barbieri F, Meo R, Bilo L, Cirillo S.
    Journal: Acta Neurol (Napoli); 1989 Oct; 11(5):351-9. PubMed ID: 2603781.
    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.
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