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Subacute sclerosing panencephalitis: causes, symptoms, diagnosis, treatment
Last reviewed: 05.07.2025
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Subacute sclerosing panencephalitis (synonyms: Van Bogaert's leukoencephalitis, Pette-Doering nodular panencephalitis, encephalitis with Dawson inclusions).
ICD-10 code
A81.1. Subacute sclerosing panencephalitis.
Epidemiology of subacute sclerosing panencephalitis
The age of the patients ranges from 4 to 20 years, and males predominate. The main preventive measure is vaccination against measles. In those vaccinated, the incidence of subacute sclerosing panencephalitis decreases by 20 times.
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What causes subacute sclerosing panencephalitis?
Subacute sclerosing panencephalitis is caused by the measles virus, which was found in the brain tissue of patients. This encephalitis affects children and adolescents who have had measles in the first 15 months of life. The incidence is 1 case per 1 million population.
Pathogenesis of subacute sclerosing panencephalitis
The pathogenesis of subacute sclerosing panencephalitis is based on the persistence and reproduction of the measles virus after the disease in the cells of the brain, due to the disruption of immunological mechanisms. Reproduction of the virus in the central nervous system is accompanied by activation of autoimmune processes, leading to damage and death of neurons. In the brain, a picture of encephalitis with the presence of glial nodules ("nodular panencephalitis"), demyelination in the subcortical formations (leukoencephalitis) is found. Changes are localized mainly in the gray and white matter of the cerebral hemispheres, brainstem and cerebellum. Microscopically, perivascular lymphomonocytic infiltration, damage and degenerative changes in neurons, proliferation of glia are detected.
Symptoms of subacute sclerosing panencephalitis
The incubation period of subacute sclerosing panencephalitis is from 3 to 15 years or longer. Subacute sclerosing panencephalitis usually develops between the ages of 4 and 20. The neurological status is characterized by motor disorders in the form of hyperkinesis, changes in muscle tone, trophic disorders, autonomic disorders, and epileptic seizures. Along with this, rapidly progressing dementia develops.
There are 4 stages of the disease.
- Stage I (lasts 2-3 months) is characterized by malaise, emotional lability, neurosis-like symptoms of subacute sclerosing panencephalitis in the form of increased irritability, anxiety, and sleep disorders. The patient's behavior changes (leaving home, psychopathic-like reactions). By the end of this stage, drowsiness increases, speech disorders (dysarthria and aphasia) are detected; movement coordination disorders - apraxia, writing disorders - agraphia, agnosia, the level of intelligence gradually decreases, memory loss progresses.
- Stage II of the disease manifests itself in various forms of hyperkinesis in the form of twitching of the entire body, head, limbs, myoclonus. Then generalized epileptic seizures and pyramidal symptoms join these disorders. The following symptoms of subacute sclerosing panencephalitis appear: diplopia, hyperkinesis, spastic paralysis, the patient stops recognizing objects, vision loss is possible.
- Stage III of the disease (6-8 months from the onset of the disease) is characterized primarily by severe breathing and swallowing disorders, hyperthermia and involuntary screaming, crying, and laughter.
- Stage IV: symptoms of subacute sclerosing panencephalitis such as opisthotonus, decerebrate rigidity, flexion contractures, and blindness are added.
At the end of the disease, the patient falls into a comatose state, trophic disorders appear. The disease ends in death, which occurs no later than 2 years from its onset. Chronic forms are rarer. In these cases, various forms of hyperkinesis of increasing severity and dementia are detected over 4-7 years.
Approximate periods of incapacity for work
Approximate periods of disability - from the time of manifestation of the disease.
Clinical examination
Medical supervision by a neurologist for the entire period of illness.
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Diagnosis of subacute sclerosing panencephalitis
The diagnosis of subacute sclerosing panencephalitis is based on the detection of high levels of antibodies to the measles virus in the blood and cerebrospinal fluid. EEG usually shows nonspecific changes.
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Differential diagnosis of subacute sclerosing panencephalitis
Subacute sclerosing panencephalitis is differentiated from other viral and prion slow infections.
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Indications for consultation with other specialists
If neurological symptoms dominate, a consultation with a neurologist is indicated; if mental disorders dominate, a consultation with a psychiatrist is indicated.
Indications for hospitalization
Indications for hospitalization are clinical (progressive mental disorders and neurological symptoms).
How to examine?
Treatment of subacute sclerosing panencephalitis
In stage I the regime is home, in stage II - semi-bed, in stages III-IV - bed.
No special diet is required. In later stages - parenteral and tube feeding.
Drug treatment of subacute sclerosing panencephalitis is symptomatic.
Prognosis of subacute sclerosing panencephalitis
Subacute sclerosing panencephalitis always has an unfavorable prognosis.