慢性炎症性脱髓鞘性多发性神经根神经病最新进展

Chronic Inflammatory Demyelinatin Polyradiculoneuropathy: update on clinical features, phenotypes and treatment options,Department of Neurology, Fujian Provincial HospitalXingyong Chen,Introduction,Traditionally, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been considered a heterogeneous disorder including a broad spectrum of clinical phenotypes. The European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) CIDP treatment guideline has defined several clinical pictures as atypical CIDP phenotypes, additional to the classical (typical) picture of CIDP,prevalence of around 6 cases per 100,000 predominantly affect males, and typically occur in middle to old ageCIDP can also occurin children. The incidence of CIDP increases with age, rising to 1.5 times the overall average in people over 65years of age,Clinical presentation,Classic CIDP is characterized by the occurrence of symmetrical weakness in both proximal and distal muscles, impaired sensation and parasthesia and absent or diminished tendon reflexesThe disease evolves over more than 8 weeks, thus distinguishing the condition from GBS which has an acute onset.The time course maybe relapsing, chronic progressive, monophasic or GBS like onset.,Newly recognised clinical features in CIDP,Acute onset of CIDP may occur in up to 18% of CIDP patients, resembling the GBSdiagnosis was changed to CIDP in 5% of GBS patientsFatigue can be the main complaint in CIDP patients(75%)Activity-induced weaknessSevere pain is present in only a minority of CIDP patients;Tremor can be a disabling symptom in CIDP (50%)a higher incidence of almost 40% of restless legs syndrome in patients with CIDPAutonomic symptoms : 23%, of which gastrointestinal and genitourinary symptoms were most frequent (mild)severe autonomic dysfunction should be regarded as a red flag when considering the diagnosis of CIDP.,CIDP PHENOTYPES,The clinical presentation of CIDP is variabledetermined by the number and distribution of the demyelinating peripheral nerve lesions,SM: sensory and motor. PE: plasma exchange; RR:relapsingremitting; 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.,DML: distal motor latency,The LewisSumner 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,CIDP may be associated with various diseases, such as infection with the human immunodeficiency virus or hepatitis C, Sjgrens syndrome, inflammatory bowel disease, melanoma, lymphoma, diabetes mellitus, and IgM, IgG, or IgA monoclonal gammopathy of unknown significance.The pathogenetic relevance of such concurrent diseases is unclear,update,Anti-neurofascin(神经束蛋白) IgG4 antibodies were associated with a subgroup of patients with CIDP showing a younger age at onset, ataxia, tremor, CNS demyelination, and a poor response to IV immunoglobulin,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 sclerosislike lesions in the juxtaventricular（脑室旁） regions.,Therapeutic options for CIDP,The acquired chronic demyelinating neuropathies include ：,chronic inflammatory demyelinating polyneuropathy (CIDP) neuropathy associated with monoclonal IgM antibodies to myelin-associated glycoprotein (MAG; anti-MAG neuropathy) multifocal motor neuropathy (MMN) POEMS syndrome. They have characteristic-though overlapping-clinical presentations, are mediated by distinct immune mechanisms, and respond to different therapies.,Figure 1 | A suggested diagnostic pathway for chronic acquired demyelinating polyneuropathies. Differential diagnosis is based on electrodiagnostic studies, bi