Schilder's disease
Schilder's disease is a rare, progressive, and potentially fatal condition that affects the central nervous system. It is also known as myelinoclastic diffuse sclerosis, a term that refers to the destruction of the myelin sheath, a protective layer that covers nerve cells. This disease is often classified as a subtype of multiple sclerosis due to its similar symptoms and pathological features.
Etiology
The exact cause of Schilder's disease is unknown. However, it is believed to be an autoimmune disease, where the body's immune system mistakenly attacks its own tissues. Some researchers suggest that it may be triggered by a viral infection, but this theory has not been definitively proven.
Symptoms
Symptoms of Schilder's disease can vary widely among patients, but they generally include neurological problems such as difficulty with balance and coordination (ataxia), weakness, fatigue, and changes in behavior or personality. Other symptoms may include headache, nausea, and vomiting. In severe cases, patients may experience seizures and loss of consciousness.
Diagnosis
Diagnosis of Schilder's disease is challenging due to its rarity and the lack of specific diagnostic criteria. It is often diagnosed based on a combination of clinical symptoms, magnetic resonance imaging (MRI) findings, and the exclusion of other similar conditions. The MRI typically shows large, symmetrical lesions in the white matter of the brain.
Treatment
There is currently no cure for Schilder's disease, and treatment is primarily aimed at managing symptoms. This may include physiotherapy for mobility issues, medication for symptom control, and counseling for emotional and psychological support.
Prognosis
The prognosis for Schilder's disease is generally poor, with most patients experiencing progressive neurological decline. However, the course of the disease can vary widely, and some patients may have periods of stability or even improvement.
See also
| Demyelinating diseases of the central nervous system | ||||||||
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| Hypersensitivity and autoimmune diseases (279.5–6) | ||||||||||||||||
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NIH genetic and rare disease info
Schilder's disease is a rare disease.
| Rare and genetic diseases | ||||||
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Rare diseases - Schilder's disease
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Contributors: Prab R. Tumpati, MD