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Poster (Scientific congresses and symposiums)
The Day George Avellis Met Miller Fisher – About the Unsual Presentation of an Anti-GQ1b IgG Antibody Syndrome
PHAN BA, Remy; Parmentier, Eric; LIEVENS, Isabelle et al.
2013The 65th Annual Meeting of the American Academy of Neurology
 

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Keywords :
Peripheral neuropathy; Miller-Fisher; Guillain-Barré
Abstract :
[en] Objective: OBJECTIVE: To describe the clinical, laboratory and electrophysiological features of a patient who presented an Avellis syndrome as the initial feature of Miller Fisher syndrome (MFS). Background: BACKGROUND: Anti-GQ1b Ig antibodies are associated with an increasing spectrum of neurological disorders, including MFS and Guillain-Barre syndrome (GBS). Design/Methods: DESIGN/METHODS: Clinical case description. Results: RESULTS: A 67-year old woman was seen for subacute dysphagia and dysphonia, preceded by rapidly worsening paresthesia of the extremities and face, with a history of upper respiratory tract infection two weeks before admission. Nasotracheal examination showed a left velopalatine and left vocal cord paresis. Twelve hours later, sensory ataxia appeared and deep tendon reflexes weakened. Diffuse paresis affecting predominantly the axial muscles developped. Oculomotricity was preserved. Brain MRI was normal, while EMG suggested a mild sensory neuropathy. Within hours, dysphagia worsened and dyspnea appeared, prompting ICU admission for airway support. She developed a proximal paresis and dysautonomia, global areflexia. CSF findings were unremarkable. IVIg were administered at a dose of 0.2g/kg per day during five days. Control EMG showed signs of polyradiculoneuropathy. She gradually recovered and was discharged at home after 32 days, with only a slight velopalatal paresis and a mild fatigue. Anti-ganglioside antibodies screen was positive for IgG-GM3, GD1b, GD3, GQ1b, GT1a and GT1b. In front of this clinical and biological picture, the diagnosis of atypical MFS was retained. Thirty day after discharge, both clinical and electrophysiological parameters were normalised. Conclusions: CONCLUSIONS: This case highlights that (i) MFS can show atypical presentation (here a pure Avellis syndrome, never reported in the context of the anti-GQ1b syndrome to our knowledge) and should be considered in front of an isolated impaired cranial nerve function, even in the absence of the classical triad of ophtalmoplegia, areflexia and ataxia, and (ii) that the boundaries between MFS and GBS are usually neater in textbooks than in real life.
Disciplines :
Neurology
Author, co-author :
PHAN BA, Remy ;  Centre Hospitalier Universitaire de Liège - CHU > Neurologie Sart Tilman
Parmentier, Eric  ;  Université de Liège - ULiège > Cert.univ.ECG
LIEVENS, Isabelle ;  Centre Hospitalier Universitaire de Liège - CHU > Neurologie Sart Tilman
MAQUET, Pierre  ;  Centre Hospitalier Universitaire de Liège - CHU > Neurologie Sart Tilman
MOONEN, Gustave  ;  Centre Hospitalier Universitaire de Liège - CHU > Neurologie Sart Tilman
Language :
English
Title :
The Day George Avellis Met Miller Fisher – About the Unsual Presentation of an Anti-GQ1b IgG Antibody Syndrome
Publication date :
March 2013
Event name :
The 65th Annual Meeting of the American Academy of Neurology
Event organizer :
the American Academy of Neurology
Event place :
San Diego, United States
Event date :
du 16 au 23 mars 2013
Audience :
International
Available on ORBi :
since 11 January 2013

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