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Ethnicity and Geographic Distribution of Pediatric Chronic Ataxia in Manitoba

Published online by Cambridge University Press:  23 September 2014

Michael S. Salman ,
Shaheen Masood ,
Meghan Azad  and
Bernard N. Chodirker
Michael S. Salman*
Affiliation:
Section of Pediatric Neurology, University of Manitoba, Winnipeg, Manitoba Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Manitoba
Shaheen Masood
Affiliation:
Section of Pediatric Neurology, University of Manitoba, Winnipeg, Manitoba
Meghan Azad
Affiliation:
Faculty of Medicine, University of Manitoba, Winnipeg, Manitoba
Bernard N. Chodirker
Affiliation:
Section of Genetics and Metabolism, University of Manitoba, Winnipeg, Manitoba Department of Pediatrics, University of Alberta, Edmonton, Alberta Canada
*
Section of Pediatric Neurology, Children's Hospital, AE 308, 820 Sherbrook Street, Winnipeg, Manitoba, R3A 1R9, Canada. Email: msalman@hsc.mb.ca.
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Abstract

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Background:

Genetic and environmental factors are important determinants of disease distribution. Several disorders associated with ataxia are known to occur more commonly in certain ethnic groups; for example, the disequilibrium syndrome in the Hutterites. The aim of this study was to determine the ethnic and geographic distribution of pediatric patients with chronic ataxia in Manitoba, Canada.

Methods:

We identified 184 patients less than 17 years-of-age with chronic ataxia during 1991-2008 from multiple sources. Their diagnosis, ethnicity and place of residence were determined following a chart review.

Results:

Most patients resided in Manitoba (N=177) and the majority in Winnipeg, the provincial capital. Thirty five Aboriginal, 29 Mennonite and 11 Hutterite patients resided in Manitoba. The latter two groups were significantly overrepresented in our cohort. Ataxia telangiectasia, mitochondrial disorders, and non-progressive ataxia of unknown etiology associated with pyramidal tracts signs and developmental delay were significantly more common in Mennonite patients. Four of five patients with neuronal migration disorders associated with chronic ataxia were Aboriginal. Few isolated disorders with chronic ataxia occurred in the 11 Hutterite patients including a Joubert syndrome related disorder.

Conclusions:

Three disorders associated with chronic ataxia were more prevalent than expected in Mennonites in Manitoba. Few rare disorders were more prevalent in the Hutterite and Aboriginal population. Further research is needed to determine the risk factors underlying these variations in prevalence within different ethnic groups. The unique risk factor profiles of each ethnic group need to be considered in health promotion endeavors.

Résumé

RÉSUMÉ

Ethnie et distribution géographique de l'ataxie chronique chez des patients d'âge pédiatrique au Manitoba.

Contexte:

Les facteurs génétiques et environnementaux sont des déterminants importants de la répartition d'une maladie dans une population. Il est bien connu que plusieurs maladies comportant de l'ataxie surviennent plus fréquemment dans certaines ethnies, comme par exemple le syndrome de déséquilibre chez les communautés huttériennes. Le but de cette étude était de déterminer la distribution ethnique et géographique des patients pédiatriques atteints d'ataxie chronique au Manitoba, Canada.

Méthode:

Nous avons identifié 184 patients de moins de 17 ans atteints d'ataxie chronique entre 1991 et 2008. Nos sources de renseignements étaient multiples. Le diagnostic, l'ethnie et le lieu de résidence étaient obtenus du dossier médical.

Résultats:

La plupart des patients résidaient au Manitoba (n = 177) et la majorité habitait Winnipeg, la capitale provinciale. Trente-cinq patients autochtones, 29 mennonites et 11 huttériens résidaient au Manitoba. Ces deux derniers groupes étaient significativement surreprésentés dans notre cohorte de patients. L'ataxietéléangiectasie, les maladies mitochondriales et l'ataxie non évolutive d'étiologie inconnue associée à des signes pyramidaux et à un retard du développement étaient significativement plus fréquentes chez les patients mennonites. Quatre des 5 patients atteints de troubles de la migration neuronale associé à une ataxie chronique étaient des Autochtones. Peu de maladies isolées avec ataxie chronique ont été observées chez les 11 Huttériens, incluant une maladie liée au syndrome de Joubert.

Conclusions:

Trois maladies comportant une ataxie chronique avaient une prévalence plus élevée que prévu chez les Mennonites du Manitoba. La prévalence de quelques maladies rares était plus élevée dans les populations huttérienne et autochtone. On devra procéder à des recherches plus poussées pour déterminer les facteurs de risque sous-jacents à ces variations de la prévalence au sein de différents groupes ethniques. Le profil de facteurs de risque unique à chaque groupe ethnique doit être pris en compte lors de campagnes de promotion de la santé.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 41 , Issue 1 , January 2014 , pp. 29 - 36
Copyright
Copyright © The Canadian Journal of Neurological 2014

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