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



L. Bonanni*, 1, V. Onofrj2, V. Scorrano3, M. Onofrj1, A. Thomas1
1 Department of Neurology, University G.D’Annunzio of Chieti-Pescara and Aging Research Center, Ce.S.I., “Gabriele d’Annunzio” University Foundation, Italy
2 University Ospedale San Raffaele, Milan, Italy
3 Neurofisiopatologia, Ospedale Civile di Pescara, Italy


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© Bonanni et al.; Licensee Bentham Open.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Department of Neurology, University G.D’Annunzio of Chieti-Pescara and Aging Research Center, Ce.S.I., “Gabriele d’Annunzio” University Foundation, Italy; E-mail: l.bonanni@unich.it


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.