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The epidemiology of intermittent and chronic ataxia in children in Manitoba, Canada
Michael S Salman1, Esther J Lee, Anindita Tjahjadi
1Section of Pediatric Neurology, Department of Pediatrics and Child Health, Children's Hospital, University of Manitoba, Winnipeg, MB, Canada. msalman@hsc.mb.ca
Insights
Chronic ataxia is a common childhood symptom, affecting 5.77 per 10,000 children. While a diagnosis is possible in most cases, it often presents with developmental delays and epilepsy, indicating a significant disease burden.
Area of Science:
- Pediatric Neurology
- Epidemiology
- Genetics
Background:
- Chronic ataxia in children presents a diagnostic challenge.
- Understanding its epidemiology is crucial for early intervention and management.
- Pediatric ataxia impacts development and quality of life.
Purpose of the Study:
- To determine the epidemiology of chronic ataxia in children in Manitoba, Canada.
- To identify common causes and presenting symptoms of childhood chronic ataxia.
- To assess the incidence, prevalence, and mortality rates of chronic ataxia in pediatric populations.
Main Methods:
- Retrospective study of children (0-16 years) with chronic ataxia (duration >2 months or recurrent episodes).
- Data collected from Winnipeg Children's Hospital between 1991 and 2008.
- Exclusion of patients with isolated peripheral nerve diseases, vestibular disorders, or brain tumors.
Main Results:
- 184 pediatric patients with chronic ataxia were identified.
- Crude incidence rate was 5.77/10,000; crude prevalence rate was 6.59/10,000.
- Common diagnoses included Angelman syndrome, ataxia telangiectasia, and mitochondrial disease; presenting symptoms included developmental delay, ataxia, and seizures.
Conclusions:
- Chronic ataxia is a relatively common early-presenting symptom in childhood.
- A specific diagnosis is achievable in approximately 70% of cases.
- The condition carries a high disease burden with significant comorbidities, despite a low mortality rate.
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.
