Subacute sclerosing panencephalitis


Subacute sclerosing panencephalitis

Description, Causes and Risk Factors:

Subacute sclerosing panencephalitis (SSPE/SSP) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. Ordinarily, the measles virus does not cause brain damage, but an abnormal immune response to measles or possibly certain mutant forms of the virus may cause severe illness. This leads to brain inflammation (swelling and irritation) that may last for years.

SSPE has been reported in all parts of the world, but in Western countries it is considered a rare disease. Fewer than 10 cases per year are reported in the United States, a dramatic decrease that followed the Nationwide Measles Immunization Program (NMIP). However, in some countries like India, over 20 cases of SSPE per million people are reported each year.

SSPE tends to occur several years after an individual has measles, even though the person seems to have fully recovered from the illness. Males are more often affected than females, and the disease generally occurs in children and adolescents.

Most individuals with SSPE will die within 1 to 3 years of diagnosis. In a small percentage of people, the disease will progress rapidly, leading to death over a short course within three months of diagnosis. Another small group will have a chronic, slowly progressive form, some with relapses and remissions. A very small number (approximately 5 percent) may experience spontaneous long term improvement and regain lost function. Prevention, in the form of measles vaccination, is the only real "cure" for SSPE.

Immunization against measles is the only known prevention for SSPE. The measles vaccine has been highly effective in reducing the numbers of affected children. Measles immunization should be done according to the recommended American Academy of Pediatrics (AAP) & WHO guidelines.

Symptoms:

General symptoms may include:

    Dementia (loss of cognitive (thought), emotional, and social abilities).

  • Bizarre behavior.

  • Myoclonic jerking (quick muscle jerking or spasms).

  • Seizures.

  • Unsteady gait.

  • Very tense muscles or muscles that lack tone, with weakness in both legs.

Onset of SSPE can occur anywhere from 7-12 years after the original measles illness. The average age of onset is nine years and few individuals live longer than three years after diagnosis.

Diagnosis:

The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid (CSF).

There may be a history of measles in an unvaccinated child. A physical examination may reveal:

    Damage to the optic nerve, which is responsible for sight.

  • Damage to the retina, the part of the eye that receives light.

  • Muscle twitching.

  • Poor performance on motor (movement) coordination tests.

The following tests may be performed:

    Electroencephalogram (EEG).

  • Brain MRI.

  • Serum antibody titer to look for signs of previous measles infection.

  • Spinal tap.

  • Brain biopsy.

Laboratory findings: Paired serum and cerebrospinal fluid (CSF) samples are tested using the Euroimmun kit for antibodies of the IgG class against measles virus in CSF. The ratio of measles-specific antibodies in the CSF and the serum in comparison to the ratio of total IgG or total albumin in the CSF and the serum (CSQrel) is used to determine whether there is an indication of measles-specific antibody production in the central nervous system (CNS). Please note that CSQrel values between 1.5 and 4.0 should be interpreted with caution when diagnosing measles, SSPE. Non-infectious diseases, such as multiple sclerosis (MS), can also cause an elevated CSF/serum ratio. While the CSF/serum quotients measured in these cases are elevated, they are significantly lower than those found in SSPE cases where the CSQrel is typically greater than 4.0. As always, the result should be interpreted within the context of all relevant clinical information i.e., age of the patient, past history of wild-type measles infection, evidence of a CNS infection of viral etiology, and other laboratory results, such as elevated serum levels of measles IgG >5000 mIU/mL.

Treatment:

Treatment for SSPE is still undetermined. A combination of oral isoprinosine (Inosiplex) and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it life long. Effective immunization against measles is the only solution presently available to the problem of this dreaded disease.

For patients in the terminal phase of the disease there is a palliative care and treatment scheme — this involves anticonvulsant therapy (to help with the body's progressive loss of control of the nervous system causing gradually more intensive spasms/convulsions) alongside supportive measures to help maintain vital functioning. It is fairly standard as the infection's spread and symptoms intensify that feeding tubes need to be inserted to keep a nutritional balance. As the disease progresses to its most advanced phase, the patient will need constant Nursing as normal bodily function declines to the complete collapse of the nervous system.

NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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