Subacute Sclerosing Panencephalitis (SSPE) - Facts and Information
Author: Disabled World : Contact: Disabled World
Published: 2010-03-11 : (Rev. 2015-02-05)
Synopsis and Key Points:
Symptoms of Subacute Sclerosing Panencephalitis SSPE include changes in behavior and mild mental deterioration such as memory loss.
The symptoms a person with SSPE experiences are subtle. They usually include symptoms such as changes in behavior and mild mental deterioration such as memory loss.
SSPE is a form of progressive neurological disorder that affects the central nervous system of children and young adults. The disorder is slow yet persistent, and is a viral infection caused by defective measles virus. SSPE is found in every part of the world today, but is considered to be a rare disease in developed nations with less than ten-percent of people who experience the disorder in America. Widespread immunization with measles vaccine has found a ninety-percent decline in the incidence of SSPE in nations that practice such immunization. In the nations of India and Eastern Europe the incidence of SSPE remains high. There is also a higher incidence rate among males than females with a ratio of three to one.
Many young people with SSPE present a history of measles infection at an early age, commonly before the age of two, followed by a latent period of six to eight years prior to the onset of neurological symptoms. Researchers believe that despite the long interval between the initial measles infection and the onset of SSPE, the infection of the person's brain happens soon after the primary measles infection, and then progresses at a slow rate. The reasons behind the persistence and slow progression of the disorder remain unknown.
The symptoms a person with SSPE experiences are subtle. They usually include symptoms such as changes in behavior and mild mental deterioration such as memory loss. The symptoms that follow are commonly involuntary jerking movements of the person's head, limb or trunk jerks, and additional motor function disturbances. The person may experience seizure activity, or become blind. As the disorder advances, the affected person might lose the ability to walk as their muscles spasm or stiffen. The person progresses towards a comatose state, followed by a vegetative state. People with SSPE commonly die as a result of fever, heart failure, or their brain's inability to continue controlling their autonomic nervous system.
Encephalitis as a whole involves a rare complication of measles infection and is categorized into three unique types. The types of encephalitis include acute encephalitis, subacute sclerosing encephalitis (SSPE), as well as subacute measles encephalitis in the immuno-suppressed. Acute encephalitis is most likely a form of autoimmune phenomenon and not an infection of the person's brain tissue. SSPE involves a progressive course that commonly begins a number of years after the person experiences an acute infection with the measles virus during their early childhood. A defective measles virus, or vaccination, may also lead to the progression of SSPE. The disorder itself is clinically characterized by a slow and erratic course that many times results in the death of the person affected. SSPE is also referred to by the names, 'Subacute sclerosing leukoencephalitis,' and, 'Dawson's encephalitis.'
Symptoms of SSPE:
The list of signs and symptoms associated with Subacute Sclerosing Panencephalitis (SSPE) is long. The symptoms of SSPE can include the following:
- Memory loss
- Optic atrophy
- Unsteady gait
- Abnormal EEG
- Myoclonic jerks
- Cortical blindness
- Brain inflammation
- Behavioral changes
- Very tense muscles
- Progressive dementia
- Involuntary movements
- Intellectual deterioration
- Homeostasis disturbances
- Neurological deterioration
- Increased measles antibodies in blood
- Increased measles antibodies in cerebrospinal fluid
- Increased gammaglobulin levels in cerebrospinal fluid
Causes of SSPE:
The measles virus usually does not cause brain damage. An abnormal immune response to the measles, or a potential mutant form of the virus, can cause either severe illness or death. Such a reaction can lead to inflammation of the person's brain, to include swelling and irritation of the person's brain that can last for a number of years. SSPE is a disorder that has been reported in all parts of the world, although in western nations it is considered to be a rare form of disease. In nations such as India, greater than twenty persons per million are affected by SSPE each year.
People with Subacute Sclerosing Panencephalitis may present with a history of measles without having received the measles vaccination. A physical examination of the person affected might demonstrate damage to their optic nerve, which is responsible for their sight. The person may also have damage to their retina, the part of their eye that receives light. They may perform poorly on motor coordination testing and experience muscle twitching.
Testing that may be performed in relation to SSPE can include a brain MRI, or an electroencephalogram (EEG). A doctor might order a serum antibody titer to look for signs of a prior measles infection. Another test that can be performed is a spinal tap.
Treatment of SSPE:
There is currently no cure for SSPE. There are; however, clinical trials of antiviral drugs to include ribavirin and Isoprinosine, as well as interferon alpha drugs, that have suggested that whether administered alone or in combination may halt the progression of the disease, prolonging the person's life. The long-term effects on people affected by SSPE, as well as the eventual outcomes, remain unknown. The most important aspect of treatment related to SSPE remains good nursing care, antispasmodic and anticonvulsant medications.
The majority of persons affected by SSPE die within one to three years of achieving a diagnosis of the disorder. A small percentage of people who have been diagnosed with SSPE experience a rapid progression of the disorder that leads to death over a short period of time; sometimes within three months of receiving a diagnosis. There is another small group of affected persons who experience a chronic and slowly progressing form of the disorder marked by relapses and remissions. Approximately five-percent of persons affected by SSPE might experience a spontaneous long-term improvement, regaining lost abilities.
Prevention remains the number one way to, 'cure,' SSPE. It is important to contact a health care provider if your child has not completed their scheduled vaccinations. The measles vaccine is included in the measles-mumps-rubella (MMR) vaccine. The measles vaccine remains highly-effective in preventing the numbers of children affected by Subacute Sclerosing Panencephalitis (SSPE).
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