Severe Outcome of Pharyngeal-Cervical-Brachial Pure Motor Axonal Neuropathy
L. Bonanni*, 1, V. Onofrj2, V. Scorrano3, M. Onofrj1, A. Thomas1
Identifiers and Pagination:Year: 2010
First Page: 1
Last Page: 4
Publisher ID: TONEUJ-4-1
Article History:Received Date: 29/4/2008
Revision Received Date: 20/7/2008
Acceptance Date: 29/7/2009
Electronic publication date: 29/1/2010
Collection year: 2010
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.
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.