Chronic inflammatory demyelinating polyradiculoneuropathy

6.09.2.3.2.1 Overview

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic demyelinating disease of the PNS that can involve sensory components, motor components, or both. The duration of the onset phase is generally more than 8 weeks; however, some CIDP patients show acute onset, as with GBS. It is characterized by progressive or relapsing-remitting weakness and numbness. There is a high incidence of fatigue that may persist for years. Electrophysiological features of CIDP include nerve conduction block and slowed conduction velocities resulting, most likely, from demyelination.

CIDP is a heterogeneous disorder with disagreement on which diseases to include under the CIDP umbrella (see Table 1), and a lack of clarity with regard to diagnostic criteria. In most subtypes, the symptoms are symmetrical. However, the Lewis-Summer motor sensory subtype is an asymmetric form with persistent conduction block at individual sites over long periods. Multifocal motor neuropathy (MMN), a motor form, is also asymmetric, and can show some sensory loss. Conversely, the sensory form of CIDP shows evidence of motor involvement. One of the subtypes, diabetes-associated CIDP, arises secondarily in up to 17% of patients with either type 1 or type 2 diabetes mellitus (see 6.19 Diabetes/Syndrome X).

CIDP is most common in the fifth and sixth decades, but may occur at any age. The MMN subtype is more common in men than women (2.6:1). A better outcome is reported to be related to younger age at onset, a relapsing-remitting course, and the absence of axonal damage.

6.09.2.3.2.2 Pathogenesis

Like MS, CIDP has a relapsing or progressive course, focal demyelination, axon degeneration that can occur early or late in the disease course, and an immune-mediated pathophysiology.

CIDP is thought to be an autoimmune disorder caused by either humoral or cell-mediated immunity to axonal, myelin, or other Schwann cell antigens. The autoantigens associated with CIDP are generally unknown. However, MMN is associated with anti-GM1 IgM antibodies, the levels of which decrease with clinical improvement.

Evidence for an autoimmune basis for CIDP is centered mainly on its general responsiveness to immunotherapy, and the fact that an inflammatory response is observed at the site of the disease. Unlike GBS, CIDP is rarely preceded by infection.

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