Science and Education Publishing American Journal of Medical Case Reports 2374-216X 3 12 2015 12 16 Atypical Myositis Presenting with Peripheral Neuropathy 419 421 EN Imene Boukhris Internal Medicine Department, University hospital of Charles Nicolle, Tunis, Tunisia Samira Azzabi Ines kechaou Eya Chérif Anis Hariz Chékib Kooli Lamia Ben Hassine Narjes Khalfallah AJMCR201531210 10.12691/ajmcr-3-12-10 2015 8 24 2015 9 27 2015 12 14 Necrotizing Autoimmune Myositis (NAM) is an Acute autoimmune disease of the proximal skeletal muscle. The increased rate of morbidity and mortality in NAM is related to the severety and extant of muscle weakness. Patients with NAM develop antibodies to a specific signal recognition particle (anti-SRP ab). Peripheral neuropathy is extremely rare during the course of the disease. We report the case of a 35 y old male, hospitalized for severe proximal and distal muscle weakness with positive anti-SRB ab and concomitant peripheral neuropathy. The outcome was severe respiratory failure and death.