Facioscapulohumeral muscular dystrophy with sinus dysfunction

Hiroshi Shigeto, Takuhisa Tamura, Yasushi Oya, Masafumi Ogawa, Mitsuru Kawai

Research output: Contribution to journalArticlepeer-review

4 Citations (Scopus)


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 (0.62L. 16% of the predicted value). Arterial blood gas analysis showed hypercapnia and hypoxia which aggravated during sleep (PaCO2 87.3 Torr, PaO2 41.5 Torr). 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.

Original languageEnglish
Pages (from-to)881-884
Number of pages4
JournalClinical Neurology
Issue number9
Publication statusPublished - Sept 1 2002
Externally publishedYes

All Science Journal Classification (ASJC) codes

  • Clinical Neurology


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