SSPE (subacute sclerosing panencephalitis)

Subacute sclerosing panencephalitis
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Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Behavioral changes, myoclonus, seizures, dementia
Complications	Progressive neurological deterioration
Onset	6-15 years after measles infection
Duration	Progressive
Types	N/A
Causes	Persistent measles virus infection
Risks	History of measles infection, especially at a young age
Diagnosis	Clinical evaluation, EEG, MRI, CSF analysis
Differential diagnosis	N/A
Prevention	N/A
Treatment	Antiviral medications, immunomodulatory therapy
Medication	N/A
Prognosis	Poor, often fatal
Frequency	Rare
Deaths	N/A

Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurological disorder that affects children and young adults. It is caused by a persistent infection of the measles virus in the brain. SSPE typically occurs 6 to 15 years after a person has measles, even though they appear to have fully recovered from the initial infection.

Pathophysiology

SSPE is characterized by a chronic, progressive inflammation of the brain (encephalitis) due to a persistent measles virus infection. The virus remains in the brain in a mutated form, evading the immune system and causing widespread neuronal damage. This leads to the characteristic symptoms of SSPE, which include behavioral changes, myoclonus (involuntary muscle jerks), seizures, and progressive intellectual deterioration.

Clinical Presentation

The clinical course of SSPE is divided into four stages:

• Stage 1: The initial stage is marked by subtle behavioral changes, such as irritability, memory loss, and poor school performance.
• Stage 2: This stage involves the onset of myoclonus, seizures, and more pronounced cognitive decline.
• Stage 3: Patients experience severe neurological impairment, including loss of motor skills, unresponsiveness, and autonomic dysfunction.
• Stage 4: The final stage is characterized by a vegetative state, leading to coma and eventually death.

Diagnosis

Diagnosis of SSPE is based on clinical evaluation, supported by laboratory and imaging studies. Key diagnostic tools include:

• Electroencephalogram (EEG): Shows characteristic periodic complexes.
• Magnetic Resonance Imaging (MRI): May reveal brain atrophy and white matter lesions.
• Cerebrospinal Fluid (CSF) Analysis: Elevated measles antibody titers in the CSF are indicative of SSPE.

Treatment

There is no cure for SSPE, and treatment focuses on managing symptoms and slowing disease progression. Antiviral medications such as ribavirin and immunomodulatory therapies like interferon may be used, although their effectiveness is limited. Supportive care, including physical therapy and seizure management, is crucial for maintaining quality of life.

Prognosis

The prognosis for SSPE is poor, with most patients succumbing to the disease within 1 to 3 years of diagnosis. Early detection and intervention may prolong survival, but the disease is ultimately fatal.

Epidemiology

SSPE is a rare condition, with an estimated incidence of 4 to 11 cases per 100,000 cases of measles. The risk is higher in individuals who contracted measles at a young age, particularly before the age of two.

Prevention

The most effective way to prevent SSPE is through widespread vaccination against measles. The measles, mumps, and rubella (MMR) vaccine is highly effective in preventing measles infection and, consequently, SSPE.

Also see

• Measles
• Encephalitis
• Myoclonus
• Viral infections of the central nervous system

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External links

• Comprehensive information from the National Institute of health.
  

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