- Schilder disease - causes and symptoms
- Schilder disease - diagnosis
- Schilder disease or multiple sclerosis?
- Schilder disease - treatment
- Schilder disease - prognosis
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Schilder disease is also known as multiple sclerosis - an acute form of multiple sclerosis. It is one of the very rare degenerative diseases that begin in childhood. It is described as myelinoclastic multiple sclerosis. It's more common in boys than girls, usually between the ages of 7 and 12. Find out what the symptoms of Schilder disease are and what the prognosis is.
Schilder diseasewas first described in 1912, but it was not until 1986 that the criteria for diagnosing the disease were described and agreed. Schilder disease is considered to be one of the varieties of multiple sclerosis.
It takes the form of acute or subacute demyelinating disorders of the central nervous system. It mainly affects young patients.
The namesdiffuse brain sclerosisordiffuse multiple sclerosis .
are also used to describe Schilder disease.Schilder disease - causes and symptoms
The exact causes of Schilder disease are unknown. The disease often appears after an infectious disease. The symptoms of Schilder disease are very nonspecific and easy to underestimate. Usually there are:
- headaches
- fever
- feeling of general fatigue and discomfort
These symptoms are associated with extensive demyelination, which is the loss of the myelin sheath around neurons in the brain and spinal cord. Loss of myelin results in a significant slowdown in neuronal transmission.
Currently, it is suggested that L-forms of bacteria may be responsible for such a process, but this requires further research.
As the disease develops, the process of demyelination covers more and more extensive areas of the brain, and this contributes to movement disorders, speech disorders, hearing disorders, and visual disturbances. The progression of the disease can affect your heart rate, blood pressure, and breathing.
This disease should not be confused with Addison-Schilder disease, a rare genetic disorder that is associated with an impairment of the biochemical pathways in myelin.
Schilder disease - diagnosis
To be diagnosed with Schilder Disease, a patient must meet the following criteria, known asPoser criteria :
- Presence of clinical criteria andsymptoms unusual for the early stage of multiple sclerosis.
- Normal CSF composition or a composition unusual for multiple sclerosis.
- Presence of one or two symmetrical plaques measuring at least 3 cm and covering the central part of the cerebral hemispheres (which is visualized by medical imaging).
- No viral or mycoplasmal infection.
- Normal blood serum levels of long-chain fatty acids.
- Normal kidney function.
Several widely available diagnostic tools can be helpful in diagnosing Schilder Disease.
Using computed tomography it is possible to visualize hypodense lesions (also referred to as "hypodense focus"). These are pathological changes with a reduced light attenuation factor.
Similar effects are obtained by using contrast MRI for examinations.
The localization of such changes is almost always subcortical and visible on T1 sequences.
The method of electroencephalography is very rarely used in the diagnosis of Schilder disease. It is only used when epileptic seizures are suspected or present.
Schilder disease or multiple sclerosis?
There are some similarities between Schilder disease and MS. In both conditions, the myelin sheath around nerve cells is lost, which affects the transmission of electrical impulses from neuron to neuron.
The symptoms of both diseases are also similar. They include, among others impairment of memory, speech and movement processes. Therefore, the diagnosis of Schilder disease must be carried out with special care.
Schilder disease - treatment
Treatment of Schilder's disease is symptomatic. Doctors after diagnosis recommend taking drugs from the group of corticosteroids. The administration of cyclophosphamide gives positive results.
In addition, conservative treatment, diet and physiotherapy are used in parallel.
There are indications of the efficacy of treating Schilder's disease with adrenocorticotropic hormone (ACTH). However, due to prohibitive costs associated with this type of treatment, it has not entered clinical practice for good.
The efficacy of intravenous immunoglobulin administration, interferon therapy or blood plasma exchange has not yet been proven.
Schilder disease - prognosis
The prognosis in the course of Schilder disease depends on the patient's response to the treatment. In most casesa clear clinical improvement is observed after the start of the therapy.
However, there are cases of deteriorating he alth, including increased physical and intellectual disabilities. However, they are rare.
The patient must be under constant medical care. He should be provided with adequate ventilation of the respiratory system, nutrition (often parenteral) and constant monitoring of electrolyte levels.
About the author
Anna Jarosz A journalist who has been involved in popularizing he alth education for over 40 years. Winner of many competitions for journalists dealing with medicine and he alth. She received, among others The "Golden OTIS" Trust Award in the "Media and He alth" category, St. Kamil awarded on the occasion of the World Day of the Sick, twice the "Crystal Pen" in the national competition for journalists promoting he alth, and many awards and distinctions in competitions for the "Medical Journalist of the Year" organized by the Polish Association of Journalists for He alth.
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