Definition:
Dejerine-Sottas disease is an uncommon genetic motor and sensory polyneuropathy that manifests progressive effects on mobility. The peripheral nerves thicken and enlarge, leading to weakness of the muscle. The progress of this disease is irregular, as well as frequently accompanied by numbness, pain, weakness, and a burning, prickling, or tingling sensations in one's legs.
Diagnosis:
MR imaging is believed to be the best technique choice for identifying Dejerine-Sottas disease. Analysis of medical history, physical examination, and lab tests are also done.
Treatment:
Treatment is aimed at slowing the progression of the disease. Symptoms and any underlying diseases are also treated to slow down progression. Medications are administered to treat the pain, which is one of the most affecting symptoms of the disease.
Symptoms and Signs:
The disease is generally characterized by the infantile start of moderate to serious upper and lower extremity weakness, as well as sensation loss. Symptoms are typically more rapidly progressive and severe compared to other common subtype Charcot-Marie-Tooth disorders. Some affected individuals may never be able to walk again, and be bound to the wheelchair by end of its first ten years; others might require only a walking stick or a comparable support throughout life.
Causes:
Dejerine-Sottas disease is a genetically transmitted disease, wherein the protective sheath surrounding the ‘long nerves' breaks down endangering and exposing the nerve. This break down or demylination occurs for unknown reasons.
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