C hronic Inflammatory Demyelinating Polyneuropathy

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.