Rinsho Shinkeigaku (Clinical Neurology)

Brief Clinical Note

McArdle's disease with insulin resistance caused by obesity

Nobuya Maeda, M.D., Fumio Kanda, M.D. Ph.D., Kenichiro Nakazawa, M.D., and Kazuo Chihara, M.D. Ph.D.

Divisions of Endocrinology/Metabolism, Neurology and Hematology/Oncology,
Department of Clinical Molecular Medicine, Kobe University Graduate School of Medicine

We reported a 29-year-old woman with McArdle's disease accompanied with insulin resistance. The patient was referred to our hospital because of muscle tenderness, swelling and weakness of lower extremities, and elevated serum CK level. Ischemic forearm exercise test showed no elevation in serum lactate and pyruvate levels. Muscle biopsy revealed significant reduction in phosphorylase activity both histochemically and biochemically. Pre-administration of glucagon had no effect on serum lactate and pyruvate levels after ischemic forearm exercise test while serum glucose elevated. The glucose clamp test confirmed insulin resistance. There was no reduction in number of insulin receptor or activity of tyrosine kinase. Her bodyweight was 78.4 kg and body mass index (BMI) was high as 32.0. Her obesity might be a causative factor of insulin resistance.

(CLINICA NEUROL, 42: 632|634, 2002)
key words: McArdle's disease, Ischemic forearm exercise test, insulin resistance, glucose clamp

(Received: 4-Jan-02)