Myoclonus ataxia and refractory coeliac disease

Cerebellum & Ataxias
Ptolemaios G SarrigiannisMarios Hadjivassiliou

Abstract

Cortical myoclonus with ataxia has only rarely been reported in association with Coeliac Disease (CD). Such reports also suggested that it is unresponsive to gluten-free diet. We present detailed electro-clinical characteristics of a new syndrome of progressive cortical hyperexcitability with ataxia and refractory CD. At our gluten/neurology clinic we have assessed and regularly follow up over 600 patients with neurological manifestations due to gluten sensitivity. We have identified 9 patients with this syndrome. All 9 patients (6 male, 3 female) experienced asymmetrical irregular myoclonus involving one or more limbs and sometimes face. This was often stimulus sensitive and became more widespread over time. Three patients had a history of Jacksonian march and five had at least one secondarily generalised seizure. Electrophysiology showed evidence of cortical myoclonus. Three had a phenotype of epilepsia partialis continua at onset. There was clinical, imaging and/or pathological evidence of cerebellar involvement in all cases. All patients adhered to a strict gluten-free diet with elimination of gluten-related antibodies in most. However, there was still evidence of enteropathy in all, suggestive of refractory celiac disease....Continue Reading

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Citations

Dec 27, 2018·Expert Review of Neurotherapeutics·Vijayashankar ParamanandamAlfonso Fasano
Feb 27, 2019·Current Treatment Options in Neurology·Panagiotis Zis, Marios Hadjivassiliou
Sep 18, 2018·Current Neuropharmacology·Hiroshi MitomaChristiane S Hampe
Dec 23, 2017·CNS & Neurological Disorders Drug Targets·Marios Hadjivassiliou
Jul 21, 2017·Neurology·Marios HadjivassiliouNigel Hoggard
May 31, 2019·Cerebellum & Ataxias·Ptolemaios Georgios SarrigiannisMarios Hadjivassiliou
Nov 13, 2015·Cerebellum & Ataxias·Hiroshi MitomaJérôme Honnorat
Sep 1, 2018·Journal of Neurology·Thomas JulianPanagiotis Zis
Aug 12, 2018·Nutrients·Ana Vinagre-AragónMarios Hadjivassiliou
Jun 2, 2017·Continuum : Lifelong Learning in Neurology·Ronald F Pfeiffer
Sep 21, 2017·Frontiers in Neuroscience·Manuela PennisiGiuseppe Lanza
May 8, 2019·Movement Disorders Clinical Practice·Silvia JesúsBettina Balint
Apr 1, 2015·The Cerebellum·Hiroshi MitomaNobuhiro Yuki
Aug 6, 2020·Journal of Neurology·Panagiotis ZisMarios Hadjivassiliou
Jan 15, 2019·Movement Disorders Clinical Practice·Vijayashankar ParamanandamTim Lynch
Dec 1, 2020·Movement Disorders : Official Journal of the Movement Disorder Society·Emily SwinkinRichard A Wennberg
Jan 12, 2021·Movement Disorders Clinical Practice·Malco RossiBart van de Warrenburg
May 1, 2021·Nutrients·Marios HadjivassiliouPtolemaios G Sarrigiannis
May 5, 2021·Cerebellum & Ataxias·Lewis Kass-IliyyaMarios Hadjivassiliou
Jun 15, 2021·Cerebellum & Ataxias·Laurence NewrickMarios Hadjivassiliou
Apr 2, 2021·Case Reports in Gastroenterology·Youstina HannaZane Gallinger

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Methods Mentioned

BETA
biopsy
biopsies
PCR

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