The rigid spine syndrome and Emery-Dreifuss muscular dystrophy.

Abstract

We present five patients, three of whom suffered from a rigid spine syndrome and two from Emery-Dreifuss muscular dystrophy. One patient with rigid spine syndrome showed a nonprogressive course, normal cardiac rhythm and mild myopathic changes in muscle histology, while in the other two patients there was a rapidly progressive course, sinus tachycardia without cardiac conduction defects and marked fiber necrosis in muscle histology. The two patients suffering from Emery-Dreifuss muscular dystrophy showed a very mildly progressing course, myopathic changes in muscle histology, cardiac conduction defects and deviations in electrocardiograms. These findings show that rigid spine syndrome can be distinguished from Emery-Dreifuss muscular dystrophy.

• Authors: I Goto, S Ishimoto, T Yamada, H Hara, Y Kuroiwa
• Journal: Clinical neurology and neurosurgery (Clin Neurol Neurosurg) Vol. 88 Issue 4 Pg. 293-8 ( 1986) ISSN: 0303-8467 [Print] Netherlands
• PMID: 3802686 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Topics

• Adolescent
• Adult
• Diagnosis, Differential
• Electrocardiography
• Humans
• Male
• Muscles (pathology)
• Muscular Dystrophies (diagnosis, pathology, physiopathology)
• Spinal Diseases (diagnosis, pathology, physiopathology)
• Syndrome