Subacute Sclerosing Panencephalitis

Classified as a persistent immune-resistant form of rubeola (measles), subacute sclerosing panencephalitis (SSPE) is a rare infection that results from a defective variant of rubeola that was generated during the acute phase of disease. The SSPE virus is slow-growing and causes cytopathologic effects in neurons; symptoms occur many years after acute disease. SSPE is a chronic illness that causes progressive encephalitis, mainly in children and young adults. Approximately 1 in every 100,000 people infected with rubeola develops SSPE. The illness consists of two stages; if SSPE reaches stage 2, it is fatal. SSPE is also known as Dawson disease, Dawson encephalitis, and measles encephalitis.

Causes

SSPE is caused by the rubeola virus, a member of the genus Morbillivirus in the Paramyxoviridae family. The highly contagious rubeola virus is transmitted by respiratory droplets. Although the incidence of rubeola declined vastly following the introduction of the rubeola vaccine, the vaccine is not available in all areas of the world. Therefore, the disease still affects approximately 30 million people annually, with the highest percentage of cases occurring in Africa. Fewer than 10 cases of SSPE are reported each year in the United States. SSPE seems to affect males more than females with a ratio of 3:1.

Complications

SSPE causes psychoneurological problems, with death resulting from heart failure or autonomic nervous system failure within 3 months to 3 years if treatment is not provided.

Assessment Findings

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