Progressive rubella panencephalitis (PRP) is a

neurological disorder Neurological disorders represent a complex array of medical conditions that fundamentally disrupt the functioning of the nervous system. These disorders affect the brain, spinal cord, and nerve networks, presenting unique diagnosis, treatment, and ...

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 Human brain, brain. The severity can be variable with symptoms including reduction or alteration in consciousness, aphasia, headache, fever, confusion, a stiff neck, and vomiting. Complications may include se ...

, 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 Retinopathy is any damage to the retina of the eyes, which may cause vision impairment. Retinopathy often refers to retinal vascular disease, or damage to the retina caused by abnormal blood flow. Age-related macular degeneration is technically in ...

, optic atrophy ** frank

dementia Dementia is a syndrome associated with many neurodegenerative diseases, characterized by a general decline in cognitive abilities that affects a person's ability to perform activities of daily living, everyday activities. This typically invo ...

leading to

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

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

Diagnosis

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

spasticity Spasticity () is a feature of altered skeletal muscle performance with a combination of paralysis, increased tendon reflex activity, and hypertonia. It is also colloquially referred to as an unusual "tightness", stiffness, or "pull" of muscles. ...

ataxia Ataxia (from Greek α- negative prefix+ -τάξις rder= "lack of order") is a neurological sign consisting of lack of voluntary coordination of muscle movements that can include gait abnormality, speech changes, and abnormalities in e ...

, mental deterioration, and

seizures A seizure is a sudden, brief disruption of brain activity caused by abnormal, excessive, or synchronous neuronal firing. Depending on the regions of the brain involved, seizures can lead to changes in movement, sensation, behavior, awareness, 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) or immunoglobulin (Ig) is a large, Y-shaped protein belonging to the immunoglobulin superfamily which is used by the immune system to identify and neutralize antigens such as pathogenic bacteria, bacteria and viruses, includin ...

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), malnutrition, poor nourishment, poor circulatory system, circulation, loss of hormone, ...

and

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

disease.

Brain biopsy Brain biopsy is the removal of a small piece of brain tissue for the diagnosis of abnormalities of the brain. It is used to diagnose tumors, infection, inflammation, and other brain disorders. By examining the tissue sample under a microscope, th ...

may be necessary to exclude other causes of encephalitis or

encephalopathy Encephalopathy (; ) means any disorder or disease of the brain, especially chronic degenerative conditions. In modern usage, encephalopathy does not refer to a single disease, but rather to a syndrome of overall brain dysfunction; this syndrome ...

. 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 This glossary of biology terms is a list of definitions of fundamental terms and concepts used in biology, the study of life and of living organisms. It is intended as introductory material for novices; for more specific and technical definitions ...

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

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

Cause

Diagnosis

Treatment

Incidence

See also

References

Further reading