PT  - JOURNAL ARTICLE
AU  - Yadegari, Samira
AU  - Razmjoo, Keyvan
AU  - Nafissi, Shahriar
TI  - Protracted disease course after deafness in a man with Brown-Vialetto-Van Laere syndrome
DP  - 2012 Jul 01
TA  - Neurosciences Journal
PG  - 253--255
VI  - 17
IP  - 3
4099  - http://nsj.org.sa/content/17/3/253.short
4100  - http://nsj.org.sa/content/17/3/253.full
SO  - Neurosciences (Riyadh)2012 Jul 01; 17
AB  - Brown-Vialetto-Van Laere syndrome (BVVLS) is a rare type of motor neuron disease, characterized by bilateral deafness, and multiple cranial nerve involvement. The disease is more frequent in females, but rapid deterioration was attributed to male gender or early onset. Herein, we report a man with BVVLS who had a longer than expected interval between onset of deafness and other motor symptoms that culminated to diagnosis. Six years later, he had gradual worsening and death. Along with potential new developing therapeutic options, this case emphasizes considering the diagnosis of BVVLS especially after a long period of stability, even in men.