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CIDP

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare acquired, symmetrical (occurs on both sides of the body), sensory-motor neuropathy (nerve disorder) and is considered to be the chronic variety of the Guillain-Barre syndrome (GBS).

Description

CIDP and GBS differ mainly in onset, course and prognosis. CIDP has a relapsing or steadily progressive course over months or years. Although remission may occur spontaneously with time, the disorder frequently follows a progressive course leading to severe functional disability.

Causes

The cause is unknown, but CIDP appears to be an immunologically mediated and acquired disorder.

Symptoms

Patients with CIDP have progressive weakness during a long period of time. The course may be stepwise or a continuous worsening over months or years. Most patients have symptoms related to muscle weakness and sensory loss, and the frequency of these is about equal. The symptoms are more frequent in the limbs than in the trunk. Patients with leg weakness show clumsy gait, tripping on uneven ground, and difficulty standing still, in stepping onto a curb, in going up or down stairs, or on arising from a seated position. Patients with hand and forearm weakness experience difficulty turning a key in a lock, picking up coins or a pencil, fastening buttons, and combing hair.

Diagnosis

Diagnosis is made on the basis of clinical symptoms and signs, electrodiagnostic studies to evaluate motor and sensory nerve conduction, and sometimes lumbar puncture and cerebrospinal fluid examination.

Treatment

Without treatment, chronic inflammatory demyelinating polyneuropathy is eventually fatal in up to 10 percent of patients. Untreated, the remainder suffer protracted and serious disability. Oral steroid therapy is the mainstay of treatment and often produces noteworthy improvements within three weeks. Prednisone is the treatment of choice. The results of therapy can be quite dramatic. Steroid therapy is highly effective in infants and children. Steroid therapy may need to be continued for years, and patients can relapse within a few months of withdrawing the medications. Patients unresponsive to steroids may benefit from a cytotoxic medication such as azathioprine. In addition to corticosteroids, patients with CIDP may respond to plasma exchange (plasmapheresis). Intravenous high-dose immunoglobulin infusions may also lead to improvement.