Details

Autor(en) / Beteiligte

• Antoine, Jean-Christophe
• Robert-Varvat, Florence
• Maisonobe, Thierry
• Créange, Alain
• Franques, Jérôme
• Mathis, Stéphane
• Delmont, Emilien
• Kuntzer, Thierry
• Lefaucheur, Jean-Pascal
• Pouget, Jean
• Viala, Karine
• Desnuelle, Claude
• Echaniz-Laguna, Andoni
• Rotolo, Francesco
• Camdessanché, Jean-Philippe

Titel

Identifying a therapeutic window in acute and subacute inflammatory sensory neuronopathies

Ist Teil von

• Journal of the neurological sciences, 2016-02, Vol.361, p.187-191

Ort / Verlag

Netherlands: Elsevier B.V

Erscheinungsjahr

2016

Quelle

MEDLINE

Beschreibungen/Notizen

• Abstract Background Patients with inflammatory sensory neuronopathy (SNN) may benefit from immunomodulatory or immunosuppressant treatments if administered timely. Knowing the temporal profile of neuronal loss in dorsal root ganglia will help to ascertain whether a final diagnosis may be reached before the occurrence of irreversible neuronal injuries. Thus, we addressed the evolution of neuronal loss in SNN by using sensory nerve action potentials (SNAPs) as a surrogate marker of neuron degeneration. Methods Eighty-six patients with acute/subacute inflammatory SNN (paraneoplastic, associated with dysimmune diseases, or idiopathic) were retrospectively studied. The monthly SNAP reduction was determined and normalized with the lower limit of normal. Disability progression was expressed by the modified Rankin score and correlated with SNAP reduction. Results The monthly SNAP reduction was similar in the four limbs although the median nerve was less severely affected. The monthly SNAP reduction was very severe within the first two months of evolution, began to slow down after seven months, and stabilized after ten months. It was tightly correlated with disability progression. Kaplan–Meier analysis showed that the median time until matching the diagnostic criteria of SNN was 8.5 months. Within this period, 42% of nerves remained excitable. Conclusions Developing treatment aiming at the stabilization of SNN is possible within the first 8 months of evolution. An improvement of the disease is possible if patients are treated within two months, which needs an early referral to an expert center and ENMG testing of the radial and ulnar nerves, which are most sensitive to changes.