The epidemiology of intermittent and chronic ataxia in children in Manitoba, Canada.

AIM: To determine the epidemiology of chronic ataxia in children in Manitoba, Canada. METHOD: A retrospective study using multiple sources and disease codes identified children (age 0-16y) with chronic ataxia (>2mo duration or recurrent episodes of ataxia) seen at Winnipeg Children's Hospital from 1...

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Publicado en:Developmental Medicine & Child Neurology Vol. 55; no. 4; pp. 341 - 348
Autores principales: Salman, Michael S, Lee, Esther J, Tjahjadi, Anindita, Chodirker, Bernard N
Formato: research Journal Article
Publicado: Wiley-Blackwell Apr2013
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Apr2013
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      pub: Wiley-Blackwell
      place: Malden, Massachusetts
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        10.1111/dmcn.12081
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        atl: The epidemiology of intermittent and chronic ataxia in children in Manitoba, Canada.
      aug:
        au:
          Salman, Michael S
          Lee, Esther J
          Tjahjadi, Anindita
          Chodirker, Bernard N
        affil: Section of Pediatric Neurology, Department of Pediatrics and Child Health, Children's Hospital, University of Manitoba, Winnipeg, MB.
      sug:
        subj:
          Angelman Syndrome Complications
          Ataxia Epidemiology
          Ataxia Etiology
          Ataxia Telangiectasia Complications
          Mitochondrial Diseases Complications
          Adolescence
          Age of Onset
          Angelman Syndrome Epidemiology
          Ataxia Mortality
          Ataxia Telangiectasia Epidemiology
          Child
          Chronic Disease
          Developmental Disabilities Epidemiology
          Developmental Disabilities Etiology
          Disease Progression
          Female
          Human
          Incidence
          Male
          Manitoba
          Mitochondrial Diseases Epidemiology
          Prevalence
          Retrospective Design
          Seizures Epidemiology
          Seizures Etiology
          Young Adult
          Adolescent: 13-18 years
          Child: 6-12 years
          Female
          Male
      ab: AIM: To determine the epidemiology of chronic ataxia in children in Manitoba, Canada. METHOD: A retrospective study using multiple sources and disease codes identified children (age 0-16y) with chronic ataxia (>2mo duration or recurrent episodes of ataxia) seen at Winnipeg Children's Hospital from 1991 to 2008. Patients with isolated peripheral nerve diseases, vestibular disorders, or brain tumors were excluded. RESULTS: We identified 184 patients (males=females; mean age 15y, SD 7y 8mo) with chronic ataxia. Median age at the presenting symptom onset was 1 year 3 months and at ataxia onset 3 years 1 month. Median duration of follow-up was 6 years 5 months. During the study period, the crude incidence rate was 5.77 in 10 000; the crude prevalence rate was 6.59 in 10 000; and the crude mortality rate 0.446 in 10 000. The most common presenting symptoms were developmental delay, ataxia, or seizures. The most common diagnoses (known in 129) were Angelman syndrome (n=16), ataxia telangiectasia (n=13), mitochondrial disease (n=9), Friedreich ataxia (n=7), stroke (n=7), and familial/genetic episodic ataxia (n=7). INTERPRETATION: Chronic ataxia is a relatively common early-presenting symptom in childhood. A specific diagnosis is possible in 70% of patients after extensive investigations. The mortality rate is relatively low and the disease burden is high with significant comorbidities including developmental delay and epilepsy.
      pubtype: Academic Journal
      doctype:
        research
        Journal Article
      ougenre: Article
    language: English
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