PROGRESSIVE RUBELLA PANENCEPHALITIS (PRP)
 
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PROGRESSIVE RUBELLA PANENCEPHALITIS (PRP)

 

DEFINITION:

A slow viral infection of the brain characterized by chronic encephalitis caused by a persistent rubella infection.

EPIDEMIOLOGY:

  • incidence: rare - only 20 patients since first recognized in 1974
  • age of onset:
    • 8-19 years
  • risk factors:
    • M only
    • congenital rubella syndrome (18/20)
    • childhood rubella (German) ( 2/20)

PATHOGENESIS:

1. Pathology

  • similar to SSPE but without intracellular inclusion bodies
  • inflammation around blood vessels
  • glial nodules within the cortex; some loss of neurons

CLINICAL FEATURES:

1. Prodrome

  • congential rubella syndrome or childhood German measles

2. Clinical Course

1. 1st Clinical Stage - Behavioural Changes

  • insidious onset
  • subtle changes in behaviour and declining school work

2. 2nd Clinical Stage - Neurologic Changes

  • seizures - sometimes myoclonic
  • cerebellar ataxia
  • spastic weakness
  • retinopathy, optic atrophy
  • frank dementia leading to coma
  • spasticity and brainstem involvement with death in 2-5 years

INVESTIGATIONS:

1. Serology

  • IgG and IgM to rubella virus

2. CSF

  • elevated IgG, M fractions to rubella virus on oligoclonal electrophoresis

3. EEG

  • generalized slowing with occasional high-voltage activity
  • no "suppression-burst" pattern

4. Imaging Studies

  • 1. CT/MRI
    • cortical atrophy with increased ventricular size
    • cerebellar atrophy

    • MANAGEMENT:

    1. Supportive

    • anticonvulsants, physiotherapy, etc.

     

     

     

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