Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Children with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) often have a presentation similar to AIDP; however, the disorder continues with a chronic or relapsing course.
The disorder may begin as early as infancy, but is seen in children and adults. Electrophysiologic studies show focal conduction block, temporal dispersion of CMAPs, prolongation of distal motor latencies, markedly slow conduction velocities, and absent or prolonged H-wave and F-wave latencies. CIDP cases often demonstrate axonal loss on EMG. The CSF protein is elevated in most cases.The differential diagnosis usually includes CMT types I and III. The presence of acute relapsing episodes point towards CIDP. Due to the more severe involvement of proximal nerves and nerve roots, a distal sural nerve biopsy may not always show inflammatory changes and demyelination.
Treatment may include corticosteroids (prednisone) and IVIG as first-line approaches and subsequently plasma exchange.
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