ACUTE CEREBELLAR ATAXIA

 

ACUTE CEREBELLAR ATAXIA

 

DEFINITION:

A movement disorder characterized by the sudden onset of acute truncal ataxia 2-3 weeks after a viral infection.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • 1-3 years of age
  • risk factors:
    • viral infections (varicella, coxsackie, echovirus)
    • prodrome of 5-10 days prior to onset of acute ataxia

PATHOGENESIS:

1. Background:

  • viral infection -> autoimmune response -> cerebellum targetted with acute injury -> acute postinfectious cerebellitis

CLINICAL FEATURES:

1. Neurological Manifestations:

1.Truncal Ataxia

  • sudden onset
  • maximal at onset (worst within the first 24 hours)
  • ranges from mild to child unable to sit or stand
  • remits after a few days but complete recovery not until 3 weeks to 5 months
  • associated symptoms:
    • mild horizontal nystagmus (50% of cases)
    • dysarthria
    • nausea and vomiting
  • most patients have a complete recovery but some may have long-standing sequelae:
    • behavioural and speech disorders
    • ataxia and incoordination

INVESTIGATIONS:

1. Cerebral Spinal Fluid

  • slight lymphocytic pleocytosis (10-30 cells/ul)
  • slightly elevated protein

2. Others

  • normal imaging studies and drug screen

MANAGEMENT:

1. Supportive

  • no treatment available
  • condition is self-resolving in most cases

 

 

Pediatric Database - ACUTE CEREBELLAR ATAXIA

Pediatric Organization - Pedbase [at] Gmail.com