Objective: Feeding dystonia is rare disorder represented by Spontaneous tongue protrusion and lip bites, classically present during eating, resulting in throwing out food bolus (1). The various articles that reported cases of feeding dystonia were secondary to neuroacantocythosis (1)

Background: This presentation reports the atypical case of a patient with Huntington’s disease wich is a fully penetrant neurodegenerative disease causes by a dominantly inherited CAG trinucleotide repeat expansion in the huntington gene on chromosome 4 by more than 35 repeats (2), revealed by feeding dystonia which represents a very suggestive sign of choreoacantocytosis with the other choreas of the lower half of the face, while the chorea of ​​the upper face is a hallmark of Huntington’s disease (3).Therefore alimentary dystonia has never been reported in Huntington’s disease

Method: We represent a clinical case of a 48-year-old patient with abnormal movements evolving from the age of 44,associated with cognitive disorders, without notion of parental cansanguinity, with several similar cases in the family over three successive generations (family genealogical tree Picture 1).

There is no history of exposure to neuroleptic drugs.

Results: The neurological examination showed: Feeding dystonia with dysarthia, associated with athetosic choreo movements to 4 limbs. Neuropsychological tests impossible to do because of language disorders.Brain MRI : cortico-subcortical atrophy (Picture 2).Normal CPK blood level.00 acanthocytes on blood smear. Absence of neuropathy on EMG. A normal copper balance.The genetic study of Huntington’s disease has objectified: an expansion of 43 +/- 3CAG.The diagnosis of huntington’s disease was retained. The patient was put on tetrabenazine.

Conclusion: Certainly feeding dystonia is a very suggestive sign of neuroacantocytosis but it is not a pathognomonic sign of the latter. To guide our investigations in neurodegenerative diseases, we must always verify the mode of genetic transmission.

References: (1) Ritu Shree MD, Sahil Mehta MD, DM , Venugopalan Y. Vishnu MD, DM Department of Neurology, Postgraduate .Institute of Medical Education and Research (PGIMER), Chandigarh, India. Feeding Dystonia. Video is part of ms.
(2) Amy Kim 1,† , Kathryn Lalonde 1,†, Aaron Truesdell 2,3, Priscilla Gomes Welter 4 , Patricia S. Brocardo 4 , Tatiana R. Rosenstock 5,6 and Joana Gil-Mohapel 1,2,*. New Avenues for the Treatment of Huntington’s Disease International journal of molicular science.4 August 2021.
(3) J.-B. Brunet de Courssou, W. Ekue, C. Nardin.Chorea-acanthocytosis and other Huntington’s disease phenocopies. Pratique Neurologique – FMC 2019;xx:1–4. © 2019 Elsevier Masson SAS.
(4) C. Simonin .Management of Huntington’s disease: From diagnosis to therapeutictrials. Pratique Neurologique – FMC 2016;7:1–
(5) Kara J. Wyant 1 & Andrew J. Ridder1 & Praveen Dayal. Huntington’s Disease-Update on Treatments. Curr Neurol Neurosci Rep (2017).
(6) Andreas R. Gantenbein, MD,1 Nathalie Damon-Perrie` re, MD,2 Jo¨ rg E. Bohlender, MD,3 Marie Chauveau, MD,2 Chrystelle Latxague, PhD,2 Marcelo Miranda, MD,4 Hans H. Jung, MD,1 * and Franc¸ ois Tison, MD, PhD2 ). Feeding Dystonia in McLeod Syndrome. Movement Disorders, Vol. 26, No. 11, 2011.
(7) Thara Larbi*, Meya Abdallah*, Saloua Hamzaoui*, Emna Gouider**, Mourad Ennafaa*, Ali Harmel*, Neziha Gouider***, Kamel Bouslama*, Mohamed Ben Dridi*, Skander M’rad*. Neuroacanthocytoses : un diagnostic à ne pas méconnaître. LA TUNISIE MEDICALE – 2011 ; Vol 89 (n°03).
(8) Gustavo L. Franklin1 *, Carlos Henrique F. Camargo1 , Alex T. Meira1 , Giovana M. Pavanelli 1 , Sibele S. Milano1 , Francisco B. Germiniani 1 , Nayra S. C. Lima2 , Salmo Raskin3 , Orlando Graziani Povoas Barsottini 4 , José Luiz Pedroso4 , Fernanda Aparecida Maggi 4 , Vitor Tumas 5 , Pedro Manzke de Carvalho5 , Ana Carolina de Oliveira6 , Bárbara Braga6 , Laura Cristina Souza6 , Rachel Paes Guimarães 6 , Luiza Gonzaga Piovesana6 , Íscia Teresinha Lopes-Cendes 6 , Paula Christina de Azevedo6 , Marcondes Cavalcante França Jr. 6 , Alberto Rolim Muro Martinez 6 and Hélio A. G. Teive1.Is Ataxia an inderstimated symptom in Huntigton’s disease.Frontiers in neurology.November 2020.

(9) .-B. Brunet de Courssou W. Ekue C. Nardin. Chorea-acanthocytosis and other Huntington’s disease phenocopies J. 2019 Elsevier Masson SAS.
(10) G. Coarelli a,b A. Durr b,c . Genetic factors and clinical variability in Huntington disease. Pratique Neurologique – FMC 2017;8:91–9.

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