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Concept
Chronic inflammatory demyelinating polyneuropathy
Summary
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired autoimmune disease of the peripheral nervous system characterized by progressive weakness and impaired sensory function in the legs and arms. The disorder is sometimes called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating polyradiculoneuropathy (because it involves the nerve roots). CIDP is closely related to Guillain–Barré syndrome and it is considered the chronic counterpart of that acute disease. Its symptoms are also similar to progressive inflammatory neuropathy. It is one of several types of neuropathy.
Several variants have been reported. Specially important are:
An asymmetrical variant of CIDP is known as Lewis-Sumner Syndrome or MADSAM (multifocal acquired demyelinating sensory and motor neuropathy).
A variant with CNS involvement named combined central and peripheral demyelination (CCPD). This variant is special because it belongs at the same time to the CIDP syndrome and to the multiple sclerosis spectrum. These cases seem to be related to the presence of anti-neurofascin autoantibodies.
Chronic inflammatory demyelinating polyneuropathy (or polyradiculoneuropathy) is considered an autoimmune disorder destroying myelin, the protective covering of the nerves. Typical early symptoms are "tingling" (sort of electrified vibration or paresthesia) or numbness in the extremities, frequent (night) leg cramps, loss of reflexes (in knees), muscle fasciculations, "vibration" feelings, loss of balance, general muscle cramping and nerve pain. CIDP is extremely rare but under-recognized and under-treated due to its heterogeneous presentation (both clinical and electrophysiological) and the limitations of clinical, serologic, and electrophysiologic diagnostic criteria. Despite these limitations, early diagnosis and treatment is favoured in preventing irreversible axonal loss and improving functional recovery.
There is a lack of awareness and treatment of CIDP.
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