C hronic Inflammatory Demyelinating Polyneuropathy
CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHYChronic 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
Electrophysiological studies demonstrating multi- focal conduction block confined to motor axons distinguish this acquired, demyelinating, multifocal motor neuropathy from motor neuron disease. 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. However, this is not usually the case in children. Clinical improvement was often dramatic and there were no reported major adverse reactions to the infusions. In older children glue sniffing may produce the clinical features of CIDP and slow nerve conduction, however, nerve biopsy is usually diagnostic. In one series, ten patients presented in childhood, eight were followed for an average of 10 years, and 7 had no disability, one case had minor disability only. In addition, CIDP has an association with HLA antigens as well as an association with the M-phenotype of alpha-one antitrypsin deficiency. However, recently high doses intravenous immunoglobulin has been shown to be effective in the management of CIDP. Onion bulb formations, a sign of repeated episodes of segmental demyelination and remyelination, depend on chronicity. The disease is seen in all age groups including the first year of life. The majority of patients have symmetrical motor and sensory involvement, although occasionally cases with predominantly motor or predominantly sensory involvement may be seen. These signs provide helpful clinical clues to separate these patients from those with axonal neuropathies. Deep tendon reflexes are invariably depressed or absent at some stage of the disease.
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