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Abstract Details

Neuromuscular Disorders (Neuropathy and Myopathy) Associated with Late Onset of Stills Disease
Autoimmune Neurology
P9 - Poster Session 9 (5:30 PM-6:30 PM)

Adult-onset Still’s disease (AOSD) is a rare inflammatory disorder characterized by a classical triad of spiking fever, arthritis, and evanescent rash. Neurological involvement in AOSD is quite infrequent with reported prevalence less than 10%.


52-year-old male with medical history of AOSD, obstructive sleep apnea and bipolar disorder presented to neurology clinic for muscle fatigue and weakness. Patient was diagnosed with AOSD based on Yamaguchi criteria.

Physical examination was notable for reduced sensation to light touch and pinprick in four toes bilaterally excluding 5th toe. Normal muscle tone, strength, and reflexes were noted. Cranial nerve examination was normal. Extended myositis panel was negative. Patient underwent MRI of the pelvis which showed mild to moderate myositis to the gluteal muscles and anterior compartments in bilateral thighs. Patient underwent EMG which demonstrated mildly prolonged peak latency with normal amplitude in sensory nerve action potential (SNAP) in right median nerve.  Mildly slowed conduction velocity with normal distal latency and normal amplitude was also noted on compound muscle action potential (CMAP) in right median nerve. EMG of right lower extremity demonstrated increased recruitment of small amplitude short duration polyphasic MUAPs observed in the vastus lateralis and tibialis anterior muscles. The EMG findings were consistent with non-irritable myopathy. Given rest of the myositis work up was unremarkable, the presentation was likely related to neurological manifestations of ASOD. Patient was continued on prednisone ands weekly Tocilizumab.

AOSD is a rare disorder pathophysiology for which is not completely understood. Neurological manifestation in AOSD have been reported occasionally including aseptic meningitis, cranial nerve palsy, encephalitis, and cerebral infarction. Case report of Parkinsonian symptoms associated with AOSD have also been reported. We here report myositis during the course of AOSD which has not been previously reported and hope to enhance information regarding the spectrum of neurological involvement in AOSD
Shaweta Khosa, MD
Dr. Khosa has nothing to disclose.
Gurveer Singh Khosa, MBBS Dr. Khosa has nothing to disclose.
Shrikant Mishra, MD, FAAN Dr. Mishra has nothing to disclose.
Bhavesh Trikamji, MD (University of California Los Angeles) Dr. Trikamji has nothing to disclose.
Robert Freundlich, MD Dr. Freundlich has nothing to disclose.