Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis that affects primarily children and young adults, caused by a persistent infection of immune resistant measles virus (which can be a result of a mutation of the virus itself). 1 in 100,000 people infected with measles develop SSPE. SSPE is ‘incurable’ but the condition can be managed by medication if treatment is started at an early stage. Much of the work on SSPE has been completed by the National Institute of Neurological Disorders and Stroke (NINDS).
SSPE is also known as Dawson Disease, Dawson encephalitis and measles encephalitis.
Characterized by a history of primary measles infection usually before the age of 2 years, followed by several asymptomatic years (6–15 on average), and then gradual, progressive psychoneurological deterioration, consisting of personality change, seizures, myoclonus, ataxia, photosensitivity, ocular abnormalities, spasticity, and coma.
The progression of symptoms begins with stage 1 — in this stage the behaviour of person become more abnormal and erratic, the person can be irritable and personality alterations can occur. This is often accompanied by memory loss and mental deterioration characterised by intellectual difficulty. As the nervous system begins to lose control of movement, the person develops myoclonic spasms/jerks (these being involuntary motions and spasms in extremities). As the disease progresses towards stage 2, the intensity of the spasms and the mental deterioration increases. The spasms can grow to such an extent that loss of the ability to walk can be a common sign. Also, the person will suffer speech impairment and increasingly deteriorated comprehension coupled with dysphagia. At this point the infection is at stage 2. The final, advanced stages of SSPE include the steady decline in body function with increased intensity of the stage 2 symptoms/signs and also blindness. At the end of the final stages the person is likely to be mute and/or comatose.
Characteristic periodic activity is seen on EEG (this activity showing widespread cortical dysfunction); pathologically, the white matter of both the hemispheres and brainstem are affected, as well as the cerebral cortex, and eosinophilic inclusion bodies are present in the cytoplasm nuclei of neurons and glial cells. Diagnosis of SSPE is often difficult due to a normal CSF profile — noted changes in the CSF profile only include a marked elevation in CSF immunoglobulin. Rubeola Ig G Antibody Titres will be high.
Death usually occurs within 3 years. If the diagnosis is made during stage 1 of the SSPE infection then it is possible to treat the disease. However, once SSPE progresses to stage 2 then it is universally fatal in all occurrences. The standard rate of decline spans anywhere between 1–3 years after the onset of the infection. The progression of each stage is unique to the sufferer and cannot be predicted although the pattern or symptoms/signs can be. Although the prognosis is bleak for SSPE past stage 1, it should be noted that there is a 5% remission rate — this may be either a full remission or an improvement in condition giving a longer progression period or at least a longer period with the less severe symptoms. Regardless of the stage that the infection is at, treatment with inosine pranobex combined with interferon can give up to a 50% remission/improvement rate.
[tubepress mode=’tag’, tagValue=’Subacute sclerosing panencephalitis’]