Subacute sclerosing panencephalitis

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Subacute sclerosing panencephalitis
Other namesDawson disease
seizures, spasticity, poor coordination, coma
Usual onset6-15 years after infection with measles
CausesMeasles virus
Risk factorsMeasles infection
Diagnostic methodEEG, Serologic testing, brain biopsy
PreventionMeasles vaccine
TreatmentSupportive treatment
MedicationIntrathecal interferon alpha, intravenous ribavirin, isoprinosine
PrognosisUsually fatal
Frequency2 in 10,000 for all age groups;[1] As high as 1 in 609 for unvaccinated infants under 15 months[2]

Subacute sclerosing panencephalitis (SSPE), also known as Dawson disease, is a rare form of progressive

measles virus. The condition primarily affects children, teens, and young adults. It has been estimated that about 2 in 10,000 people who get measles will eventually develop SSPE.[1] However, a 2016 study estimated that the rate for unvaccinated infants under 15 months was as high as 1 in 609.[2][3] No cure for SSPE exists, and the condition is almost always fatal. SSPE should not be confused with acute disseminated encephalomyelitis, which can also be caused by the measles virus, but has a very different timing and course.[4]

SSPE is caused by the wild-type virus, not by vaccine strains.[5][6]

Signs and symptoms

SSPE is characterized by a history of primary

myoclonic seizures, blindness, ataxia, and eventually death.[7][8]

Stages of Progression

Symptoms progress through the following 4 stages:[9][10]

  • Stage 1: There may be personality changes, mood swings, or depression. Fever, headache, and memory loss may also be present. This stage may last up to 6 months.
  • Stage 2: This stage may involve jerking, muscle spasms, seizures, loss of vision, and dementia.
  • Stage 3: Jerking movements are replaced by writhing (twisting) movements and rigidity. At this stage, complications may result in
    blindness
    or death.
  • Stage 4: Progressive
    persistent vegetative state, which may be preceded by or concomitant with paralysis, occurs in the final stage, during which breathing, heart rate, and blood pressure are affected. Death usually occurs as a result of fever, heart failure, or the brain’s inability to control the autonomic nervous system
    .

Pathogenesis

A large number of

envelope proteins have restricted expression.[11] As a result, infectious particles like the M protein are not produced, and the virus is able to survive persistently without evoking an immune response. Eventually the infection will lead to SSPE.[12]

Diagnosis

According to the Merck Manual:[8]

"SSPE is suspected in young patients with dementia and neuromuscular irritability. EEG, CT or MRI, CSF examination, and measles serologic testing are done. EEG shows periodic complexes with high-voltage diphasic waves occurring synchronously throughout the recording. CT or MRI may show cortical atrophy or white matter lesions. CSF examination usually reveals normal pressure, cell count, and total protein content; however, CSF globulin is almost always elevated, constituting up to 20 to 60% of CSF protein. Serum and CSF contain elevated levels of measles virus antibodies. Anti-measles IgG appears to increase as the disease progresses. If test results are inconclusive, brain biopsy may be needed."

Treatment

There is no cure available.

anticonvulsants.[8]
Following onset of stage 2, the disease is invariably fatal.

Prognosis

In the classic presentation of the disease, death occurs in 1 to 3 years,[15] but faster and slower progressions can occur. Faster deterioration in cases of acute fulminant SSPE leads to death within 3 months of diagnosis.[16][17] Although the prognosis is bleak for SSPE past stage 1, there is a 5% spontaneous remission rate—this may be either a full remission that may last many years or an improvement in condition giving a longer progression period or at least a longer period with the less severe symptoms.[17][18]

Epidemiology

SSPE is a rare condition, although there is still relatively high incidence in Asia and the Middle East. However, the number of reported cases is declining since the introduction of the measles vaccine—eradication of the measles virus prevents the SSPE mutation and therefore the progression of the disease or even the initial infection itself.[citation needed]

References

  1. ^
    PMID 16235165
    .
  2. ^
    doi:10.1093/ofid/ofw194.81
    .
  3. ^ Sun, Lena (October 28, 2016). "New data shows a deadly measles complication is more common than thought". The Washington Post. Retrieved October 28, 2016.
  4. PMID 24865261
    .
  5. PMID 29985487
    .
  6. PMID 18037676
    .
  7. ^ "CDC pinkbook". 2019-03-29.
  8. ^ a b c "merckmanuals.com".
  9. ^ "medline.gov".
  10. ^ National Institute of Neurological Disorders and Stroke (NINDS) (27 March 2019). "Subacute Sclerosing Panencephalitis Information Page | National Institute of Neurological Disorders and Stroke". ninds.nih.gov. Retrieved 21 November 2021. The initial symptoms of SSPE are subtle and include mild mental deterioration (such as memory loss) and changes in behavior (such as irritability) followed by disturbances in motor function, including uncontrollable involuntary jerking movements of the head, trunk or limbs called myoclonic jerks. Seizures may also occur. Some people may become blind. In advanced stages of the disease, individuals may lose the ability to walk, as their muscles stiffen or spasm. There is progressive deterioration to a comatose state, and then to a persistent vegetative state. Death is usually the result of fever, heart failure, or the brain's inability to continue controlling the autonomic nervous system.
  11. ISBN 978-0-07-174271-9
    .
  12. PMID 6888557
    .
  13. PMID 32809508
    , retrieved 2023-03-10
  14. ^
  15. ^ "SSPE information page www.ninds.nih.gov".
  16. PMID 485888
    .
  17. ^
    PMID 11807185
    .
  18. ^

Further reading

External links
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