EBV and Concomitant Acute Motor and Sensory Axonal Neuropathy (AMSAN) in a Healthy 15-Year-Old Female.

Abstract

Acute motor and sensory axonal neuropathy (AMSAN) is a rare and severe form of acute axonal injury caused by immune damage to the axonal membrane. AMSAN is an axonal variant of GBS. GBS occurs from immune injury to the myelin sheath, axonal variants of GBS (AMSAN and AMAN) differ in that insult is to the axonal membrane. AMSAN is seldom seen, especially in pediatric and adolescent patients. Unlike AMAN (acute motor axonal neuropathy), which has been well described in literature to be secondary to C. Jejuni infection, there is no known etiology of AMSAN. Here we present a case of an otherwise healthy 15-year-old female who presented with new onset facial and bulbar weakness that rapidly progressed to functional paralysis requiring intubation. With no clear diagnosis and after failure of improvement with high dose steroids, IVIG and plasma exchange transfusion, diagnosis was finally made with electromyography and nerve conduction study. In addition, extensive lab work was completed and was only notable for primary acute EBV infection. This case represents a new presenting symptom of AMSAN, a unique finding of concomitant primary EBV infection, the possibility of primary EBV infection as the triggering event in AMSAN and stresses the importance of electromyography and nerve conduction study when evaluating patients with weakness.