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Progressive Rubella Panencephalitis

By Brenda L. Tesini, MD, Assistant Professor of Medicine and Pediatrics, Division of Infectious Diseases; Associate Hospital Epidemiology, University of Rochester School of Medicine and Dentistry; Strong Memorial Hospital and Golisano Children's Hospital, University of Rochester Medical Center

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Progressive rubella panencephalitis is a neurologic disorder occurring in children with congenital rubella. It is presumably due to persistence or reactivation of rubella virus infection.

Most viruses that infect humans can affect both adults and children and are discussed elsewhere in The Manual. Viruses with specific effects on neonates are discussed in see Infections in Neonates. This chapter covers viral infections that are typically acquired during childhood (although many may also affect adults).

Some children with congenital rubella syndrome (eg, with deafness, cataracts, microcephaly, and intellectual disability) develop neurologic deficits in early adolescence.

Diagnosis

  • CSF examination and serologic testing

  • CT

  • Sometimes brain biopsy

The diagnosis of progressive rubella panencephalitis is considered when a child with congenital rubella develops progressive spasticity, ataxia, mental deterioration, and seizures. Testing involves at least CSF examination and serologic testing. CSF total protein and globulin and rubella antibody titers in CSF and serum are elevated.

CT may show ventricular enlargement due to cerebellar atrophy and white matter disease.

Brain biopsy may be necessary to exclude other causes of encephalitis or encephalopathy. Rubella virus usually cannot be recovered by viral culture or immunohistologic testing.

Treatment

No specific progressive rubella panencephalitis treatment exists. Symptoms (eg, seizures, muscle spasticity, weakness) are managed as appropriate.