DEJERINE SOTTAS DISEASE Meaning and
Definition
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Dejerine-Sottas Disease, also known as hereditary motor and sensory neuropathy type III (HMSN III), is a rare genetic disorder that affects the peripheral nerves. It is categorized as a type of Charcot-Marie-Tooth disease (CMT), which is a group of inherited conditions that damage the peripheral nerves responsible for transmitting signals from the brain and spinal cord to the muscles and sensory organs.
Individuals with Dejerine-Sottas Disease typically experience a progressive degeneration of the peripheral nerves, leading to muscle weakness, sensory loss, and impaired reflexes in the limbs. Symptoms often appear in early childhood or adolescence and worsen over time. The disease is caused by mutations in the genes responsible for the production of myelin, the protective covering of nerve fibers.
This condition leads to the thickening of nerve fibers, causing a decrease in nerve signal transmission efficiency. As a result, affected individuals may have difficulty walking, experience foot deformities, and often require assistive devices such as braces or orthopedic shoes. In severe cases, respiratory complications and other organ dysfunctions can occur.
While there is no cure for Dejerine-Sottas Disease, treatment focuses on managing symptoms and providing supportive care to improve quality of life. Physical therapy and assistive devices can help maintain mobility and minimize complications associated with muscle weakness. Genetic counseling is recommended for affected individuals and their families due to the hereditary nature of the disease. Research efforts continue to better understand the underlying genetic defects and develop potential therapies to alleviate the symptoms of Dejerine-Sottas Disease.
Common Misspellings for DEJERINE SOTTAS DISEASE
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