CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY

     Chronic inflammatory demyelinationg polyradiculoneuropathy (CIDP) is a slowly progressive disorder of nerve cells which manifests as a symmetric neuropathy of the upper and lower extremities.  It is similar to Guillain-Barre syndrome in its autoimmune etiology; however, it differs in that it follows a subacute course developing over weeks. This condition can present at any age but usually in the fifth or sixth decades of life and may follow a relapsing-remitting course. Cerebral spinal fluid examination reveals few to no cellular components (a lack of pleocytosis) and an elevated protein although a normal protein level does not exclude the diagnosis.  Treatment may be offered with therapeutic plasma exchange plus prednisone and/or intravenous immune globulin (IVIG).  Plasma exchange therapy does not cure the disease, rather it lessens the symptoms; therefore, plasma exchange must be repeated (usually QOD).  Exchange therapy may be administered alone or with prednisone (oral dose of 60 mg/day for one months duration followed by a tapering regimen) or IVIG (IVIG therapy should come after plasma exchange to avoid removing IVIG during exchange therapy).  Response to therapy may not be immediate; therefore, therapy with IVIG should be continued for at least two months, prednisone for at least one month, and at least six plasma exchanges should be administered before judging response to therapy.  After successful therapy, patients must be followed and monitored for signs of recurrence.