Progressive rubella panencephalitis

Progressive Rubella Panencephalitis (PRP) is a rare, progressive, and often fatal neurological disorder that follows a congenital rubella infection. Although rubella, also known as German measles, is typically a mild childhood illness, its effects on a developing fetus can be severe, leading to a range of congenital defects known as Congenital Rubella Syndrome (CRS). PRP represents a late manifestation of CRS, occurring several years after birth in individuals who had been infected with the rubella virus in utero.

Etiology

PRP is caused by the persistent infection of the nervous system by the rubella virus. The exact mechanism by which the virus remains latent and subsequently reactivates is not fully understood. However, it is believed that the virus persists in brain tissue, leading to chronic inflammation and progressive damage over time.

Clinical Presentation

The onset of PRP typically occurs in late childhood or early adolescence, years after the initial rubella infection. Symptoms are progressive and may include mental retardation, motor dysfunction, ataxia (loss of control of body movements), spasticity (stiff or rigid muscles), and seizures. As the disease progresses, individuals may experience worsening neurological decline, leading to severe disability and eventually death.

Diagnosis

Diagnosis of PRP is challenging and is often based on a combination of clinical presentation, history of congenital rubella infection, and laboratory tests. Magnetic Resonance Imaging (MRI) of the brain may show characteristic patterns of inflammation and damage. Additionally, detection of rubella antibodies in the cerebrospinal fluid (CSF) can support the diagnosis.

Treatment

There is no cure for PRP, and treatment is primarily supportive. Management may include anticonvulsant medications to control seizures, physical therapy to manage motor dysfunction, and other interventions aimed at improving quality of life. The progressive nature of the disease often necessitates comprehensive care and support for both the patient and their family.

Prevention

The most effective way to prevent PRP is through the prevention of congenital rubella infection. This is achieved by vaccinating against rubella. The MMR vaccine, which protects against measles, mumps, and rubella, is highly effective and widely recommended. Ensuring high vaccination coverage in the population significantly reduces the incidence of congenital rubella syndrome and, consequently, PRP.

Prognosis

The prognosis for individuals with PRP is generally poor, with the disease progressing to severe neurological disability and often leading to death within a few years of onset. The rate of progression can vary among individuals.

NIH genetic and rare disease info

• NIH info on Progressive rubella panencephalitis

Progressive rubella panencephalitis is a rare disease.