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Finger Cold-Induced Vasodilatation, Sympathetic Skin Response, and R—R Interval Variation in Patients With Progressive Spinal Muscular Atrophy

Department of Pediatrics, National Hospital Organization, Chiba Medical Center, Chiba, Japan, hideji-chiba{at}umin.ac.jp, Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba, Japan, hideji-chiba{at}umin.ac.jp

Yuzo Tanabe, MD

Division of Neurology, Chiba Children's Hospital, Chiba, Japan

Yasuo Hachiya, MD

Department of Pediatrics, Metropolitan Fuchu Medical Center for Severe Motor and Intellectual Disabilities, Tokyo, Japan

Eiko Otsuka, MD

Department of Pediatrics, Metropolitan Fuchu Medical Center for Severe Motor and Intellectual Disabilities, Tokyo, Japan

Satoko Kumada, MD

Department of Pediatrics, Metropolitan Fuchu Medical Center for Severe Motor and Intellectual Disabilities, Tokyo, Japan

Wakana Furushima, MD

Department of Pediatrics and Developmental Biology, Graduate School of Medicine, Tokyo Medical and Dental University, Tokyo, Japan

Jun Kohyama, MD

Department of Pediatrics and Developmental Biology, Graduate School of Medicine, Tokyo Medical and Dental University, Tokyo, Japan

Sumimasa Yamashita, MD

Division of Pediatric Neurology, Kanagawa Children's Medical Center, Yokohama, Japan

Jun-ichi Takanashi, MD

Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba, Japan

Yoichi Kohno, MD

Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba, Japan

To elucidate autonomic function in spinal muscular atrophy, we evaluated finger cold-induced vasodilatation, sympathetic skin response, and R—R interval variation in 10 patients with spinal muscular atrophy: 7 of type 1, 2 of type 2, and 1 of type 3. Results of finger cold-induced vasodilatation, sympathetic skin response, and R—R interval variation were compared with those of healthy children. Finger cold-induced vasodilatation was abnormal in 6 of 10patients with spinal muscular atrophy; it was normal in the healthy children. The mean sympathetic skin response latency and amplitude did not differ significantly from those of the healthy children. Amplitudes of sympathetic skin response to sound stimulation were absent or low in all six patients with spinal muscular atrophy. No significant difference was found in the mean R—R interval variation of patients with spinal muscular atrophy and healthy children. Results show that some patients with spinal muscular atrophy have autonomic dysfunction, especially sympathetic nerve hyperactivity, that resembles dysfunction observed in amyotrophic lateral sclerosis. (J Child Neurol 2005;20:871—875).

Journal of Child Neurology, Vol. 20, No. 11, 871-875 (2005)
DOI: 10.1177/08830738050200110301


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