scholarly journals Intravenous Immunoglobulins (IVIG) in Chronic Inflammatory Demyelinating Polyneuropathy ( CIDP ): time to maximal recovery

Author(s):  
S Baker ◽  
A Opala

Background: The response of Chronic Inflammatory Demyelinating Polyneuropathy ( CIDP ) to Intravenous Immunoglobulins (IVIG) treatment is well established. However, determination whether patients who do not respond to 2 IVIG treatments or those whose condition stabilizes (ICE Trial) would benefit from additional treatments remains unclear. We aim to identify time period required to reach maximal strength gains from IVIG treatment (plateau). Furthermore, we will assess nerve conduction studies (NCS) changes over time with IVIG treatment. This will help in establishing a time course for treatment of CIDP with IVIG to maximize recovery. Methods: We performed a retrospective chart review of 27 patients with CIDP, with diagnosis confirmed by European Federation of Neurological Societies/Peripheral Nerve Society Guidelines (EFNS/PNS). Each patient’s strength response including: grip strength, knee extension, elbow flexion and dorsiflexion (using JAMAR Dynamometer) and NCS changes over time during IVIG treatment were analyzed. The primary outcome is duration of IVIG treatment, in months, required to reach a plateau in strength. Secondary outcome is NCS change including: Terminal Latencies, Conduction Velocities, Compound Sensory and Motor action potentials in nerves of upper and lower extremities over treatment time (emerging trends). Results: Pending (available by April 2015) Conclusion: Pending (available by April 2015)

Author(s):  
A Opala ◽  
S Baker

Background: The response of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) to Intravenous Immunoglobulins (IVIG) treatment is well established . However, determination if patients not responding to 2 IVIG treatments or those whose condition stabilizes (ICE Trial) may benefit from additional doses remains unclear. We aim to identify time period required to reach maximal strength gains from IVIG treatment. Methods: Retrospective chart review of 14 patients with CIDP was performed. Change in Grip strength (GS), Knee extension (KE), Elbow Flexion (EF) and Dorsflexion(DF) was analyzed with a dynamometer during IVIG therapy. Averages for : percent change from baseline(Max%Δ),cumulative grams(g) of IVIG and time in weeks(w) required for maximal strength recovery was determined per function (+/−SEM).Anciliary therapy for all patients was recorded. Results: Strongest improvement was observed for DF(124+/−30%,p<0.001), followed by KE(113+/−19%,p<0.01),GS(100+/−21%,p<0.001) and EF(98+/−14%p<0.05).GS improved the fastest(19.1+/−3w) followed by DF(29.5+/−7w),KE(29.6+/−4w) and EF(31+/−6w). Cumulative IVIG dose to reach Max%Δ was highest for EF(869+/−201g) and lowest for GS(573+/−78g). Conclusions: Our study has demonstrated effectiveness of multiple treatments with IVIG to reach significant improvement in strength. Different muscle groups manifested different time-dependency ,reflecting variable amounts of IVIG required. Improvement was identified to be present on a ongoing basis ,with therapy lasting between 19.1-31 weeks,requiring between 869-573g of IVIG.


Author(s):  
Umair J. Chaudhary ◽  
Yusuf A. Rajabally

Abstract Background The frequency and causes of underdiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) are uncertain. We aimed to assess the frequency and electroclinical features of pre-referral CIDP underdiagnosis and the duration of delay prior to diagnosis and treatment initiation in a tertiary specialist clinic. Methods We retrospectively investigated 60 consecutive patients attending our Inflammatory Neuropathy Service, between 2015 and 2019, with a final diagnosis of treatment-responsive definite/probable CIDP. We reviewed the clinical and electrophysiological data in light of European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines and determined the frequency, causes and delay in diagnosis of CIDP. Results An initial alternative diagnosis to that of CIDP had been made in 68.3% (41/60) of patients. The commonest alternative diagnosis was of Guillain–Barré syndrome (GBS) in 23.3% (14/60) patients. Non-GBS underdiagnoses (27/60; 45%) mainly consisted of genetic neuropathy (8/27; 29.6%), diabetic neuropathy (5/27; 18.5%) and chronic idiopathic axonal polyneuropathy (4/27; 14.8%). Non-GBS underdiagnoses were predominantly due to non-recognition of proximal weakness (70.4%), multifocal deficits (18.5%) or proprioceptive loss (7.4%). Electrophysiological misinterpretation was contributory to pre-referral non-GBS underdiagnoses of CIDP in 85% of patients. Mean diagnostic delay in patients with non-GBS underdiagnoses of CIDP was of 21.3 months (range 2–132 months). Conclusion Underdiagnosis of CIDP is frequent and may lead to significant diagnostic and treatment delay. We suggest that lack of comprehensive and precise attention to typical electroclinical features of CIDP and its diagnostic criteria at the time of initial evaluation are equally contributory to underdiagnoses.


Author(s):  
Adrian R. Opala ◽  
Kevin Kennedy ◽  
Steven K. Baker

ABSTRACTBackground:The response of chronic inflammatory demyelinating polyneuropathy (CIDP) to intravenous immunoglobulins (IVIgs) treatment is well established. However, it remains unclear whether patients not responding to two IVIg treatments or those whose condition stabilizes (ICE trial) may benefit from additional doses. We aim to identify the time period required to reach maximal strength gains from IVIg treatment.Methods:Retrospective chart review of 14 patients with CIDP was performed. Change in handgrip (HG), Knee extension (KE), elbow flexion, and dorsiflexion was analyzed with a dynamometer during IVIg therapy. Strength improvements in Nm or kg, cumulative grams (g) of IVIg, and time in days required for maximal strength recovery were determined per function (± standard error of the mean). Ancillary therapy was recorded for all patients.Results:Improvements in strength of each function were significant (p < 0.05). Earliest improvement was in HG (137.07 ± 21.23) and latest in KE (238.15 ± 38.9). Majority of patients improved by 200 days of therapy. HG required the lowest cumulative grams of IgG (561.71 ± 97.21) and KE the most (798 ± 120.7).Conclusion:Our study has demonstrated the effectiveness of multiple treatments with IVIg to reach significant improvement in strength. Different muscle groups manifested different time dependency, reflecting the requirement of variable amounts of IVIg. Improvement was identified on an ongoing basis, with therapy lasting between 20.2 and 37.3 weeks, requiring between 562 and 798 g of IVIg.


Medicina ◽  
2022 ◽  
Vol 58 (1) ◽  
pp. 110
Author(s):  
An-Che Cheng ◽  
Te-Yu Lin ◽  
Ning-Chi Wang

Antiretroviral therapy (ART) can restore protective immune responses against opportunistic infections (OIs) and reduce mortality in patients with human immunodeficiency virus (HIV) infections. Some patients treated with ART may develop immune reconstitution inflammatory syndrome (IRIS). Mycobacterium avium complex (MAC)-related IRIS most commonly presents as lymphadenitis, soft-tissue abscesses, and deteriorating lung infiltrates. However, neurological presentations of IRIS induced by MAC have been rarely described. We report the case of a 31-year-old man with an HIV infection. He developed productive cough and chronic inflammatory demyelinating polyneuropathy (CIDP) three months after the initiation of ART. He experienced an excellent virological and immunological response. Sputum culture grew MAC. The patient was diagnosed with MAC-related IRIS presenting as CIDP, based on his history and laboratory, radiologic, and electrophysiological findings. Results: Neurological symptoms improved after plasmapheresis and intravenous immunoglobulin (IVIG) treatment. To our knowledge, this is the first reported case of CIDP due to MAC-related IRIS. Clinicians should consider MAC-related IRIS in the differential diagnosis of CIDP in patients with HIV infections following the initiation of ART.


2021 ◽  
Vol 8 (5) ◽  
pp. e1031
Author(s):  
Gautier Breville ◽  
Agustina M. Lascano ◽  
Pascale Roux-Lombard ◽  
Nicolas Vuilleumier ◽  
Patrice H. Lalive

ObjectiveTo determine whether CSF interleukin 8 (IL-8) concentration can help to distinguish Guillain-Barré syndrome (GBS) from chronic inflammatory demyelinating polyneuropathy (CIDP) at the initial stage of the disease.MethodsWe performed retrospective immunoassay of IL-8 in CSF, collected at the University Hospitals of Geneva between 2010 and 2018, from patients diagnosed with GBS (n = 45) and with CIDP (n = 30) according to the Brighton and European Federation of Neurological Societies/Peripheral Nerve Society criteria by a physician blinded to biological results.ResultsCSF IL-8 was higher in GBS (median: 83.9 pg/mL) than in CIDP (41.0 pg/mL) (p < 0.001). Receiver operating characteristic analyses indicated that the optimal IL-8 cutoff was 70 pg/mL. Above this value, patients were more likely to present GBS than CIDP (specificity 96.7%, sensitivity 64.4%, positive predictive value [PPV] 96.7%, and negative predictive value [NPV] 64.4%). Among GBS subcategories, IL-8 was higher in acute inflammatory demyelinating polyneuropathy (AIDP, median: 101.8 pg/mL) than in other GBS variants (median: 53.7 pg/mL). In addition, with CSF IL-8 above 70 pg/mL, patients were more likely to present AIDP than acute-onset CIDP (p < 0.001; specificity 100%, sensitivity 78.8%, PPV 100%, and NPV 46.2%) or other CIDP with nonacute presentation (p < 0.0001; specificity 95.8%, sensitivity 78.8%, PPV 96.3%, and NPV 76.7%).ConclusionCSF IL-8 levels can help to differentiate AIDP variant of GBS from CIDP, including acute-onset CIDP, with high specificity and PPV. This may improve early and appropriate treatment.Classification of EvidenceThis study provides Class II evidence that CSF IL-8 levels accurately distinguish patients with GBS from those with CIDP.


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