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The diagnostic value of hyperammonaemia induced by the non-ischaemic forearm exercise test
  1. Jean-Yves Hogrel1,
  2. Jorien B E Janssen2,
  3. Isabelle Ledoux1,
  4. Gwenn Ollivier1,
  5. Anthony Béhin3,
  6. Tanya Stojkovic3,
  7. Bruno Eymard3,
  8. Nicol C Voermans2,
  9. Pascal Laforet3
  1. 1 Institute of Myology, Pitié-Salpêtrière Hospital, Paris, France
  2. 2 Department of Neurology, Radboud University Medical Centre, Nijmegen, The Netherlands
  3. 3 Paris-Est Neuromuscular Center, Institute of Myology, Pitié-Salpêtrière Hospital, Assistance Publique Hôpitaux de Paris, Paris, France
  1. Correspondence to Dr Jean-Yves Hogrel, Institut de Myologie, Groupe Hospitalier Pitié-Salpêtrière, 75651 Paris Cedex 13, France; jy.hogrel{at}


Aims The non-ischaemic forearm exercise test (NIFET) is used as a diagnostic tool for the screening of patients with exercise intolerance and for the diagnosis of various metabolic muscle disorders. The production of lactate and ammonia are generally analysed to guide the diagnosis. The aim of this retrospective study was to determine the level of ammonia rise, which can be suggestive of a muscle disease.

Methods This retrospective study involved 1440 patients who underwent NIFET. The clinical files of the patients with hyperammonaemia were methodically studied. Normal values were derived from 60 healthy controls.

Results 110 patients with hyperammonaemia were detected. They were classified as either having mild (between 94 and 141 µmol/L) or severe (more than 141 µmol/L) hyperammonaemia. Their diagnosis was studied with respect to the increase in lactate induced by the NIFET.

Conclusions Severe postexercise hyperammonaemia, even in the presence of a normal lactate response, is strongly suggestive of a muscle glycogen storage disease. Mild hyperammonaemia in the absence of other abnormalities is most likely non-specific and not indicative of a muscle disease.

  • neuromuscular

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  • J-YH and JBEJ contributed equally.

  • Handling editor Tahir Pillay.

  • Contributors J-YH and PL designed the study. AB, TS, BE, PL and NCV gathered and interpreted the clinical data. J-YH, IL and GO collected the exercise data. J-YH and JBEJ analysed the data and drafted the paper. All authors read, revised and approved the final manuscript.

  • Funding JBEJ was funded by Prinses Beatrix Spierfonds. This study was funded in part by the Association Française contre les Myopathies.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Statement All the raw data presented in this study are available to the corresponding author.