Subacute sclerosing panencephalitis: causes, symptoms, diagnosis, treatment

Subacute sclerosing panencephalitis (synonyms: Van-Bogart leucoencephalitis, Pette-Dering nodular panencephalitis, encephalitis with Dawson inclusions).

ICD-10 code

A81.1. Subacute sclerosing panencephalitis.

Epidemiology of subacute sclerosing panencephalitis

The age of the cases is from 4 to 20 years, the male is predominant. The main preventive measure is vaccination against measles. In vaccinated patients, the incidence of subacute sclerosing panencephalitis is reduced by a factor of 20.

[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12],

What causes subacute sclerosing panencephalitis?

Subacute sclerosing panencephalitis is caused by the measles virus, which was detected in the brain tissue of patients. Such encephalitis is affected by children and adolescents who have suffered measles in the first 15 months of life. The incidence is 1 case per 1 million population.

Pathogenesis of subacute sclerosing panencephalitis

At the heart of the pathogenesis of subacute sclerosing panencephalitis is the persistence and reproduction of measles virus after the transferred disease in the brain cells, due to the violation 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 there is a picture of encephalitis with the presence of glial nodules ("nodular panencephalitis"), demyelination in the subcortical formations (leukoencephalitis). Changes are localized primarily in the gray and white matter of the cerebral hemispheres, the brain stem and the cerebellum. Microscopically detect perivascular lymphomonocyte infiltration, damage and degenerative changes of neurons, proliferation of glia.

Symptoms of subacute sclerosing panencephalitis

The incubation period of subacute sclerosing panencephalitis is from 3 to 15 years and longer. Subacute sclerosing panencephalitis usually develops between the ages of 4 and 20 years. 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 progressive dementia develops.

There are 4 stages of the disease.

• I stage (lasts 2-3 months) characterizes malaise, emotional lability, neurosis-like symptoms of subacute sclerosing panencephalitis in the form of increased irritability, anxiety, and sleep disturbances. The patient changes behavior (leaving the house, psychopathic reactions). By the end of this stage, drowsiness develops, speech disorders (dysarthria and aphasia) are revealed; violations of coordination of movements - apraxia, writing disorders - agraphy, agnosia, the level of intelligence gradually decreases, memory loss progresses.
• II stage of the disease manifests itself in different forms of hyperkinesis in the form of twitching of the whole trunk, head, limbs, myoclonia. Then generalized epileptic seizures and pyramidal symptoms join these disorders. The following symptoms of subacute sclerosing panencephalitis appear : diplopia, hyperkinesis, spastic paralysis, the patient ceases to recognize objects, a loss of vision is possible.
• III stage of the disease (6-8 months from the onset of the disease), characterized primarily by severe breathing disorders, swallowing, hyperthermia and involuntary screaming, crying, laughter.
• Stage IV: Symptoms of subacute sclerosing panencephalitis, such as opisthotonus, decerebral rigidity, flexural contractures, and blindness, are added.

In the final illness the patient falls into a coma, there are trophic disorders. The disease ends with a lethal outcome, which occurs no later than 2 years from its inception. Chronic forms are more rare. In these cases within 4-7 years different forms of hyperkinesia of increasing severity and the phenomenon of dementia are revealed.

Approximate terms of incapacity for work

Approximate terms of incapacity for work - since the manifestation of the disease.

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Clinical examination

Medical supervision by a neurologist for the entire period of the illness.

[15], [16], [17], [18], [19], [20], [21], [22]

Diagnosis of subacute sclerosing panencephalitis

Diagnosis of subacute sclerosing panencephalitis is the detection of high levels of antibodies to measles virus in the blood and spinal cord fluid. On the EEG, nonspecific changes are usually detected.

[23], [24], [25], [26], [27], [28], [29], [30], [31]

Differential diagnosis of subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis is differentiated from other viral and prion slow infections.

[32], [33], [34], [35], [36]

Indications for consultation of other specialists

With the dominance of neurological symptoms, consultation of a neurologist is shown, with the domination of mental disorders - a psychiatrist.

Indications for hospitalization

Indications for hospitalization are clinical (progressive disorders of the psyche and neurological symptoms).

Treatment of subacute sclerosing panencephalitis

The regime in the first stage is home, in II - semi-bed, in III-IV stages - bed.

A special diet is not required. In the later stages - parenteral and probe nutrition.

Drug treatment of subacute sclerosing panencephalitis is symptomatic.

Prognosis of subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis has always an unfavorable prognosis.