Abstract Details

Title The Day George Avellis Met Miller Fisher - About the Unsual Presentation of an Anti-GQ1b IgG Antibody Syndrome

Topic Peripheral Nerve

Presentation(s) P01 - (-)

Poster/Presentation
Number 133

Objective

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.

Authors/Disclosures
PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
Pierre Maquet	No disclosure on file
Gustave C. Moonen, MD, PhD	No disclosure on file
Björn Tackenberg, MD, FAAN (F. Hoffmann-La Roche AG)	Dr. Tackenberg has received personal compensation for serving as an employee of F. Hoffmann-La Roche AG. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Roche. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Merck Serono. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novartis. Dr. Tackenberg has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for GILEAD. Dr. Tackenberg has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alexion. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Celgene. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Tackenberg has received personal compensation in the range of $50,000-$99,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Tackenberg has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Celgene. Dr. Tackenberg has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Roche. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Merck Serono. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen. Dr. Tackenberg has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Novartis. Dr. Tackenberg has received stock or an ownership interest from F. Hoffmann-La Roche AG. The institution of Dr. Tackenberg has received research support from Biogen. The institution of Dr. Tackenberg has received research support from Novartis. The institution of Dr. Tackenberg has received research support from Roche.

��ɫ��

��ɫ��