Slowly progressive distal muscular atrophy of the bilateral upper limbs (O'Sullivan-McLeod syndrome) partially alleviated by intravenous immunoglobulin therapy.
We report a case of O'Sullivan-McLeod syndrome in a 59-year-old man, who had experienced slowly progressive weakening of both hands since he was 20 years of age. Mild hyperIgEemia and eosinophilia were present. Nerve conduction studies revealed reduced F wave-evoked frequencies for the median and ulnar nerves. Intravenous immunoglobulin (IVIG) at a dose of 400 mg/kg/day was given for 5 days. After IVIG, the muscle weakness of the distal upper extremities improved together with increased F wave-evoked frequencies. These effects lasted for a few months. These observations suggest that immune-mediated neural damage partially contributes to O'Sullivan-McLeod syndrome.
Kawano
Yuji
Y
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka.
Nagara
Yuko
Y
Murai
Hiroyuki
H
Kikuchi
Hitoshi
H
Ohyagi
Yasumasa
Y
Kira
Jun-ichi
J
D001132:Arm / Q000000981:diagnostic imaging*; D018450:Disease Progression; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D009133:Muscular Atrophy / Q000000981:diagnostic imaging* / Q000188:drug therapy*; D011859:Radiography; D013577:Syndrome
D016756:Immunoglobulins, Intravenous