What is Dejerine-Sottas disease?

Dejerene-Sottas (DS) is a subtype of Charcot-Marie-Tooth disease (CMT), a genetic, neurological disorder that causes damage to the peripheral nerves — tracts of nerve cell fibers that connect the brain and spinal cord to muscles and sensory organs. DS is named for Joseph Dejerine and Jules Sottas, French neurologists who first described the disorder in 1893.

What is Charcot-Marie-Tooth disease type 4 (CMT4)?

CMT4 is a subtype of CMT, a genetic, neurological disorder that causes damage to the peripheral nerves — tracts of nerve cell fibers that connect the brain and spinal cord to muscles and sensory organs.

What is Charcot-Marie-Tooth disease type X (CMTX)?

CMTX is a subtype of CMT, a genetic, neurological disorder that causes damage to the peripheral nerves — tracts of nerve cell fibers that connect the brain and spinal cord to muscles and sensory organs.

What are Charcot-Marie-Tooth disease type 1 (CMT1) and type 2 (CMT2)?

These are the two most common forms of CMT, a genetic, neurological disorder that causes damage to the peripheral nerves — tracts of nerve cell fibers that connect the brain and spinal cord to muscles and sensory organs.

A subtype of CMT1, called CMT1A (caused by a defect in the PMP22 gene on chromosome 17) accounts for around 60 percent of all CMT cases.

For axons and Schwann cells, communication is the key to a healthy relationship. Axons send chemical messages that attract Schwann cells and encourage myelin formation, and Schwann cells appear to send messages that nourish and protect axons. The various genetic defects that cause CMT often disrupt these interactions.

The many different types of Charcot-Marie-Tooth (CMT) are distinguished by age of onset, inheritance pattern, severity, and whether they're linked to defects in axon or myelin.

What is Charcot-Marie-Tooth disease (CMT)?

CMT causes degeneration of the peripheral nerves, leading to muscle weakness in the body’s extremities.