Neuroradiology and clinical aspects of Usher syndrome

• DOI: 10.1111/j.1399-0004.1996.tb02366.x
• Publication Date: 2010-07-19T09:41:32Z
• Authors: Marta L. Tamayo; Cesar Maldonado; Silvia L. Plaza; Gustavo M. Alvira; Gustavo E. Tamayo; Marta Zambrano; Jaime L. Frias; Jaime E. Bernal
• Keywords: Depression; Neuroradiology

We describe the neurological evaluation and MRI analysis of 30 patients, belonging to 16 families with Usher syndrome (US) type I II (US1 US2). In addition classic visual audiological abnormalities seen in these we observed abnormal gait 88.9% US1 66.7% US2 patients coordination 33.4% US1, 58.3% US2. Borderline mental retardation, depression or bipolar affective disorder were 16.7% 33.3% patients. showed cerebellar 50% US 1 75% but no clear correlation was between structural clinical findings. A pattern for classification is suggested.