ABSTRACT

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune mediated neuropathy. The characteristic clinical picture is one of slowly progressive weakness and sensory loss. Muscle stretch reflexes are depressed or absent. The diagnosis is supported by findings of elevated CSF protein, demyelinating electrodiagnostic features and an abnormal nerve biopsy. There are several lines of evidence that suggest CIDP is an autoimmune disorder involving autoreactive antibodies and T cells. Beneficial treatments include corticosteroids, intravenous immunoglobulin and plasma exchange. Patients with a progressive course who are refractory to such traditional therapy may be candidates for a more aggressive treatment approach involving high dose immune suppression followed by hematopoietic stem cell transplantation (HSCT).