Author/Authors :
Yadegari, Samira Department of Neurology - Tehran University of Medical Sciences, Tehran , Ghorbani, Askar Department of Neurology - Tehran University of Medical Sciences, Tehran , Ansari Dezfouli, Mitra School of Biology - University College of Science - University of Tehran, Tehran , Nafissi, Shahriar Department of Neurology - Tehran University of Medical Sciences, Tehran
Abstract :
Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare
neurological disorder. We report our finding about four
patients clinically and electrophysiologically diagnosed as
BVVLS and denoted their clinical features with comparison
to previous reports. The first symptom was bilateral hearing
loss and the onset of other cranial nerves involvement
varied between 0-15 years. Our patients represented some
rare features like upper motor neuron signs in one patient
and two cases interestingly developed some clinical
improvement. This is the first report of BVVLS from Iran. Our
patients which represent Caucasian population had
generally similar features like previously reported cases.
