慢性炎癥性脫髓鞘性多發(fā)性神經(jīng)根神經(jīng)病進(jìn)展

1、Chronic Inflammatory Demyelinatin Polyradiculoneuropathy: update on clinical features, phenotypes and treatment options,Department of Neurology, Fujian Provincial HospitalXingyong Chen,Introduction,Traditionally, chr

2、onic inflammatory demyelinating polyradiculoneuropathy (CIDP) has been considered a heterogeneous disorder including a broad spectrum of clinical phenotypes. The European Federation of Neurological Societies/Peripheral

3、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 predomi

4、nantly affect males, and typically occur in middle to old ageCIDP 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,C

5、linical 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 ev

6、olves 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

7、 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 weakness

8、Severe 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 w

9、hich 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 o

10、f CIDP is variabledetermined by the number and distribution of the demyelinating peripheral nerve lesions,SM: sensory and motor. PE: plasma exchange; RR:relapsing–remitting; CS:corticosteroids;,Atypical CIDP,,Distal par

11、esthesia 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 d

12、ependent axonal neuropathy.,DML: distal motor latency,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

13、 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, especiall

14、y 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 li

15、mb (EFNS).,Associated conditions,CIDP may be associated with various diseases, such as infection with the human immunodeficiency virus or hepatitis C, Sjögren's syndrome, inflammatory bowel disease, melanoma, ly

16、mphoma, diabetes mellitus, and IgM, IgG, or IgA monoclonal gammopathy of unknown significance.The pathogenetic relevance of such concurrent diseases is unclear,update,Anti-neurofascin(神經(jīng)束蛋白) IgG4 antibodies were associa

17、ted 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

18、 the splenium of the corpus callosum(胼胝體壓部). Fluid-attenuated inversion recovery images in patients 10 and 31 showed multiple sclerosis–like lesions in the juxtaventricular（腦室旁） regions.,Therapeutic options for CIDP,The

19、 acquired chronic demyelinating neuropathies include ：,chronic inflammatory demyelinating polyneuropathy (CIDP) neuropathy associated with monoclonal IgM antibodies to myelin-associated glycoprotein (MAG; anti-MAG neuro

20、pathy) 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

21、 suggested diagnostic pathway for chronic acquired demyelinating polyneuropathies. Differential diagnosis is based on electrodiagnostic studies, biopsies, serum biomarkers and antibodies. Abbreviations: CIDP, chronic inf