Subacute sclerosing encephalitis (SSPE) is a rare disease caused by the measles virus. Its incidence has dropped significantly since the measles vaccine was introduced, but unfortunately, despite treatment trials, no cures have yet been seen. What are the causes and symptoms of subacute sclerosing encephalitis (SSPE)?

Subacute sclerosing panencephalitis(SSPE - Subacute sclerosing panencephalitis, Dawson's disease, subacute encephalitis with inclusion bodies) usually develops 7 years after suffering an early measles infection and mainly affects children under 12, more often boys than girls. The incidence of SSPE has decreased significantly since the introduction of the measles vaccine and is now around 1 in 100,000.

Subacute sclerosing encephalitis presents with progressive dementia, incoordination, ataxia, myoclonus, and other focal neurological symptoms. It is a chronic disease, usually lasting several years. However, there have been reports of a violent course leading to death within a few months. Only about 10 percent of patients achieve spontaneous recovery that can last for many years.

In the course of infection, there are perivascular infiltrates in the cortex and white matter of the brain made of plasma cells and other mononuclear cells, as well as foci of demyelination and gliosis in the white matter and deeper layers of the cerebral cortex. In addition, the presence of degenerative changes in the neurons of the cortex, the basal and ponsal ganglia, and in the lower olive nuclei are observed. In neurons and glial cells there are intranuclear and endoplasmic eosinophilic inclusion bodies. Labeling of inclusion bodies by immunofluorescence shows a positive result, characteristic of the measles virus.

Subacute sclerosing encephalitis: symptoms

Subacute sclerosing encephalitis develops gradually without the accompanying fever. The first symptoms can be:

  • forgetting;
  • difficulties with learning and school activities;
  • restlessness.

Then, within weeks or even months, they appear:

  • motor coordination disorders;
  • ataxia;
  • myoclonus within the limbs andtorso;
  • apraxia;
  • choreoathetotic or ballroom movements;
  • pyramid symptoms;
  • speech disorder;
  • seizures;
  • dystonic position.

In the final stage of the disease, there are visual disturbances, including blindness, deafness and spastic tetraplegia, resembling the state of decongestion.

Subacute sclerosing encephalitis: research

The diagnosis of subacute sclerosing encephalitis is based on the clinical picture, cerebrospinal fluid examination and electroencephalography (EEG).

Increased levels of anti-measles antibodies can be found both in the blood serum and in the cerebrospinal fluid. On electrophoretic examination of the cerebrospinal fluid, these antibodies appear as oligoclonal IgG bands. CSF protein and glucose levels are normal and pleoocytosis is normal or slightly elevated.

Generalized disturbances in the bioelectrical cortex of the cerebral cortex often occur in the EEG examination, with the image of "discharge and suppression" of high-voltage slow waves or spike-slow wave assemblies appearing with a frequency of 4-20 s synchronously or independently of myoclonus.

Imaging tests are also used in the diagnosis of subacute sclerosing encephalitis. Computed tomography shows cortical atrophy and low-density focal or multifocal lesions in the white matter. In the case of T2-weighted MRI images, changes in the periventricular white matter can be observed.

Subacute sclerosing encephalitis: treatment

Despite attempts to use many drugs in patients, such as bromodeoxyuridine, amantadine, inosine or isoprosine, no recovery has been observed. In some cases, clinical improvement or disease progression is achieved by intraventricular administration of interferon alpha followed by intravenous or intraventricular administration of ribavirin. However, it should be remembered that this type of therapy is associated with the occurrence of characteristic side effects, which include meningitis, encephalopathy, as well as symptoms of motor neurons.

NFZ

Category:

Infectious diseases