RT Journal Article
SR Electronic
T1 Protracted disease course after deafness in a man with Brown-Vialetto-Van Laere syndrome
JF Neurosciences Journal
JO Neurosciences (Riyadh)
FD Prince Sultan Military Medical City
SP 253
OP 255
VO 17
IS 3
A1 Samira Yadegari
A1 Keyvan Razmjoo
A1 Shahriar Nafissi
YR 2012
UL http://nsj.org.sa/content/17/3/253.abstract
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.