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Related Experiment Videos

Cardiomyopathy in motor neuron diseases.

H-J Gdynia1, A Kurt, S Endruhn

  • 1University of Ulm, Department of Neurology, Oberer Eselsberg 45, 89081 Ulm, Germany. hans-juergen.gdynia@uni-ulm.de

Journal of Neurology, Neurosurgery, and Psychiatry
|April 15, 2006
PubMed
Summary

Cardiomyopathy is frequently observed in patients with motor neuron diseases (MND), including amyotrophic lateral sclerosis (ALS) and hereditary spastic paraplegias (HSP). Routine cardiac evaluations are recommended for all MND patients, even without traditional risk factors.

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Area of Science:

  • Neurology
  • Cardiology
  • Genetics

Background:

  • Myocardial involvement is uncommon in motor neuron diseases (MND).
  • Cardiac autonomic dysfunction is known in amyotrophic lateral sclerosis (ALS), but not in hereditary spastic paraplegias (HSP).
  • This study investigated MND patients with coexisting cardiomyopathy and no major cardiac risk factors.

Purpose of the Study:

  • To examine the prevalence and characteristics of cardiomyopathy in patients with ALS and HSP.
  • To determine if cardiomyopathy in MND patients is associated with known cardiovascular risk factors.
  • To assess the need for routine cardiac screening in MND patients.

Main Methods:

  • Study included four patients with definite ALS and two with probable HSP.
  • Comprehensive clinical, cardiological, electrophysiological, and laboratory data were analyzed.

Related Experiment Videos

  • Skeletal muscle biopsies were performed in two ALS patients.

    • Main Results:

    • All investigated MND patients presented with cardiomyopathy.
    • No major cardiovascular risk factors were identified, except for hyperlipoproteinemia and mild hypertension in one patient.
    • Secondary causes of cardiomyopathy, such as coronary heart disease, myocarditis, or mitochondrial damage, were ruled out.

    Conclusions:

    • The occurrence of cardiomyopathy may not be as rare as previously thought in MND.
    • An underlying pathophysiological cause for cardiomyopathy in these patients was not evident.
    • Routine cardiological evaluation is recommended for all patients diagnosed with MND.