A case of Bickerstaff encephalitis with overlapping Gullian Barre syndrome in a pediatric patient treated with therapeutic plasma exchange

Abstract

Bickerstaff brain stem encephalitis (BBE) is a rare brainstem disorder characterized by acute onset of ophthalmoplegia, ataxia, and altered consciousness. Guillain Barre syndrome (GBS), Miller Fischer syndrome and BBE share certain similarities such as the presence of anti-ganglioside antibodies. The use of Therapeutic Plasma Exchange (TPE) has been reserved for severe to fulminant cases of BBE mostly as an 'off label' use. The role of TPE in the overlapping syndrome of BBE and GBS has not been explored much, especially in the paediatric population. Herein, we describe a case of 2-year-old male who presented with features of BBE and later evolved to an overlapping syndrome with BBE and GBS. A multi-disciplinary team managed the patient and TPE was initiated as a part of the treatment plan. Five cycles of TPE were done from day 24 after which the patient improved. In our case, TPE was used as rescue therapy in patients with BBE overlapping with GBS. The effectiveness of TPE can be further explored as a modality in such disorders.