Recurrent Ataxia in Children and Adolescents

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. 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.

Neurological vertigo in the emergency room in pediatric and adult age: systematic literature review and proposal for a diagnostic algorithm

“…of subjects Comments Lanzi G. et al, 1994 [ 12 ] Italy 12.5 to 24.2 years. old Prospective study Not specified 47 Vertigo is a rare symptom in patients with juvenile migraine Salman MS. et al, 2017 [ 13 ] Canada 6 to 32.75 years old Retrospective study 1991 to 2008 185 Vertigo/dizziness are often associated with chronic ataxia. Genetic, metabolic and inflammatory disorders should be considered in these patients Langhagen T. et al, 2013 [ 14 ] Germany 1.4 to 18 years old Retrospective study November 2009 to April 2012 168 Migraine-related vertigo is the most common cause of vertigo and dizziness in children and adolescents Ramantani G. et al, 2009 [ 15 ] Germany 9 years old Case report Not specified 1 Episodes of vertigo can be rarely associated with tuberous sclerosis Caldarelli M. et al, 2007 [ 16 ] Italy 2 months to 16 years Retrospective study January 1993 to August 2005 30 Vertigo is a complaint presenting in 30% of pediatric patients with symptomatic Chiari malformation Type I Kalashnikova LA.…”

“…The acute manifestation of vertigo in MS falls into two categories: acute vestibular syndrome as central form and BPPV as peripheral form [ 35 ]. It is then necessary an adequate differential diagnosis, that can be difficult in MS with atypical central signs [ 13 , 35 ]. Cochleo-vestibular dysfunction with vertigo have been described also in chronic-inflammatory-demyelinating neuropathy [ 64 ].…”

Neuroimaging in cerebellar ataxia in childhood: A review

“…Recurrent ataxia is defined as ataxia that recurs after complete or almost complete resolution of an episode of ataxia 158 . Inherited (genetic) recurrent ataxia may be caused by channelopathy, conventionally called episodic ataxia or metabolic syndromes such as mitochondrial diseases, organic acidopathies, and urea cycle defects, which is often termed intermittent ataxia 158 …”

“…Recurrent ataxia is defined as ataxia that recurs after complete or almost complete resolution of an episode of ataxia. 158 Inherited (genetic) recurrent ataxia may be caused by channelopathy, conventionally called episodic ataxia or metabolic syndromes such as mitochondrial diseases, organic acidopathies, and urea cycle defects, which is often termed intermittent ataxia. 158 Intermittent CA…”

Migraine with Brainstem Aura Associated with SCN2A Mutation: A Case Report

“…Diagnosis of this disease in a pediatric patient could be challenging due to anamnesis difficulties in this age group and occurrence similar clinical manifestations in other conditions such as episodic ataxia, multiple sclerosis, and other inflammatory and metabolic disorders like mitochondriopathies and glucose transporter type 1 (GLUT1) deficiency. 3 Quite frequently, the possibility of MBA is not even considered in many patients with recurrent and paroxysmal brainstem symptoms. The objective of this article is to report a case of pediatric MBA where there was significant diagnostic delay and describe possible genetic basis of this condition.…”