Ethnicity and Geographic Distribution of Pediatric Chronic Ataxia in Manitoba

Salman, Michael S.; Masood, Shaheen; Azad, Meghan B.; Chodirker, Bernard N.

doi:10.1017/s031716710001622x

Cited by 5 publications

(8 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Details on the methodology, epidemiology, ethnicity, and geographic distribution have been published on this cohort. 2,9 Ethical approval for the study was given by the Research Ethics Board of the University of Manitoba. Demographic and clinical information, diagnostic data, and neuroimaging reports were retrieved from the patients' hospital medical charts as described previously.…”

Section: Methodsmentioning

confidence: 99%

Recurrent Ataxia in Children and Adolescents

Salman

Klassen

Johnston

2017

Can. J. Neurol. Sci.

Self Cite

View full text Add to dashboard Cite

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.

show abstract

Section: Methodsmentioning

confidence: 99%

Recurrent Ataxia in Children and Adolescents

Salman

Klassen

Johnston

2017

Can. J. Neurol. Sci.

Self Cite

View full text Add to dashboard Cite

show abstract

“…Details on the epidemiology, ethnicity, and geographic distribution of the cohort have been published. 2,7 Ethical approval for the study was given by the Research Ethics Board of the University of Manitoba. Demographic information, diagnostic data, and neuroimaging reports were retrieved from the patients' hospital medical charts as described previously.…”

Section: Methodsmentioning

confidence: 99%

“…The 40 patients with the non-progressive course were divided into six mutually exclusive subgroups depending on the presence or absence of developmental delay, pyramidal tract signs, and epilepsy as described previously. 7 Their neuroimaging features were variable overall and also within their respective clinical subgroups. Some had normal imaging, while others had cerebellar hypoplasia, or progressive cerebellar atrophy despite the absence of clinical progression.…”

Section: General Neuroimaging Findingsmentioning

confidence: 99%

Neuroimaging Findings and Repeat Neuroimaging Value in Pediatric Chronic Ataxia

Salman

Chodirker

Bunge

2016

Can. J. Neurol. Sci.

View full text Add to dashboard Cite

Background: Chronic ataxia, greater than two months in duration, is encountered relatively commonly in clinical pediatric neurology practise and presents with diagnostic challenges. It is caused by multiple and diverse disorders. Our aims were to describe the neuroimaging features and the value of repeat neuroimaging in pediatric chronic ataxia to ascertain their contribution to the diagnosis and management. Materials and Methods: A retrospective charts and neuroimaging reports review was undertaken in 177 children with chronic ataxia. Neuroimaging in 130 of 177 patients was also reviewed. Results: Nineteen patients had head computed tomography only, 103 brain magnetic resonance imaging only, and 55 had both. Abnormalities in the cerebellum or other brain regions were associated with ataxia. Neuroimaging was helpful in 73 patients with 30 disorders: It was diagnostic in 9 disorders, narrowed down the diagnostic possibilities in 14 disorders, and revealed important but non-diagnostic abnormalities, e.g. cerebellar atrophy in 7 disorders. Having a normal magnetic resonance imaging scan was mostly seen in genetic diseases or in the early course of ataxia telangiectasia. Repeat neuroimaging, performed in 108 patients, was generally helpful in monitoring disease evolution and in making a diagnosis. Neuroimaging was not directly helpful in 36 patients with 10 disorders or by definition the 55 patients with unknown disease etiology. Conclusions: Normal or abnormal neuroimaging findings and repeat neuroimaging are very valuable in the diagnosis and management of disorders associated with pediatric chronic ataxia.RÉSUMÉ: Résultats en neuro-imagerie et utilité d'examens répétés en neuro-imagerie dans les cas d'ataxie chronique chez l'enfant. Contexte: L'ataxie chronique, dont la durée excède deux mois, est un phénomène relativement fréquent dans la pratique clinique en neurologie pédiatrique et représente un défi en matière de diagnostic. Elle est causée par plusieurs différents types de désordres. Notre objectif est ici de décrire les divers aspects de la neuro-imagerie et l'utilité d'examens répétés en neuro-imagerie dans les cas d'ataxie chronique chez l'enfant, et ce, afin de déterminer leur apport en ce qui concerne le diagnostic et le suivi de cette pathologie. Matériel et méthodes: Nous avons passé en revue des récapitulatifs graphiques ainsi que des rapports en neuro-imagerie concernant 177 enfants atteints d'ataxie chronique. Les examens en neuro-imagerie de 130 patients sur 177 ont également été analysés. Résultats: Dix-neuf patients n'ont passé qu'un examen de tomographie assistée par ordinateur; 103 d'entre eux n'ont passé qu'un examen d'imagerie par résonnance magnétique (IRM); enfin, 55 ont été soumis à ces deux examens. Des anomalies dans le cervelet et d'autres régions du cerveau ont été associées à l'ataxie. La neuro-imagerie s'est révélée utile dans le cas de 73 patients atteints de 30 désordres: ainsi, elle a permis le diagnostic formel de 9 désordres; elle a permis d'exclure des possibilités ...

show abstract

“…Patients were excluded if their ataxia was caused by primary brain tumor or by disorders solely involving the vestibular or peripheral nervous systems. Further details on the methodology have been published on this cohort [1,12]. Clinical variables were extracted from the patients' hospital charts including presenting symptoms, other symptoms, past medical history, family history, and physical exam findings.…”

Section: Methodsmentioning

confidence: 99%

Can Latent Class Analysis Be Used to Improve the Diagnostic Process in Pediatric Patients with Chronic Ataxia?

2016

Self Cite

View full text Add to dashboard Cite

Chronic ataxia is a relatively common symptom in children. There are numerous causes of chronic ataxia, making it difficult to derive a diagnosis in a timely manner. We hypothesized that the efficiency of the diagnostic process can be improved with systematic analysis of clinical features in pediatric patients with chronic ataxia. Our aim was to improve the efficiency of the diagnostic process in pediatric patients with chronic ataxia. A cohort of 184 patients, aged 0-16 years with chronic ataxia who received medical care at Winnipeg Children's Hospital during 1991-2008, was ascertained retrospectively from several hospital databases. Clinical details were extracted from hospital charts. The data were compared among the more common diseases using univariate analysis to identify pertinent clinical features that could potentially improve the efficiency of the diagnostic process. Latent class analysis was then conducted to detect unique patterns of clinical features and to determine whether these patterns could be associated with chronic ataxia diagnoses. Two models each with three classes were chosen based on statistical criteria and clinical knowledge for best fit. Each class represented a specific pattern of presenting symptoms or other clinical features. The three classes corresponded to a plausible and shorter list of possible diagnoses. For example, developmental delay and hypotonia correlated best with Angelman syndrome. Specific patterns of presenting symptoms or other clinical features can potentially aid in the initial assessment and diagnosis of pediatric patients with chronic ataxia. This will likely improve the efficiency of the diagnostic process.

show abstract

Ethnicity and Geographic Distribution of Pediatric Chronic Ataxia in Manitoba

Cited by 5 publications

References 21 publications

Recurrent Ataxia in Children and Adolescents

Recurrent Ataxia in Children and Adolescents

Neuroimaging Findings and Repeat Neuroimaging Value in Pediatric Chronic Ataxia

Can Latent Class Analysis Be Used to Improve the Diagnostic Process in Pediatric Patients with Chronic Ataxia?

Contact Info

Product

Resources

About