Pharyngeal-cervical-brachial syndrome: An atypical case of Guillain-Barre syndrome with anti-sulfatide IgM antibodies

Abstract

Pharyngeal-cervical-brachial (PCB) syndrome, a rare form of Guillain-Barre syndrome (GBS) is defined by rapidly progressive  oropharyngeal and cervicobrachial weakness associated with areflexia in the upper limbs. We report the observation of a 30-year-old adult male who presented, 10 days after acute diarrhea, oculomotor and severe bulbar palsy followed by cervicobrachial diparesis with  hyporeflexia in the upper limbs. Cerebral magnetic resonance imaging (MRI), the otorhinolaryngological examination, the cerebrospinal fluid (CSF) analysis, the prostigmine test were normal as was the electromyography (EMG) performed on day 10. Laboratory test results such as antiacetylcholin receptor antibodies were negative. At the EMG performed on day 18, there were some signs of demyelination; the  diagnosis of PCB syndrome was made. Determination of antiganglioside antibodies revealed positive Anti-sulfatide IgM antibodies. After administration of immunoglobulin therapy, symptoms got better with a complete recovery in 6 weeks.

French title: Syndrome pharyngo-cervico-brachial : Un cas  atypique de syndrome de guillainbarre avec des anticorps Anti-Sulfatide IgM