Distal acquired demyelinating symmetric neuropathy (DADS)

Distal acquired demyelinating symmetric neuropathy

What is distal acquired demyelinating symmetric neuropathy (DADS), and how does it relate to anti-myelin-associated glycoprotein (anti-MAG) neuropathy? 

Distal acquired demyelinating symmetric neuropathy (DADS) is a length-dependent sensory or sensorimotor neuropathy with demyelinating features on electrodiagnostic studies (typically prolonged distal motor latencies) and associated with an IgM paraprotein (usually with a kappa light chain) in about two-thirds of patients.

When an IgM paraprotein is present with DADS, several features are typical. Patients are usually men in their 60s or 70s with predominantly large fiber sensory loss in their distal lower extremities.

Motor involvement occurs to a lesser extent as the disease progresses. Patients have significant gait ataxia and may manifest hand tremors.

In about 50% of patients with DADS and IgM paraprotein, the paraprotein reacts against MAG, which is thought to interfere with Schwann cell–axon interactions.

In general, patients with DADS and IgM paraprotein are poorly responsive to immunomodulatory treatments but benefit in some patients given IVIG, rituximab, cyclophosphamide, or fludarabine has been reported.

The DADS pattern without an IgM paraprotein is a nonspecific phenotype that can represent many different neuropathies but CIDP should be considered.

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