C hronic Inflammatory Demyelinating Polyneuropathy

A detailed Summary of C hronic Inflammatory Demyelinating Polyneuropathy

CHRONIC 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.

Some patients may present with predominantly multi- focal motor signs masquerading as motor neuron disease. Electrophysiological studies demonstrating multi- focal conduction block confined to motor axons distinguish this acquired, demyelinating, multifocal motor neuropathy from motor neuron disease. The latter may be associated with high serum antibody titers to gangliosides and has been reported to respond to immunosuppression with cyclophosphamide. A CIDP-like syndrome may develop in cases of inherited neuropathy. These patients typically have a positive family history of affected kin, and have bony abnormalities such as pes cavus and hammer toes since an early age, but develop subacute deterioration with proximal muscle weakness and raised CSF protein. The newly acquired symptoms respond to corticosteroid therapy, hence the term prednisone-responsive hereditary motor and sensory neuropathy.

The disease is seen in all age groups including the first year of life. In adults there may be a history of preceding infection, immunization, or injection of foreign material within one month before the onset of the symptoms. However, this is not usually the case in children. The majority of patients have symmetrical motor and sensory involvement, although occasionally cases with predominantly motor or p

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Category: Science