Rare DiseasesWhat is Subacute Sclerosing Panencephalitis?

What is Subacute Sclerosing Panencephalitis?

Subacute sclerosing panencephalitis (SSPE) is a serious complication that develops in a small percentage of individuals infected with the measles virus. The measles virus is a respiratory virus that can affect the respiratory system when it enters the body. However, the virus can sometimes spread to the nervous system and affect the membranes of the brain and nerve cells. The initial infection caused by the measles virus usually passes with mild symptoms, but the virus is not completely eliminated from the body and can reactivate in the nervous system in some cases.

The symptoms of SSPE typically worsen over time, and the person’s mental and physical condition is severely affected as the disease progresses. This condition is characterized as a progressive degenerative process due to damage to the nervous system. SSPE is usually manifested by symptoms such as seizures, muscle spasms, difficulty swallowing, vision disorders, and mental disorders. The diagnosis of SSPE is made using neurological tests such as clinical symptoms, EEG, and brain magnetic resonance imaging. Treatment options are limited and generally focus on managing symptoms. SSPE treatment may include antiviral drugs, anticonvulsants, and symptomatic treatment methods. However, these treatments cannot stop the progression of the disease and usually help alleviate symptoms.

Causes of Subacute Sclerosing Panencephalitis

The exact cause of subacute sclerosing panencephalitis is not known, but it is known that the disease occurs after a measles virus infection. The following factors are highlighted regarding the development of SSPE:

Measles Virus Infection: SSPE is associated with the measles virus. SSPE usually occurs years after the initial exposure to the measles virus.

Immune System Problems: Disorders of the immune system or a weakened immune system can increase the risk of SSPE.

Genetic Factors: Some studies suggest that SSPE may be associated with genetic predisposition.

Effect of the Virus on the Brain: Long-term effects of the measles virus on the brain and inflammatory reactions it causes may play a role in the development of SSPE.

SSPE (Subacute Sclerosing Panencephalitis) Disease

Symptoms of Subacute Sclerosing Panencephalitis

The symptoms of SSPE can worsen over time and may include:

  • mental disorders and lack of concentration
  • muscle spasms and coordination disorders
  • loss of appetite, weight loss, and difficulty swallowing
  • vision disorders, optic nerve involvement
  • seizures

Diagnosis of Subacute Sclerosing Panencephalitis

The diagnosis of subacute sclerosing panencephalitis is generally made through a combination of clinical symptoms, neurological examination findings, and various tests. SSPE diagnosis can be made using the following methods:

Clinical Evaluation: The patient’s symptoms and medical history are carefully examined. SSPE usually occurs in patients with a history of measles.

Neurological Examination: Neurological examination focuses on evaluating muscle stiffness, muscle spasms, increased reflexes, and other neurological findings.

Electroencephalography (EEG): EEG measures brain activity and can typically show abnormal findings in SSPE patients. Typically, epileptiform discharges and slowing are observed.

Brain Imaging: Magnetic resonance imaging or computed tomography scans can be used to determine structural changes and atrophy in the brain.

Measles Antibody Tests: The presence of antibodies against the measles virus can be investigated through blood tests. Measles antibody levels in the blood can be measured for SSPE diagnosis.

Cerebrospinal Fluid Analysis: Examination of cerebrospinal fluid through lumbar puncture can reveal specific findings in SSPE.

Treatment of Subacute Sclerosing Panencephalitis

Treatment of subacute sclerosing panencephalitis is difficult and there is no fully effective treatment method. Treatment generally includes symptomatic and supportive measures, and may include the following elements:

Antiviral Drugs: Antiviral drugs against the measles virus can be used. However, these drugs are generally ineffective in altering the course of the disease.

Anticonvulsants: SSPE patients often experience seizures. In this case, anticonvulsant drugs can be used to control seizures.

Muscle Relaxants: Muscle relaxants can be used to alleviate muscle stiffness and spasms.

Symptomatic Treatments: Symptomatic treatments can be applied for the patients’ symptoms.

Physical Therapy: Physical therapy and rehabilitation programs can help increase muscle strength, improve mobility, and correct posture.

Supportive Care: Providing emotional and psychosocial support to patients and their families is important. SSPE can be emotionally challenging for patients and their families.

A 29-year-old young man who has been bedridden for 17 years due to SSPE disease reacts only to Haluk Levent songs

Management of Subacute Sclerosing Panencephalitis

The treatment of subacute sclerosing panencephalitis is quite difficult because the disease has a progressive course and the chance of complete recovery is low. Treatment may include:

Anticonvulsant Drugs: Seizures frequently occur in SSPE patients. Anticonvulsant drugs can be used to control these seizures.

Steroids: Steroid drugs can help reduce brain inflammation and alleviate the severity of symptoms.

Immunosuppressive Drugs: In some cases, drugs that suppress the immune system response can be used.

Symptomatic Treatments: Medications or therapies can be used to alleviate symptoms. For example, muscle relaxants can be used to relieve muscle spasms.

Supportive Treatment: Supportive treatments should be provided to improve the patient’s overall health and quality of life.

Epilepsy Surgery: In rare cases, epilepsy surgery may be considered to control severe seizures associated with SSPE.

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