Subacute sclerosing leucoencephalitis

Subacute Sclerosing Leucoencephalitis

Subacute sclerosing leucoencephalitis (SSLE) is a rare, progressive neurological disorder that affects the central nervous system. It is characterized by inflammation and degeneration of the brain's white matter, leading to a variety of neurological symptoms. SSLE is often associated with a persistent infection of the measles virus, although it can occur in the absence of a known viral trigger.

Pathophysiology

SSLE is believed to result from a persistent infection of the central nervous system by the measles virus. The virus remains in the brain in a mutated form, evading the immune system and causing chronic inflammation. This inflammation leads to demyelination, or the loss of the protective myelin sheath surrounding nerve fibers, which disrupts normal nerve signal transmission.

The exact mechanism by which the measles virus persists and causes damage in SSLE is not fully understood. It is hypothesized that genetic factors, immune response abnormalities, and viral mutations all play a role in the disease's pathogenesis.

Clinical Presentation

The symptoms of SSLE typically appear several years after the initial measles infection, often between 7 to 10 years later. The disease progresses through several stages:

1. Stage 1: The initial stage is characterized by subtle changes in behavior, personality, and cognitive function. Patients may experience irritability, memory loss, and difficulty concentrating.

2. Stage 2: As the disease progresses, motor symptoms such as myoclonus (involuntary muscle jerks), ataxia (loss of coordination), and seizures become more prominent.

3. Stage 3: In the advanced stages, patients may develop severe neurological deficits, including spasticity, blindness, and dementia. The disease eventually leads to a vegetative state.

4. Stage 4: The final stage is marked by autonomic dysfunction and eventual death, often due to complications such as infections or respiratory failure.

Diagnosis

The diagnosis of SSLE is based on clinical presentation, history of measles infection, and supportive laboratory findings. Key diagnostic tests include:

- Electroencephalogram (EEG): Shows characteristic periodic complexes. - Cerebrospinal fluid (CSF) analysis: May reveal elevated levels of measles-specific antibodies. - Magnetic Resonance Imaging (MRI): Can show white matter lesions consistent with demyelination.

Treatment

There is currently no cure for SSLE, and treatment is primarily supportive. Antiviral medications, such as ribavirin, and immunomodulatory therapies, such as interferon, have been used with limited success. Symptomatic treatments, including anticonvulsants for seizures and muscle relaxants for spasticity, are often employed to improve quality of life.

Prognosis

The prognosis for SSLE is generally poor, with most patients succumbing to the disease within 1 to 3 years of diagnosis. Early detection and intervention may help slow disease progression, but the overall outcome remains unfavorable.

Also see

- Measles - Demyelinating disease - Neurological disorder - Encephalitis

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External links

• Comprehensive information from the National Institute of health.
  

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