Progressive rubella panencephalitis
Encyclopedia
Progressive rubella panencephalitis (PRP) is a neurological disorder
which may occur in a child with congenital rubella
. It is a slow viral infection of the brain characterized by chronic encephalitis
, usually manifesting between 8-19 years of age.
It is believed to be due to a persistence or reactivation of rubella virus infection.
, ataxia
,
mental deterioration, and seizures. Testing involves at least CSF examination and serology
.
Elevated CSF total protein and globulin and elevated rubella antibody
titers in CSF and serum occur.
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 cannot usually be recovered by viral culture or immunohistologic testing.
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:
but without intracellular
inclusion bodies
.
Only 20 patients have been identified since first recognized in 1974.
Neurological disorder
A neurological disorder is a disorder of the body's nervous system. Structural, biochemical or electrical abnormalities in the brain, spinal cord, or in the nerves leading to or from them, can result in symptoms such as paralysis, muscle weakness, poor coordination, loss of sensation, seizures,...
which may occur in a child with congenital rubella
Rubella
Rubella, commonly known as German measles, is a disease caused by the rubella virus. The name "rubella" is derived from the Latin, meaning little red. Rubella is also known as German measles because the disease was first described by German physicians in the mid-eighteenth century. This disease is...
. It is a slow viral infection of the brain characterized by chronic encephalitis
Encephalitis
Encephalitis is an acute inflammation of the brain. Encephalitis with meningitis is known as meningoencephalitis. Symptoms include headache, fever, confusion, drowsiness, and fatigue...
, usually manifesting between 8-19 years of age.
It is believed to be due to a persistence or reactivation of rubella virus infection.
Diagnosis
The diagnosis is considered when a child with congenital rubella develops progressive spasticitySpasticity
Spasticity is a feature of altered skeletal muscle performance in muscle tone involving hypertonia, which is also referred to as an unusual "tightness" of muscles...
, ataxia
Ataxia
Ataxia is a neurological sign and symptom that consists of gross lack of coordination of muscle movements. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum...
,
mental deterioration, and seizures. Testing involves at least CSF examination and serology
Serology
Serology is the scientific study of blood serum and other bodily fluids. In practice, the term usually refers to the diagnostic identification of antibodies in the serum...
.
Elevated CSF total protein and globulin and elevated rubella antibody
Antibody
An antibody, also known as an immunoglobulin, is a large Y-shaped protein used by the immune system to identify and neutralize foreign objects such as bacteria and viruses. The antibody recognizes a unique part of the foreign target, termed an antigen...
titers in CSF and serum occur.
CT may show ventricular enlargement due to cerebellar atrophy
Atrophy
Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations , poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply to the target organ, disuse or lack of exercise or disease intrinsic to the tissue itself...
and white matter
White matter
White matter is one of the two components of the central nervous system and consists mostly of myelinated axons. White matter tissue of the freshly cut brain appears pinkish white to the naked eye because myelin is composed largely of lipid tissue veined with capillaries. Its white color is due to...
disease.
Brain biopsy
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 Alzheimer's disease, tumors, infection, inflammation, and other brain disorders....
may be necessary to exclude other causes of encephalitis or encephalopathy
Encephalopathy
Encephalopathy means disorder or disease of the brain. In modern usage, encephalopathy does not refer to a single disease, but rather to a syndrome of global brain dysfunction; this syndrome can be caused by many different illnesses.-Terminology:...
.
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.Progression
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
- retinopathyRetinopathyRetinopathy is a general term that refers to some form of non-inflammatory damage to the retina of the eye. Frequently, retinopathy is an ocular manifestation of systemic disease.-Pathophysiology:Causes of retinopathy are varied:...
, optic atrophy - frank dementiaDementiaDementia is a serious loss of cognitive ability in a previously unimpaired person, beyond what might be expected from normal aging...
leading to comaComaIn medicine, a coma is a state of unconsciousness, lasting more than 6 hours in which a person cannot be awakened, fails to respond normally to painful stimuli, light or sound, lacks a normal sleep-wake cycle and does not initiate voluntary actions. A person in a state of coma is described as... - spasticity and brainstem involvement with death in 2-5 years
Incidence
PRP is very rare and similar to SSPESubacute sclerosing panencephalitis
Subacute sclerosing panencephalitis is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection of immune resistant measles virus . No cure for SSPE exists, but the condition can be managed by medication if treatment is started at...
but without intracellular
Intracellular
Not to be confused with intercellular, meaning "between cells".In cell biology, molecular biology and related fields, the word intracellular means "inside the cell".It is used in contrast to extracellular...
inclusion bodies
Inclusion bodies
Inclusion bodies are nuclear or cytoplasmic aggregates of stainable substances, usually proteins. They typically represent sites of viral multiplication in a bacterium or a eukaryotic cell and usually consist of viral capsid proteins...
.
Only 20 patients have been identified since first recognized in 1974.
External links
- PEDBASE.org - The Pediatric Database - Progressive Rubella Panencephalitis
- Wiley InterScience - Characterization of immune complexes in progressive rubella panencephalitis
- PubMed - Progressive rubella panencephalitis. Late onset after congenital rubella.
- The Merck Manual - Rubella
- A Practical Guide to Clinical Virology (Second Edition) - 40. Slow viruses: CONVENTIONAL SLOW VIRUS INFECTIONS - SUBACUTE SCLEROSING PANENCEPHALITIS (SSPE)