Adult Onset Scapuloperoneal Myopathy

Overview

Journal J Neurol Neurosurg Psychiatry

Publisher BMJ Publishing Group

Specialties Neurology
Neurosurgery
Psychiatry

Date 1975 Oct 1

PMID 1202162

Citations 11

Authors

P K Thomas

G D Schott

J A Morgan-Hughes

Affiliations

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Abstract

Six cases are described of muscle weakness and wasting of scapuloperoneal distribution with an onset in early adult or middle life and a relatively benign progression. One case also showed mild facial weakness. Four cases were probably sporadic but in two, a mother and daughter, autosomal dominant inheritance was likely. Electromyographic studies demonstrated myopathic features in all, and this was confirmed by muscle biopsy in five. Electrocardiographic abnormalities were present in three cases, but their significance is uncertain. It is considered that adult onset scapuloperoneal myopathy constitutes a clinically distinct condition.

Citing Articles

Distal myopathies.

Dimachkie M, Barohn R Neurol Clin. 2014; 32(3):817-42, x.

PMID: 25037092 PMC: 4109660. DOI: 10.1016/j.ncl.2014.04.004.

Neurogenic scapuloperoneal syndrome in childhood.

Mercelis R, Demeester J, Martin J J Neurol Neurosurg Psychiatry. 1980; 43(10):888-96.

PMID: 7441268 PMC: 490709. DOI: 10.1136/jnnp.43.10.888.

Scapuloperoneal syndrome with cardiomyopathy: report of a family with autosomal dominant inheritance and unusual features.

Chakrabarti A, Pearce J J Neurol Neurosurg Psychiatry. 1981; 44(12):1146-52.

PMID: 7334411 PMC: 491236. DOI: 10.1136/jnnp.44.12.1146.

Emery-Dreifuss syndrome.

Petty R, Thomas P, Landon D J Neurol. 1986; 233(2):108-14.

PMID: 3701378 DOI: 10.1007/BF00313856.

Benign congenital muscular dystrophy with autosomal dominant heredity: problems of classification.

Schmalbruch H, KAMIENIECKA Z, Fuglsang-Frederiksen A, TROJABORG W J Neurol. 1987; 234(3):146-51.

PMID: 3585421 DOI: 10.1007/BF00314133.

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