Four Cases of Brown-Vialetto-Van Laere Syndrome from Iran: clinical and electrophysiologic findings

Brown-Vialetto-Van Laere syndrome (BVVLS) is a rareneurological disorder. We report our finding about fourpatients clinically and electrophysiologically diagnosed asBVVLS and denoted their clinical features with comparisonto previous reports. The first symptom was bilateral hearingloss and the onset of other cranial nerves involvementvaried between 0-15 years. Our patients represented somerare features like upper motor neuron signs in one patientand two cases interestingly developed some clinicalimprovement. This is the first report of BVVLS from Iran. Ourpatients which represent Caucasian population hadgenerally similar features like previously reported cases.