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

Research output: Contribution to journalArticle

5 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)515-518
Number of pages4
JournalInternal Medicine
Volume46
Issue number8
DOIs
Publication statusPublished - Apr 17 2007

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Spinal Muscular Atrophy
Passive Immunization
Intravenous Immunoglobulins
Upper Extremity
Ulnar Nerve
Median Nerve
Neural Conduction
Muscle Weakness
Eosinophilia
Hand
Mcleod Type Neuroacanthocytosis

All Science Journal Classification (ASJC) codes

  • Internal Medicine

Cite this

Slowly progressive distal muscular atrophy of the bilateral upper limbs (O'Sullivan-McLeod syndrome) partially alleviated by intravenous immunoglobulin therapy. / Kawano, Yuji; Nagara, Yuko; Murai, Hiroyuki; Kikuchi, Hitoshi; Ohyagi, Yasumasa; Kira, Jun-Ichi.

In: Internal Medicine, Vol. 46, No. 8, 17.04.2007, p. 515-518.

Research output: Contribution to journalArticle

Kawano, Yuji ; Nagara, Yuko ; Murai, Hiroyuki ; Kikuchi, Hitoshi ; Ohyagi, Yasumasa ; Kira, Jun-Ichi. / Slowly progressive distal muscular atrophy of the bilateral upper limbs (O'Sullivan-McLeod syndrome) partially alleviated by intravenous immunoglobulin therapy. In: Internal Medicine. 2007 ; Vol. 46, No. 8. pp. 515-518.
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