Continuous Spectrum of Pharyngeal-Cervical-Brachial Variant of Guillain-Barré Syndrome

Abstract

Guillain-BarrÉsyndrome (GBS) is characterized by acute onset of tetraparesis and areflexia,¹ and Fisher syndrome (FS) is characterized by acute onset of ophthalmoplegia, ataxia, and areflexia.² Bickerstaff brainstem encephalitis (BBE) may be considered a central nervous system subtype of FS associated with consciousness disturbance.³ Ropper⁴ described 3 patients who experienced acute progression of oropharyngeal, neck, and shoulder weakness. The signs were facial palsy, blepharoptosis, absence of sensory disturbance, and preserved tendon jerk in the legs. Because of elevated protein levels in the cerebrospinal fluid, as well as electrophysiological findings (a denervation pattern and decreased conduction velocity in peripheral nerves), Ropper⁴ speculated that these patients had a GBS variant, which he called “pharyngeal-cervical-brachial weakness” (PCB). Ropper⁵ described an additional patient who had eye movement limitation, generalized areflexia, severe ataxia, and oropharyngeal, neck, and shoulder weakness. He concluded that this was a case of FS combined with PCB, but because the patient also showed consciousness disturbance, the diagnosis may have been BBE combined with PCB. A patient with GBS who manifested tetraplegia at onset and PCB in the recovery phase has been described.⁶ These limited clinical observations suggest that PCB, GBS, FS, and BBE form a continuous spectrum.