Slowly Progressive Distal Muscular Atrophy of the Bilateral upper Limbs (O'Sullivan-McLeod Syndrome) Partially Alleviated by Intravenous Immunoglobulin Therapy

Yuji Kawano; Yuko Nagara; Hiroyuki Murai; Hitoshi Kikuchi; Yasumasa Ohyagi; Jun-ichi Kira

doi:10.2169/internalmedicine.46.6221

CASE REPORTS

Slowly Progressive Distal Muscular Atrophy of the Bilateral upper Limbs (O'Sullivan-McLeod Syndrome) Partially Alleviated by Intravenous Immunoglobulin Therapy

Yuji Kawano, Yuko Nagara, Hiroyuki Murai, Hitoshi Kikuchi, Yasumasa Ohyagi, Jun-ichi Kira

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Keywords: allergy, atopy, IgE, OSullivan-McLeod syndrome, motor neuron disease

JOURNAL OPEN ACCESS

2007 Volume 46 Issue 8 Pages 515-518

DOI https://doi.org/10.2169/internalmedicine.46.6221

Details

Abstract

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.

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