scholarly journals A Severe Course of Relapsing-Remitting Acute-Onset Chronic Inflammatory Demyelinating Polyneuropathy in a Young Man

2021 ◽  
pp. 72-76
Author(s):  
Anna P. Patnaik ◽  
Joseph Mininni ◽  
Neil C. Porter ◽  
Nicholas A. Morris

Acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) is an immune mediated neuropathy characterized by progressive weakness and sensory impairment lasting over 2 months. Guillain-Barré-Strohl syndrome (GBS) is an immune mediated polyneuropathy with a similar presentation often over less than 4 weeks. While some have argued for the existence of recurrent GBS, most classify the syndrome as a form of relapsing-remitting CIDP. However, there are cases of GBS with treatment-related fluctuations that must be distinguished from A-CIDP as patients with A-CIDP require long-term immunotherapy. In this case report, we discuss a patient with multiple relapses over 3 years, who is more likely to have A-CIDP. His ganglioside profile, which has rarely been reported in A-CIDP, included high concentrations of anti-GM1, anti-GD1a, and anti-GD1b antibodies, which may account for his severe disease course.

2015 ◽  
Vol 10 (01) ◽  
pp. 85 ◽  
Author(s):  
Antonios Kerasnoudis ◽  
Kallia Pitarokoili ◽  
Ralf Gold ◽  
Min-Suk Yoon ◽  
◽  
...  

History-taking and nerve conduction studies are fundamental for the diagnosis and assessment of the severity of acute (AIDP) or chronic inflammatory demyelinating polyneuropathy (CIDP). The diagnostic challenge of distinguishing these two immune-mediated subacute polyradiculoneuropathies remains high, as intravenous immunoglobulin and steroids exert short-term clinical improvement in the majority of the CIDP cases, whereas steroids have no effect on AIDP patients. Accordingly, the precise classification of subacute polyradiculoneuropathies significantly affects the early application of steroids in CIDP. This review aims to give a timely update on the application of clinical, electrophysiological and nerve ultrasound parameters in distinguishing subacute CIDP from AIDP.


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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