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Author Spotlight: Deciphering the Role of ATM in Ataxia-Telangiectasia and the Associated Cerebellar Degeneration
Published on: December 27, 2024
Acute Ataxia in Childhood: 11-Year Experience at a Major Pediatric Neurology Referral Center
Kavita Thakkar1, Stephen M Maricich2, Gulay Alper3
1Department of Pediatrics, Division of Child Neurology, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA thakkarkp@upmc.edu.
Insights
Post-infectious cerebellar ataxia is the most common cause of acute pediatric ataxia, often following viral illness. Most children recover fully within 30 days, indicating a favorable prognosis for this condition.
Area of Science:
- Pediatric Neurology
- Neuroscience
Background:
- Acute ataxia in children presents a diagnostic challenge.
- Identifying the etiology is crucial for appropriate management and prognosis.
Purpose of the Study:
- To categorize the causes of acute ataxia in pediatric patients.
- To analyze the clinical features and outcomes of different ataxia etiologies.
Main Methods:
- Retrospective review of 120 pediatric neurology referrals over 11 years.
- Categorization of ataxia causes including post-infectious cerebellar ataxia, drug intoxication, and others.
- Analysis of patient demographics, clinical history, CSF findings, and MRI results.
Main Results:
- Post-infectious cerebellar ataxia was the most frequent diagnosis (59%), typically in children aged 1-6 years with preceding viral illness.
- Cerebrospinal fluid (CSF) pleocytosis occurred in 40%, while brain MRIs were normal.
- The majority of patients (91%) with post-infectious cerebellar ataxia achieved recovery within 30 days.
Conclusions:
- Post-infectious cerebellar ataxia is the leading cause of acute ataxia in childhood with a generally good prognosis.
- Differentiating post-infectious cerebellar ataxia from other acute ataxia presentations is clinically important.
- Early identification and supportive care are key for favorable outcomes in pediatric acute ataxia.
Abstract:
We categorized the causes of acute ataxia in the pediatric population-referred to the Division of Neurology-at a large, urban pediatric medical center. Of the 120 cases identified over the past 11 years, post-infectious cerebellar ataxia was the most commonly diagnosed (59%), followed by drug intoxication, opsoclonus-myoclonus ataxia syndrome, episodic ataxia, acute cerebellitis, cerebellar stroke, ADEM, meningitis, cerebral vein thrombosis, Leigh's disease, Miller-Fisher syndrome, and concussion. Among the patients with post-infectious cerebellar ataxia, 85% were 1-6 years old and all had a history of antecedent viral illness. CSF pleocytosis was present in 40% of patients; all had normal brain MRIs. The majority (91%) recovered within 30 days. We conclude that post-infectious cerebellar ataxia remains the most common cause of acute ataxia in childhood and that it carries a good prognosis. We also differentiate acute post-infectious cerebellar ataxia from other causes with similar presentations.
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