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Amyotrophy in prion diseases.

B B Worrall1, L P Rowland, S S Chin

  • 1Department of Neurology, Columbia University College of Physicians & Surgeons, New York, NY, USA. bbw9r@virginia.edu

Archives of Neurology
|January 14, 2000
PubMed
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Amyotrophy, muscle wasting, is an occasional feature of prion diseases like Creutzfeldt-Jakob disease. This review highlights motor neuron degeneration in prion disease cases, emphasizing spinal cord examination at autopsy.

Area of Science:

  • Neurology
  • Pathology
  • Prion Diseases

Background:

  • Amyotrophic lateral sclerosis (ALS) was historically linked to viral infections, with some Creutzfeldt-Jakob disease (CJD) patients exhibiting amyotrophy.
  • Interest in amyotrophy within prion diseases and its connection to ALS has waned over the past 15 years.

Purpose of the Study:

  • To investigate the presence and significance of amyotrophy in prion diseases.
  • To re-evaluate the potential link between prion diseases and amyotrophic lateral sclerosis.

Main Methods:

  • Systematic review of case reports of prion disease published after 1968.
  • Defined amyotrophy by clinical fasciculation and electromyographic findings.
  • Examined autopsy reports for evidence of motor neuron degeneration in the spinal cord.

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Main Results:

  • Amyotrophy was identified in 27 patients with proven prion disease (13 sporadic CJD, 2 familial CJD, 12 Gerstmann-Sträussler-Scheinker disease).
  • Clinical fasciculations were present in 23 patients, and 10 had electromyographic evidence of denervation.
  • Spinal cord examination in 8 patients revealed motor neuron loss in 6 and vacuolation in 1, supporting neurodegeneration.

Conclusions:

  • Amyotrophy is an occasional, prominent feature of Creutzfeldt-Jakob disease and other prion diseases.
  • This finding reinforces the importance of assessing lower motor neuron function in prion disease patients.
  • Thorough spinal cord examination during autopsy is crucial for diagnosing prion diseases with amyotrophy.