RESEARCH ARTICLE

Severe Outcome of Pharyngeal-Cervical-Brachial Pure Motor Axonal Neuropathy

The Open Neurology Journal 29 Jan 2010 RESEARCH ARTICLE DOI: 10.2174/1874205X01004010001

Abstract

We present two further cases of the pharyngeal-cervical-brachial (PCB) form of GBS, with unfavourable outcome, showing dramatic dissociation between upper and lower body Symptoms.

Both patients showed rapidly progressive motor denervation with disappearance of Compound Muscle Action Potentials (CMAPs) in upper limbs muscles. Sensory Nerve Action Potentials (SNAPs) were instead normal. Normal reflexes, F waves and action potentials were elicited in lower limbs. Despite i.v. Immunoglobulin treatment no recovery was observed and both patients died within a year from onset of symptoms.

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