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- Vol.42 No.9
- CLINICA NEUROL, 42: 881－884, 2002
- Vol.42 No.9 contents
Brief Clinical Note
Facioscapulohumeral muscular dystrophy with sinus dysfunction
Hiroshi Shigeto, M.D.1), Takuhisa Tamura, M.D.2)*, Yasushi Oya, M.D.1), Masafumi Ogawa, M.D.1) and Mitsuru Kawai, M.D.1)
1)Department of Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry
2)Department of Medicine, National Sanatorium Higashi-Saitama Hospital (*Present address: Department of Internal Medicine, National Sanatorium Kawatana Hospital)
We report a 47-year-old man with facioscapulohumeral muscular dystrophy (FSHD) presenting with sinus dysfunction. He became unable to roll over and stand up at the age of 42, but he could still walk. Facial muscle involvement, scapular winging, asymmetrical involvement, funnel chest, and the absence of contractures were typical of FSHD. Electrocardiogram (ECG) and cardiac echogram showed the overload of both right atrium and ventricle. On Holter ECG, transient P wave inversion and P-P interval elongation (maximally 2.4 seconds) repeatedly appeared mainly during sleep. There was no bundle branch block, atrioventricular junctional rhythm, or increase of premature ventricular beats. Vital capacity was decreased (0.62 L, 16% of the predicted value). Arterial blood gas analysis showed hypercapnia and hypoxia which aggravated during sleep (PaCO2 87.3Torr, PaO2 41.5Torr). Sleep apnea was not observed. Intracardiac ECG was not performed and he died 2 weeks later. In FSHD, sinus node dysfunction may become distinct especially in the setting of respiratory failure.
(CLINICA NEUROL, 42: 881－884, 2002)
key words: facioscapulohumeral muscular dystrophy (FSHD), sinus dysfunction, cardiac dysfunction, Holter electrocardiogram
(Received: 10-Apr-02)
