Schilder's disease - a rare disease of the nervous system characterized by progressive and massive cerebral sclerosis.Disease occurs mainly in children, but can occur in other age groups.
causes of the disease is not known.It is assumed that the main role is played by a combination of genetic predisposition and a number of external factors.The latter include infectious diseases (measles, flu, sore throat, tuberculosis), endocarditis, trauma (including generic), birth asphyxia, congenital infections (syphilis, gonorrhea).
disease is based on the process of demyelination of nerve cells, that is, the loss of the myelin sheath, the carrier function of the transmission of a nerve impulse.Demyelinated cells can no longer function properly, leading to the gradual replacement of nerve cells are not fulfilling their fu
clinical picture Schilder's disease is very diverse and has many faces.In early childhood, it can be shown development of spastic paralysis (paralysis is not sluggish, spasmatic muscles), mental disorders and sometimes inflammation of the optic nerve.
In addition, the clinical manifestations of Schilder's disease may include:
Due to such a diverse clinical differential diagnosis of Schilder's disease is rather complicated.
First of all, you want to exclude an inflammatory disease of the brain, and it can be done with a lumbar puncture - spinal fluid Schilder's disease, as a rule, is not changed.In establishing the correct diagnosis can help histological examination of the affected area of the brain, as well as methods of radiation diagnosis.
flow Schilder's disease is typically progressive, with rare and short-lived recessions main symptoms.
Cases like lightning development of the disease for several days, and prolonged course, lasting 10-15 years.The average life expectancy with the disease Schilder after the establishment of an accurate diagnosis from a few months to 3-5 years.
forecast a very serious, effective treatments that slow the process of development, does not exist yet.Immunosuppressive drugs and interferons can achieve a reduction of symptoms and even small periods of relative remission.Most of the treatment is aimed at reducing human suffering and struggle with comorbidities.
are currently under active investigation Schilder's disease at the National Institute of Neurology and Stroke, in the United States.The program of studies appears to study the nature of the disease, prevention methods, and the search for effective ways to treat it.
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