Subacute Sclerosing Panencephalitis

Introduction[edit|edit source]

Subacute sclerosing panencephalitis (SSPE) is a slowly progressivebrain disordercaused by mutant measlesvirus(also known as Dawson disease). SSPE typically affects children and adolescents several years after initial infection with the measles.and its incidence is proportional to that of measles. High-income countries have experienced solid declines in SSPE incidence following universalvaccinationagainst measles.

1. Behavioral abnormalities,myoclonus,dementia, visual problems, andpyramidal and extrapyramidalsymptoms are common in patients.
2. The sickness progresses slowly and eventually results in death within 1-3 years.^[1]

ImageSSPE -MRIscans of thebrainat the time of presentation in the neurology clinic (A and B) and 3 months later (C and D). The initial MRI scan (A and B) reveals a focal abnormality in the subcortical white matter of the leftfrontal lobe. In the followup scan, advanced and diffusecorticalatrophy is present, signified by the ventriculomegaly and markedly enlarged sulci.

Epidemiology[edit|edit source]

1 in 100,000 people infected with measles develop SSPE, with a latency of typically a decade (range 4-23 years). Developed nations have benefited from widespread vaccination, as SSPE is very rare in these countries.^[2]

Etiology[edit|edit source]

SSPE is caused by the measles virus, a single-stranded RNA virus (of the paramyxoviridae family). It ishighly contagious, with measles being transmitted to 12 to 18 persons from one infected individual. Transmission occurs through aerosols from person to person. Many complications can occur acutely and chronically, for example the neurological complications seen in SSPE.^[3]

Histopathologically, SSPE shows the same changes seen in other chronic viralencephalitiscases (egNeurological Presentations of HIV Infection) and post-infectious encephalomyelitis.^[2]

Clinical Presentation[edit|edit source]

SSPE presents with progressive neuropsychological deterioration, consisting of personality change,seizures, myoclonus,ataxia, photosensitivity, ocular abnormalities,spasticity, andcoma.^[2]

1. The early symptoms are usually poor school performance, forgetfulness, temper outbursts, easily distracteded, poorsleep, and hallucinations.
2. Unexpected muscular jerks of the arms, head, or body may occur, and finally seizures may occur, together with abnormal uncontrollable muscle movements. Intellect, eyesight and speech continue to deteriorate.
3. Later, the muscles become increasingly rigid, and swallowing becomes difficult (dysphagia), potentially causing person to choke on their saliva, resulting inpneumonia.^[4]

Diagnosis[edit|edit source]

怀疑是引发SSPE的年轻人have had the measles virus and who present later with mental deterioration and muscle jerks.

1. The diagnosis may be confirmed by examination ofcerebrospinal fluid, abloodtest that reveals high levels ofantibodyto the measles virus, by an abnormal electroencephalogram (EEG), and byMRIorCTthat showsbrainabnormalities.
2. A biopsy of the brain may need to be done if the tests cannot reveal a cause.^[4]

Treatment and Prognosis[edit|edit source]

Unfortunately nothing can be done to stop the progression of subacute sclerosing panencephalitis.

Good nursing care is the most important aspect of treatment for SSPE, along with anticonvulsant andantispasmodic drugswhen needed.^[5]

1. Treatments that attempt to prolong and improve thequality of lifecan have some side effects/complications.^[3]
2. The mortality rate is exceptionally high in the case of SSPE, about 95%, while the remaining cases undergo spontaneous remission.^[3]

References[edit|edit source]