Subacute Sclerosing Panencephalitis (SSPE)

Subacute Sclerosing Panencephalitis (SSPE) is a rare, progressive, and fatal neurodegenerative disease caused by persistent infection with the measles virus. Despite its rarity, understanding SSPE is crucial for timely diagnosis and treatment.

Subacute Sclerosing Panencephalitis is a chronic, progressive encephalitis that predominantly affects children and young adults. It is characterized by inflammation of the brain, which leads to a gradual decline in cognitive and motor functions. SSPE is triggered by a persistent infection of the central nervous system with a mutated form of the measles virus.

Symptoms of SSPE

Early Symptoms

The initial symptoms of SSPE can be subtle and are often mistaken for other neurological or psychiatric conditions.

Behavioural Changes: The earliest signs often include personality changes, irritability, and poor school performance.
Cognitive Decline: A gradual decline in cognitive abilities is expected, including memory loss, diminished concentration, and difficulty in problem-solving.

Progressive Symptoms

As the disease advances, symptoms become more pronounced and debilitating.

Myoclonus: Involuntary muscle jerks, known as myoclonus, are a hallmark of SSPE. These jerks are often rhythmic and can affect various parts of the body.
Visual Disturbances: Visual impairments such as blurred vision or partial blindness may occur due to the involvement of the optic nerves.
Motor Dysfunction: Progressive motor dysfunction includes muscle weakness, unsteady gait, and loss of coordination.
Seizures: Generalized or focal seizures may develop, varying in frequency and intensity.

Late Symptoms

In the advanced stages, SSPE leads to severe neurological impairment.

Dementia: Profound cognitive decline results in dementia, characterized by severe memory loss, confusion, and disorientation.
Quadriparesis: The loss of motor function progresses to quadriparesis, where there is weakness in all four limbs.
Coma: Eventually, the disease progresses to a vegetative state or coma, leading to death.

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Diagnosis of SSPE

Clinical Evaluation

The diagnosis of SSPE is based on a combination of clinical findings, laboratory tests, and imaging studies.

History and Symptoms: A thorough clinical history, including a previous measles infection, coupled with the characteristic symptoms, raises suspicion for SSPE.
Neurological Examination: A detailed neurological examination is crucial to identify the extent of cognitive and motor impairments.

Laboratory Tests

Several laboratory tests aid in confirming the diagnosis of SSPE.

EEG (Electroencephalogram): EEG typically shows characteristic periodic complexes, which are high-amplitude bursts of slow waves.
CSF (Cerebrospinal Fluid) Analysis: Elevated levels of measles-specific antibodies in the CSF are indicative of SSPE.
Serology: Blood tests to detect elevated measles-specific antibodies can support the diagnosis.

Imaging Studies

MRI (Magnetic Resonance Imaging): MRI scans may reveal white matter lesions, brain atrophy, and other changes consistent with SSPE.
CT (Computed Tomography) Scan: Although less sensitive than MRI, CT scans can show brain atrophy and calcifications.

Treatment for SSPE

Antiviral Therapy

While there is no cure for SSPE, antiviral therapies aim to slow disease progression.

Interferon: Intrathecal administration of alpha-interferon has shown some efficacy in slowing disease progression.
Ribavirin: Ribavirin, an antiviral medication, has been used in combination with interferon, though results vary.

Immunomodulatory Therapy

Immunomodulatory therapies aim to modulate the immune response.

Inosine Pranobex: This immunomodulatory drug has shown some promise in clinical trials, mainly when used in combination with antiviral therapies.

Symptomatic Treatment

Management of symptoms is crucial to improving the quality of life for SSPE patients.

Anticonvulsants: Medications such as valproic acid and clonazepam are used to control seizures and myoclonus.
Physical Therapy: Physical therapy helps maintain mobility and manage spasticity.

Prognosis of SSPE

The prognosis of SSPE is generally poor, with most patients succumbing to the disease within a few years of diagnosis. Early and aggressive treatment can potentially slow disease progression and extend survival, but the overall outcome remains dire. Factors influencing prognosis include the age of onset, the severity of symptoms at diagnosis, and the response to treatment.

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Causes of SSPE

Measles Virus

The primary cause of SSPE is the persistent infection of the central nervous system by a mutated form of the measles virus. This mutated virus evades the immune system and remains dormant within the brain, eventually triggering the inflammatory and degenerative processes characteristic of SSPE.

Risk Factors

Several factors increase the risk of developing SSPE.

Age at Measles Infection: Children who contract measles before the age of two are at a higher risk of developing SSPE later in life.
Immunization Status: Unvaccinated individuals are at a significantly higher risk of measles infection and subsequent SSPE.
Genetic Susceptibility: Genetic factors may play a role in determining susceptibility to SSPE, though this is not fully understood.

Prevention of SSPE

The most effective way to prevent SSPE is through measles vaccination. The measles, mumps, and rubella (MMR) vaccine is highly effective in preventing measles infection and, consequently, SSPE. Widespread immunization efforts are crucial in reducing the incidence of this devastating disease.

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