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Recurrent Ataxia in Children and Adolescents

  • Michael S. Salman (a1) (a2), Samantha F. Klassen (a2) and Janine L. Johnston (a3) (a2)

Abstract

Background: Recurrent ataxia is encountered infrequently in clinical pediatric neurology practise and presents with diagnostic challenges. It is caused by several disorders. Our aims were to describe the epidemiology and clinical features in children with recurrent ataxia. Materials and Methods: A retrospective review was undertaken in 185 children with chronic ataxia, who presented during 1991 to 2008. Several databases were searched to ensure optimum ascertainment. Patients with brain tumors or isolated disorders of the peripheral nerves or vestibular system were excluded. Results: Recurrent ataxia was reported in 21 patients. Their age range was between 6 and 32.75 years (males=12). The crude period prevalence rate for the 18-year study period was 7.44/100,000. Eight patients had episodic ataxia and seven had inflammatory and metabolic disorders. In the rest the etiology was unknown. Many patients presented with ataxia, dizziness, and vertigo. The frequency and duration of the ataxic episodes varied from several per day to one every few months. Other clinical features included developmental delay and seizures. Neuroimaging in episodic ataxia was normal and abnormal in inflammatory or metabolic disorders. Acetazolamide provided symptomatic relief in patients with episodic ataxia, while steroids were beneficial in patients with an inflammatory etiology. One child with a metabolic disorder died. Conclusions: Recurrent ataxia is an uncommon presentation in children and mortality is rare. Genetic, metabolic, and inflammatory disorders should be considered in these patients. Neuroimaging is essential. Acetazolamide in selected patients provides good symptomatic relief.

Ataxie récurrente chez les enfants et les adolescents. Contexte: L’ataxie récurrente est observée peu fréquemment dans la pratique clinique en neurologie pédiatrique et pose des problèmes en matière de diagnostic. Elle est aussi causée par plusieurs troubles. Notre objectif est donc de décrire chez des enfants l’épidémiologie et les traits cliniques de ce trouble de coordination. Matériel et méthodes: Nous avons mené une analyse rétrospective chez 185 enfants atteints d’ataxie chronique qui ont fait l’objet d’une consultation entre 1991 et 2008. Des recherches ont également été conduites en lien avec plusieurs bases de données. Les patients présentant une tumeur au cerveau ou des troubles isolés du système nerveux périphérique ou du système vestibulaire ont été exclus. Résultats: Des cas d’ataxie récurrente ont été signalés chez vingt-et-un patients. Leur tranche d’âge allait de 6 à 32,75 ans (hommes = 12). Durant cette période d’étude de 18 ans, le taux de prévalence brut a été de 7,44/100 000. Huit patients ont souffert d’ataxie épisodique et sept d’entre eux de troubles inflammatoires et métaboliques. Pour ce qui est des autres patients, leur étiologie est demeurée inconnue. Beaucoup d’entre eux se sont présentés avec des symptômes d’ataxie, d’étourdissement et de vertige. La fréquence et la durée des épisodes ataxiques ont varié de plusieurs fois par jour à une fois tous les quelques mois. D’autres traits cliniques ont notamment inclus des retards de développement et des troubles convulsifs. Dans le cas de l’ataxie épisodique, les résultats en neuro-imagerie se sont révélés normaux ; ils sont toutefois apparus anormaux en ce qui regarde les troubles inflammatoires et métaboliques. L’acétazolamide a procuré un soulagement symptomatique aux patients souffrant d’ataxie épisodique alors que des stéroïdes ont été bénéfiques pour les patients chez qui on avait observé une étiologie inflammatoire. Enfin, mentionnons qu’un enfant atteint d’un trouble métabolique est décédé. Conclusions: L’ataxie récurrente demeure inhabituelle chez les enfants ; qui plus est, c’est très rarement qu’on en meurt. Dans le cas de ces patients, on doit envisager la présence de troubles génétiques, métaboliques et inflammatoires. Les techniques de la neuro-imagerie, elles, demeurent essentielles. Quant à l’acétazolamide, il procure à des patients sélectionnés un soulagement symptomatique efficace.

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Copyright

Corresponding author

Correspondence to: Michael S. Salman, Section of Pediatric Neurology, Children’s Hospital, AE 308, 820 Sherbrook Street, Winnipeg, MB, R3A 1R9, Canada. Email: msalman@hsc.mb.ca

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Keywords

Recurrent Ataxia in Children and Adolescents

  • Michael S. Salman (a1) (a2), Samantha F. Klassen (a2) and Janine L. Johnston (a3) (a2)

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