Myoadenylate deaminase deficiency: absence of correlation with exercise intolerance in 452 muscle biopsies

J Neurol. 1987 Aug;234(6):385-9. doi: 10.1007/BF00314082.

Abstract

A histochemical assay was routinely performed of myoadenylate deaminase (MAD) in muscle biopsy specimens. MAD was absent in 13 cases, i.e. 2.9% of the specimens. In 10 cases the deficiency was confirmed biochemically. The diagnoses in the 13 patients were: polyneuropathy (n = 5), infantile spinal muscular atrophy (n = 3), congenital myopathy with type 2 fibre atrophy, facioscapulohumeral myopathy, polymyositis, myotonic dystrophy and hyperornithinaemia with gyrate atrophy of the retina. In contrast, 35 unrelated patients presenting with exercise-related muscle cramps or pains showed normal histochemical MAD activity. The biopsy specimens in all of these patients were essentially normal and in none of them was the diagnosis of a neuromuscular disease made. The results failed to confirm the association of MAD deficiency with aches, cramps and pains or exertional myalgia.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • AMP Deaminase / deficiency*
  • Adolescent
  • Adult
  • Aged
  • Biopsy
  • Child
  • Female
  • Histocytochemistry
  • Humans
  • Male
  • Middle Aged
  • Muscles / enzymology*
  • Muscles / pathology
  • Muscular Diseases / enzymology
  • Muscular Diseases / etiology*
  • Muscular Diseases / pathology
  • Nucleotide Deaminases / deficiency*
  • Physical Exertion*

Substances

  • Nucleotide Deaminases
  • AMP Deaminase