Muscle Nogo-a expression is a prognostic marker in lower motor neuron syndromes
Article first published online: 23 APR 2007
Copyright © 2007 American Neurological Association
Annals of Neurology
Volume 62, Issue 1, pages 15–20, July 2007
How to Cite
Pradat, P.-F., Bruneteau, G., Gonzalez de Aguilar, J.-L., Dupuis, L., Jokic, N., Salachas, F., Le Forestier, N., Echaniz-Laguna, A., Dubourg, O., Hauw, J.-J., Tranchant, C., Loeffler, J.-P. and Meininger, V. (2007), Muscle Nogo-a expression is a prognostic marker in lower motor neuron syndromes. Ann Neurol., 62: 15–20. doi: 10.1002/ana.21122
- Issue published online: 27 JUL 2007
- Article first published online: 23 APR 2007
- Manuscript Accepted: 9 FEB 2007
- Manuscript Revised: 6 FEB 2007
- Manuscript Received: 13 OCT 2006
- Association Française contre les Myopathies (AFM)
- Association pour la Recherche sur la Sclérose Latérale Amyotrophique (ARS)
- Fondation pour la Recherche Médicale (FRM)
- Fondation pour le Recherche sur le Cerveau (FRC)
- Association pour la Recherche et le Développement des Moyens de Lutte contre les Maladies Neurodégénératives
A proportion of patients with pure lower motor neuron syndrome (LMNS) progress to amyotrophic lateral sclerosis (ALS). Early detection of this progression is impossible, which delays the patient's access to treatment. Muscle expression of Nogo-A is a new candidate marker of ALS. We tested whether detection of Nogo-A in a muscle biopsy from patients with LMNS predicts progression to ALS.
Thirty-three patients who had undergone a muscle biopsy during the diagnostic workup of spinal LMNS were observed for 12 months. Nogo-A expression was measured by Western blot in muscle biopsy samples and compared with the final diagnosis.
Nogo-A expression was detected in 17 patients and was absent in 16 patients. The detection of Nogo-A in muscle biopsy samples from LMNS patients correctly identified patients who further progressed to ALS with 91% accuracy, 94% sensitivity, and 88% specificity. In patients who later developed typical ALS, Nogo-A may be detected as early as 3 months after the onset of symptoms.
Nogo-A test is able to identify ALS early in the course of the disease when diagnosis is difficult, requiring further progression. Use of the test in clinical practice may shorten the delay before introduction of neuroprotective drugs or inclusion in clinical trials. Ann Neurol 2007