Document Detail

Muscle Nogo-A expression is a prognostic marker in lower motor neuron syndromes.
MedLine Citation:
PMID:  17455292     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: 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. METHODS: 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. RESULTS: 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. INTERPRETATION: 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.
Pierre-François Pradat; Gaelle Bruneteau; Jose-Luis Gonzalez de Aguilar; Luc Dupuis; Natasa Jokic; François Salachas; Nadine Le Forestier; Andoni Echaniz-Laguna; Odile Dubourg; Jean-Jacques Hauw; Christine Tranchant; Jean-Philippe Loeffler; Vincent Meininger
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Annals of neurology     Volume:  62     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  2007 Jul 
Date Detail:
Created Date:  2007-08-02     Completed Date:  2007-10-12     Revised Date:  2008-01-22    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  15-20     Citation Subset:  IM    
Fédération des Maladies du Système Nerveux, Centre référent maladie rare SLA, Hôpital de la Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, 75651 Paris, France.
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MeSH Terms
Biopsy / methods
Disease Progression
Follow-Up Studies
Middle Aged
Motor Neuron Disease / metabolism*,  pathology*
Muscle, Skeletal / metabolism*
Myelin Proteins / metabolism*
Retrospective Studies
Reg. No./Substance:
0/Myelin Proteins; 0/Nogo protein
Comment In:
Ann Neurol. 2007 Dec;62(6):676   [PMID:  17702029 ]
Ann Neurol. 2007 Jul;62(1):1-2   [PMID:  17474106 ]
Ann Neurol. 2007 Dec;62(6):676-7; author reply 677   [PMID:  17894379 ]

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