Progressive rubella panencephalitis (PRP) is a

neurological disorder A neurological disorder is any disorder of the nervous system. Structural, biochemical or electrical abnormalities in the brain, spinal cord or other nerves can result in a range of symptoms. Examples of symptoms include paralysis, muscle weakn ...

which may occur in a child with congenital

rubella Rubella, also known as German measles or three-day measles, is an infection caused by the rubella virus. This disease is often mild, with half of people not realizing that they are infected. A rash may start around two weeks after exposure and ...

. It is a slow viral infection of the brain characterized by chronic

encephalitis Encephalitis is inflammation of the brain. The severity can be variable with symptoms including reduction or alteration in consciousness, headache, fever, confusion, a stiff neck, and vomiting. Complications may include seizures, hallucinations, ...

, usually manifesting between 8–19 years of age. It is believed to be due to a persistence or reactivation of rubella virus infection.

Cause

It develops 6 months to 4 years after the primary rubella infection, which in most cases is a congenital rubella. In children with congenital rubella infection the deficits remain stable; neurological deterioration after the first few years of life is not believed to occur. Progression of the disease can be divided into two stages: * 1st stage: Behavioural Changes ** insidious onset ** subtle changes in behaviour and declining school work * 2nd stage: Neurological Changes ** seizures – sometimes myoclonic ** cerebellar ataxia ** spastic weakness ** retinopathy, optic atrophy ** frank

dementia Dementia is a disorder which manifests as a set of related symptoms, which usually surfaces when the brain is damaged by injury or disease. The symptoms involve progressive impairments in memory, thinking, and behavior, which negatively affe ...

leading to

coma A coma is a deep state of prolonged unconsciousness in which a person cannot be awakened, fails to respond normally to painful stimuli, light, or sound, lacks a normal wake-sleep cycle and does not initiate voluntary actions. Coma patients exhi ...

** spasticity and brainstem involvement with death in 2–5 years

Diagnosis

The diagnosis is considered when a child with congenital rubella develops progressive spasticity,

ataxia Ataxia is a neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes, and abnormalities in eye movements. Ataxia is a clinical manifestation indicating dysfunction of ...

, mental deterioration, and

seizures An epileptic seizure, informally known as a seizure, is a period of symptoms due to abnormally excessive or neural oscillation, synchronous neuronal activity in the brain. Outward effects vary from uncontrolled shaking movements involving much o ...

. Testing involves at least CSF examination and

serology Serology is the scientific study of Serum (blood), serum and other body fluids. In practice, the term usually refers to the medical diagnosis, diagnostic identification of Antibody, antibodies in the serum. Such antibodies are typically formed in r ...

. Elevated CSF total protein and globulin and elevated rubella

antibody An antibody (Ab), also known as an immunoglobulin (Ig), is a large, Y-shaped protein used by the immune system to identify and neutralize foreign objects such as pathogenic bacteria and viruses. The antibody recognizes a unique molecule of the ...

titers in CSF and serum occur. CT may show ventricular enlargement due to cerebellar

atrophy Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations (which can destroy the gene to build up the organ), poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply t ...

and

white matter White matter refers to areas of the central nervous system (CNS) that are mainly made up of myelinated axons, also called tracts. Long thought to be passive tissue, white matter affects learning and brain functions, modulating the distribution ...

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

Treatment

Although no specific treatment exists, the disease can be managed with anticonvulsants, physiotherapy, etc.

Incidence

PRP is very rare and similar to SSPE but without intracellular

inclusion bodies Inclusion bodies are aggregates of specific types of protein found in neurons, a number of tissue cells including red blood cells, bacteria, viruses, and plants. Inclusion bodies of aggregations of multiple proteins are also found in muscle cells ...

. Only 20 patients have been identified since first recognized in 1974.

Cause

Diagnosis

Treatment

Incidence

See also

References

Further reading