Peripheral Neuropathies
Peripheral neuropathies include disorders that damage the peripheral nerves—those lying outside the brain and spinal cord. Peripheral nerves stimulate muscle movement, transmit sensation and control involuntary activities, such as blood pressure and heartbeat. When they don’t function properly, it results in muscle weakness, loss of muscle function, and some loss of sensory nerve function. Disorders of the peripheral nerves are frequently inherited, with symptoms developing during childhood or adolescence. Children and teens who have peripheral neuropathies usually live full lives, though the disorders require lifelong management.
Charcot-Marie-Tooth Disease
The most common inherited neuropathy, Charcot-Marie-Tooth Disease (CMT) affects one in 2,500 children in the U.S. It occurs when nerves in the extremities deteriorate, causing the muscles to weaken from lack of stimulation. Patients gradually lose normal use of their feet and legs, and hands and arms, as nerve degeneration progresses. Many patients also have some loss of sensory nerve function. CMT is not life-threatening and rarely affects brain function.
Dejerine-Sottas Syndrome
Dejerine-Sottas Syndrome (DSS) is a severe form of CMT that typically develops in infancy. Children who have DSS experience delayed motor milestones and severe motor, sensory and skeletal defects. Symptoms vary widely among affected children—some may require a wheelchair before they reach adolescence, while others may need milder walking support. DSS typically progresses more rapidly than other forms of CMT.
Friedreich’s Ataxia
Friedreich’s ataxia (FA) is a rare disease that mainly affects the nervous system and the heart. Common symptoms include muscle weakness and ataxia—difficulty maintaining balance and coordination. It does not impact cognition or brain development. Effects on the heart range from mild, nonsymptomatic abnormalities to life-threatening problems that require surgery or other treatment.
Web Resources
Charcot-Marie-Tooth Association
http://www.charcot-marie-tooth.org
Muscular Dystrophy Association
Information and Referrals
Gillette provides treatment and services for Charcot-Marie-Tooth disease and other peripheral neuropathies through the Neuromuscular Clinic. For more information about the clinic, call 651-312-3176. To refer a patient to Gillette’s neuromuscular clinic, please call: 1-800-719-4040, ext. 3944, or 651-290-8707.
