HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS REGISTER		Author Keyword(s) Vol Page [Advanced]

This Article Full Text Full Text (PDF) Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Add article to my folders Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Gdynia, H-J Articles by Sperfeld, A-D Search for Related Content PubMed PubMed Citation Articles by Gdynia, H-J Articles by Sperfeld, A-D

Cardiomyopathy in motor neuron diseases

University of Ulm, Department of Neurology, Oberer Eselsberg, Ulm, Germany

Correspondence to:
Correspondence to:
Dr H-J Gdynia
University of Ulm, Department of Neurology, Oberer Eselsberg 45, 89081 Ulm, Germany; hans-juergen.gdynia{at}uni-ulm.de

ABSTRACT
Objective: Myocardial involvement in motor neuron diseases (MND) is an uncommon feature. In amyotrophic lateral sclerosis (ALS) abnormalities of the autonomic nervous system affecting cardiac function have been described, for the hereditary spastic paraplegias (HSP) comparable manifestations are unknown. This study observed ALS and HSP patients with coexisting cardiomyopathy without major cardial risk factors.

Methods: Four patients with definite ALS and two pHSP patients. In all patients detailed clinical, cardiological, electrophysiological and laboratory data were analysed. In two ALS patients skeletal muscle biopsy was performed.

Results: In all investigated MND patients cardiomyopathy was present. Beside hyperlipoproteinaemia and mild hypertension in one case, none of the patients showed major cardiovascular risk factors. There was no evidence for a secondary cause of cardiomyopathy like coronary heart disease, myocarditis, or mitochondrial damage mimicking MND.

Conclusion: This report could not conclude that the occurrence of cardiomyopathy is rare logically. Although an underlying pathophysiological cause was not obvious, it is proposed that in all MND patients a routine cardiological evaluation should be performed.

Abbreviations: ALS, amyotrophic lateral sclerosis; CM, cardiomyopathy; HSP, hereditary spastic paraparesis; pHSP, pure hereditary spastic paraparesis; MND, motor neuron disease; SMA, spinal muscular atrophy

Keywords: amyotrophic lateral sclerosis; hereditary spastic paraparesis; spinal muscular atrophy; cardiomyopathy