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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, a deadly brain disorder, is crucial for timely diagnosis and treatment. So, what is SSPE? 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.
- Behavioral 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 disease 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.
Causes of SSPE
The measles virus typically doesn't harm the brain. On the other hand, severe illness or even death may result from an aberrant immune response to measles or specific virus mutants. This immune reaction causes brain inflammation (swelling and irritation) that can persist for years.
SSPE has been reported worldwide but is rare in Western countries. In the United States, very few cases are seen due to the nationwide measles vaccination program. SSPE typically develops several years after a person has had measles, even if they appear to recover fully. It is more common in males and usually affects children and adolescents.
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Get Second OpinionDiagnosis 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
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
The goal of immunomodulatory treatments is to alter the immune response. Measles immunization plays a key role in preventing SSPE by reducing the risk of persistent measles virus infection and subsequent immune complications.
- 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.
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Prognosis of SSPE
The prognosis of SSPE disease 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.
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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040-68334455Frequently Asked Questions
What is the survival rate for subacute sclerosing panencephalitis (SSPE)?
The survival rate for SSPE is low. Once symptoms appear, the disease typically progresses rapidly, and most individuals die within 1 to 3 years after onset. However, survival time can vary depending on the age of onset and the severity of the disease.
What causes SSPE to occur in some cases of measles?
SSPE occurs in rare cases when the measles virus infects the brain and persists in the body for years, leading to progressive neurological damage. The exact mechanism is not fully understood, but it is believed to result from a defective immune response to the virus after an initial measles infection, often in childhood.
What age is at risk for SSPE?
SSPE primarily affects children and young adults, typically occurring 6 to 8 years after a previous measles infection. It is more common in children under 2 years old who have had a measles infection, especially in cases where the infection occurred before vaccination became widespread.
Which areas of the body are degenerated in SSPE?
In SSPE, the degeneration primarily affects the brain, particularly the gray matter. The disease leads to damage in regions responsible for motor control, cognition, and sensory processing, leading to severe neurological symptoms such as seizures, motor dysfunction, and cognitive decline.
What is the difference between encephalitis and SSPE?
Encephalitis is an inflammation of the brain caused by infections or autoimmune reactions, which can occur acutely. SSPE, on the other hand, is a progressive and rare neurological disorder that occurs years after a measles infection, causing long-term, gradual brain degeneration. While encephalitis occurs suddenly and can be treated, SSPE develops slowly and is fatal with no effective cure.
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