C hronic Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a sporadic acquired disorder which may mimic an inherited neuropathy in childhood. In fact, the commonest chronic neuropathy seen in children is a hereditary motor and sensory neuropathy (HMSN) type I. Evidence of familial involvement is perhaps the single most important characteristic in distinguishing hereditary from acquired disorders in children. It is important to recognize the acquired CIDP since it is potentially a treatable disease and its diagnosis may be suggested by clinical, electrophysiological and nerve biopsy features.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is distinguished from the more common acute demyelinating neuropathy, the Guillain-Barre syndrome (GBS), chiefly by clinical course and prognosis. On the one hand, both disorders have similar clinical features, and both share the CSF albumin-cytological dissociation and the pathological abnormalities of multi- focal inflammatory segmental demyelination with associated nerve conduction features reflecting demyelination. An autoimmune basis is suspected for both CIDP and GBS. On the other hand, CIDP has a more protracted clinical course, is rarely associated with preceding infections in children and responds to corticosteroid therapy. In addition, CIDP has an association with HLA antigens as well as an association with the M-phenotype of alpha-one antitrypsin deficiency.
The clinical course can follow several patterns. The onset is usually gradual but there was around 15% rapid rate of onset in a series of 92 patients. It also showed that 65% have a relapsing course compared to 35% with progressive or monophasic course. There was a significantly earlier age of onset in patients with relapsing disease, a finding similar to that in multiple sclerosis. In fact, some authors have suggested that CIDP syndrome may be the peripheral nervous sys…

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