title: Muscle Nogo-A expression is a prognostic marker in lower motor neuron syndromes.
abstract: 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.
  
  
  CONCLUSIONS
  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.
authors: 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.
authors: Pierre-François
authors: PF
authors: Pradat
authors: Gaelle
authors: G
authors: Bruneteau
authors: Jose-Luis
authors: JL
authors: Gonzalez de Aguilar
authors: Luc
authors: L
authors: Dupuis
authors: Natasa
authors: N
authors: Jokic
authors: François
authors: F
authors: Salachas
authors: Nadine
authors: N
authors: Le Forestier
authors: Andoni
authors: A
authors: Echaniz-Laguna
authors: Odile
authors: O
authors: Dubourg
authors: Jean-Jacques
authors: JJ
authors: Hauw
authors: Christine
authors: C
authors: Tranchant
authors: Jean-Philippe
authors: JP
authors: Loeffler
authors: Vincent
authors: V
authors: Meininger
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy / Q000379:methods; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000378:metabolism* / Q000473:pathology*; D018482:Muscle, Skeletal / Q000378:metabolism*; D009185:Myelin Proteins / Q000378:metabolism*; D000070798:Nogo Proteins; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D009185:Myelin Proteins; D000070798:Nogo Proteins; C000603199:RTN4 protein, human