What is the diagnosis and management of ataxia in children?

Diagnosis and Management of Ataxia in Children

MRI of the brain is the first-line diagnostic imaging for children with ataxia, as it provides superior detection of intracranial pathology compared to CT scanning. 1

Etiology and Classification

Childhood ataxia can be categorized based on temporal presentation:

• Acute ataxia (develops within 72 hours)

  • Most common causes:
    • Infectious/postinfectious disorders (33.6% of cases) 2, 1
    • Brain tumors (11.2% of cases) 2, 1
    • Toxic exposures/intoxications 2
    • Trauma-related (5% of cases) 2, 1
    • Migraine-related ataxia 2
    • Peripheral neuropathies 2
    • Vascular events (rare but critical) 2

• Chronic ataxia (lasting longer than 2 months) 2

  • Non-progressive: Often due to congenital brain malformations or perinatal injury 2
  • Progressive: Usually inherited disorders or acquired posterior fossa lesions 2

• Intermittent/episodic ataxia 2

  • May indicate migraine, benign positional vertigo, or metabolic disorders 2

Diagnostic Approach

Clinical Assessment

• Determine time course (acute, intermittent, chronic) 2
• Evaluate for extracerebellar symptoms (somnolence, encephalopathy, focal weakness, cranial nerve involvement) 2
• Screen for recent infections, especially varicella 3, 4
• Consider toxin exposure, especially in children under 6 years 5

Neuroimaging

• Brain MRI: First-line imaging for all forms of ataxia 1

  • Higher yield of abnormalities (63.9%) compared to CT (29.3%) 2
  • Most sensitive for detecting cerebellar inflammation, demyelination, and tumors 2

• Head CT: Consider when 2, 1

  • MRI is not immediately available
  • Acute trauma with concern for intracranial hemorrhage
  • Acute infectious causes requiring urgent intervention

• Specialized imaging: Based on clinical suspicion

  • MRI spine: For conditions with spinal cord involvement (e.g., ADEM) 1
  • MRA head/neck: For suspected posterior circulation stroke or vascular malformation 2, 1
  • MIBG scan and/or MRI chest/abdomen/pelvis: For opsoclonus-myoclonus-ataxia syndrome to evaluate for neuroblastoma 2, 1

Laboratory Testing

• Lumbar puncture: Consider in suspected infectious/inflammatory causes 4
  • Abnormal in approximately 39% of cases 4
• Toxicology screening: Important in acute presentations without clear infectious cause 2
• Genetic testing: For suspected hereditary ataxias 6

Management Approach

Management depends on the underlying etiology:

• Postinfectious cerebellar ataxia 1

  • Supportive care (most common approach)
  • Most cases are self-limiting with complete recovery within 2-3 weeks 5
  • Monitor for rare complications like hemicerebellitis 5

• Brain tumors 1

  • Neurosurgical consultation for potential resection
  • Further management based on tumor type and staging

• Toxic causes 1

  • Removal of offending agent
  • Supportive care until toxin clearance

• Traumatic causes 2

  • Management of intracranial injury according to type and severity
  • CT head without contrast is the initial imaging of choice 2
  • Consider CTA head/neck if vertebral artery dissection is suspected 2

Special Considerations

• Age considerations: Post-infectious cerebellar ataxia is more common in children under 5 years (86% of cases) 4

• Watchful waiting: May be appropriate for young children with 2, 4

  • Recent viral illness
  • No extracerebellar signs
  • Negative toxicology screen

• Red flags requiring immediate imaging 2

  • Age >3 years with symptoms >3 days
  • Presence of extracerebellar symptoms
  • Asymmetric neurological findings
  • Progressive worsening

• Prognosis: Most acute ataxias in children are benign and self-limiting, with complete recovery in the majority of cases 5, 4