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NEUROLOGY 2004;62:1891-1893
© 2004 American Academy of Neurology
Brief Communications

Progressive myopathy with circulating autoantibody against giantin in the Golgi apparatus

K. Sahashi, MD PhD*, T. Ibi, MD PhD, K. Ohno, MD PhD, K. Sahashi, MD, N. Nakao, MD PhD and H. Kondo, MD PhD

From the Department of Neurology (Drs. Sahashi*, Ibi, and Nakao), Aichi Medical University, Japan; Department of Neurology (Dr. Ohno), Mayo Clinic, Rochester, MN; Department of Neurology (Dr. Sahashi), Nagoya First Red Cross Hospital, Aichi, Japan; and Department of Gene Therapy (Dr. Kondo), University of Cambridge, UK.

Address correspondence and reprint requests to Dr. K. Sahashi*, Department of Neurology, Aichi Medical University School of Medicine, 21 Karimata, Yazako, Nagakute, Aichi 480-1195, Japan; e-mail: sahashi{at}amugw.aichi-med-u.ac.jp

A woman aged 59 years with adult-onset progressive myopathy had anti-Golgi (giantin) autoantibody in the serum. Limb-muscle biopsy revealed chronic myopathy with paucity of cellular reactions and reduced immunostaining for {alpha}-dystroglycan. The similarity of the current patient with cases of hereditary {alpha}-dystroglycanopathies (Fukuyama-type congenital muscular dystrophy, Walker-Warburg syndrome, muscle-eye-brain disease, congenital muscular dystrophy type 1C, and limb-girdle muscular dystrophy type 2I) suggests that the Golgi apparatus is the target organelle in a subset of myopathies.

Received August 28, 2003. Accepted in final form January 27, 2004.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the May 25 issue to find the title link for this article




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