Dejerine-Sottas Disease

Dejerine-Sottas disease is a rare, genetic disorder affecting the body’s nerves characterized by difficulty or inability to move. The protein layer covering the nerves called the myelin sheath is damaged, affecting the functioning of these nerves.

Some of the main symptoms of Dejerine-Sottas disease include:

• Loss of sensation in the lower legs, arms and feet
• Weakness of the limbs and arms
• Deformities in the foot
• Delayed acquisition of motor skills
• Mild loss of hearing
• Limitation in eye movements
• Reduced muscle tone

This condition can be diagnosed from the symptoms as well as your child’s medical and familial history. Other tests such as nerve conduction velocity and electromyogram can confirm the diagnosis of the condition.

Dejerine-Sottas disease cannot be cured; however the progression of this condition can be controlled with the help of physical therapy (strengthening exercises for your arms and limbs), use of special shoes to provide support to the ankles, and medications to reduce pain. Patients with the disorder may also require wheel chairs or crutches as the disease progresses.