慢性炎症性脱髓鞘性多发性神经根神经病最新进展概要1ppt课件.pptxVIP

Chronic Inflammatory Demyelinatin Polyradiculoneuropathy: update on clinical features, phenotypes and treatment options;Introduction;prevalence of around 6 cases per 100,000 predominantly affect males, and typically occur in middle to old age CIDP can also occur?in children. The incidence of CIDP increases with age, rising to 1.5 times the overall average in people over 65?years of age ;Clinical presentation;Newly recognised clinical features in CIDP;CIDP PHENOTYPES;SM: sensory and motor. PE: plasma exchange; RR:relapsing–remitting; CS:corticosteroids; ;Atypical CIDP;Distal paresthesia and hypesthesia are the most frequent symptoms, followed by proprioceptive ataxia ;DADSP(distal acquired demyelinating symmetric polyneuropathy ) : distal or predominantly sensory large fibre neuropathy length dependent axonal neuropathy. ;The Lewis–Sumner syndrome (LSS), or multifocal acquired demyelinating sensory and motor neuropathy (MADSAM), has been reported in up to 15% of patients fulfilling the EFNS/PNS criteria for CIDP ;The combination of symmetric pattern of weakness without bulbar involvement clinically distinguishes this phenotype from lower motor neuron disease, but distinction can be difficult, especially if electrophysiological criteria of demyelination are not met completely. ;Focal CIDP is defined as involvement of the brachial or lumbosacral plexus or of one or more peripheral nerves in one upper or lower limb (EFNS). ; Associated conditions;update;Diffusion-weighted images in patient 10 showed signal abnormalities in the splenium of the corpus callosum(胼胝体压部). Fluid-attenuated inversion recovery images in patients 10 and 31 showed multiple sclerosis–like lesions in the juxtaventricular（脑室旁） regions. ;Therapeutic options for CIDP;The acquired chronic demyelinating neuropathies include ：;Figure 1 | A suggested diagnostic pathway for chronic acquired demyelinating polyneuropathies. Differential diagnosis is based on electrodiagnostic studies, biopsies