Autoimmune Diseases Polyneuropathy Handbook

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Neuropathy CIDP

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Guillain-Barre syndrome an important clinical diagnosis because: (1) it represents about one third of all initially undiagnosed acquired neuropathies;(2) most patients with Chronic Inflammatory Demyelinating Poly neuropathy will respond to immunosuppressive therapy,  relapses are common.

Clinical features of neuropathy
Patients with Chronic Inflammatory Demyelinating Poly neuropathy usually present with progressive, step-wise or repeated attacks of  muscle weakness that are present for at least two months.  Sensory complaints usually consists of numbness and tingling; painful paresthesias are uncommon. The peak incidence is 40-60 years of age.

Neurologic exam usually reveals proximal muscle weakness, sometimes even more affected than distal musculature. Both upper and lower extremities are involved, although the legs are usually affected more severely. Neck flexor weakness distinguishes CIDP from most other neuropathies. Cranial nerves are usually spared, although facial muscles may be affected. Ophthalmoplegia in CIDP is rare and the prevalence ranges from 3 to 8 percent in several case series. Of note, the ophthalmoplegia may precede the systemic weakness by days to months. Deep tendon reflexes are absent or depressed. Sensory findings are typically mild and often include impaired touch and vibratory sensation, with less involvement of small-fiber sensation (pain and temperature).

James Austin first documented the steroid responsiveness of Chronic Inflammatory Demyelinating Poly neuropathy in 1958. More recent randomized controlled studies have documented that prednisone and plasma exchange are both beneficial.

Plasma exchange performed twice weekly for 3 weeks generally result in transient improvement in both progressive and recurrent cases.

Intravenous immune globulin (IVIG) has also been found to be beneficial in both CIDP & GBS type polyneuropathies.

Some advocate initiation of single, daily-dose prednisone until patients show significant improvement. The mean time for initial response is two months. After attaining maximum benefit (usually 6-12 months), prednisone is slowly tapered. Unfortunately, the tapering of prednisone may result in a relapse of CIDP. The addition of azathioprine offers no advantage over prednisone alone. The possible adverse effects of long-term steroid use must also be considered. The use of high-dose intravenous steroids has only recently been investigated. 

A recent report suggests the possible benefit of interferon-alpha 2A in CIDP patients. The authors  postulate that interferon-alpha may modulate proinflammatory cytokines that have a role in immune-mediated demyelination.

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