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脑脊液检查结果与吉兰-巴雷综合征患者的临床特征、亚型和疾病过程的关系。

CSF Findings in Relation to Clinical Characteristics, Subtype, and Disease Course in Patients With Guillain-Barré Syndrome.

机构信息

From the Department of Neurology (H.A.-H., H.A., T.H.), Aarhus University Hospital, Denmark; Department of Neurology (A.Y.D., B.v.d.B., C.V., J.R., S.E.L., S. Arends, L.W.G.L., K.K., B.C.J.), Erasmus MC, University Medical Centre Rotterdam, the Netherlands; Department of Neurology (A.M.S.), University of Michigan School of Medicine, Ann Arbor; Department of Neurology (S.A.Z.), University of Pittsburgh Medical Center, PA; Department of Neurology (H.J.W., A.D.), College of Medical, Veterinary and Life Sciences, University of Glasgow, United Kingdom; Department of Neurology (D.R.C.), Johns Hopkins University School of Medicine, Baltimore, MD; Department of Neurology (K.C.G.), St. Elizabeth's Medical Centre, Tufts University, School of Medicine, Boston, MA; Laboratory of Gut-Brain Signaling (Z.I.); Laboratory Sciences and Services Division (LSSD) (Z.I.), icddr,b; National Institute of Neurosciences and Hospital (Q.D.M.), Dhaka, Bangladesh; Department of Neurology (S.H.S.), Odense University Hospital and University of Southern Denmark; Department of Neurology (S. Kusunoki), Kindai University Faculty of Medicine, Osaka-Sayama City, Japan; Department of Neurology (C.C.), Neuromuscular Unit, Bellvitge University Hospital-IDIBELL, CIBERER, Barcelona, Spain; Division of Neurology (K.B.), Department of Medicine, Groote Schuur Hospital, University of Cape Town, South Africa; Department of Neurology (J.A.L.M.), Royal Victoria Infirmary, Newcastle upon Tyne Hospitals NHS Foundation Trust, United Kingdom; Department of Neurology (B.v.d.B., H.K.), Franciscus Gasthuis & Vlietland (location: Vlietland Hospital), Schiedam; Department of Neurology (S. Arends, P.W.W.), Haga Hospital, Den Haag; Department of Neurology (L.W.G.L., L.H.V.), St. Elisabeth-TweeSteden Hospital, Tilburg, the Netherlands; Department of Neurology (L.B.), IRCCS Ospedale Policlinico San Martino, Genova, Italy; Department of Neurology (S. Kuwabara), Chiba University, Chuo-ku, Japan; Department of Neurology (P.V.d.B.), Neuromuscular Reference Centre, University Hospital Saint-Luc, University of Louvain, Brussels, Belgium; Department of Neurology (S.M.), Hospital de Pediatría J.P. Garrahan, Buenos Aires, Argentina; Dysimmune Neuropathies Unit (G.A.M.), Department of Systems Medicine, Tor Vergata University Hospital, Rome, Italy; Department of Medicine (N.S.), University of Malaya, Kuala Lumpur, Malaysia; Department of Neurology (G.G.), University Hospital of Modena, Italy; Department of Clinical Neurophysiology (Y.P.), Reference Centre for NMD, CHU Nantes, Nantes, France; Department of Neurology (J.B.), Saarland University Medical School, Homburg (previous hospital), and MVZ Pfalzklinikum (J.B.), Kusel, Germany (current hospital); Department of Neurology (K.K., R.P.K.), Albert Schweitzer Hospital, Dordrecht, the Netherlands; Department of Neurology (C.M.), Hospital Británico, Buenos Aires, Argentina; Department of Neurology (M.J.S.T.), Hospital Marques de Valdecilla, Santander; Department of Neurology (L.Q., L.M.-A.), Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, CIBERER and ERN-NMD, Spain; Department of Neurology (Y.W.), Affiliated Hospital of Jining Medical University, Shandong Province, China; Neuromuscular and Neuroimmunology Service (E.N.-O.), IRCCS Humanitas Research Hospital, Milan University, Italy; Nuffield Department of Clinical Neurosciences (S.R.), University of Oxford and Oxford University Hospitals NHS Foundation Trust, United Kingdom; Department of Neurosciences, Ophthalmology, Rehabilitation, Genetics and Maternal Sciences (A.S.), University of Genova, and IRCCS San Martino Hospital (A.S.), Genova, Italy; Department of Neurology (J.P.), Hospital Clínico de Santiago, Travesia Choupana, Santiago de Compostela (A Coruña), Spain; Department of Neurology (F.H.V.), Franciscus Gasthuis & Vlietland (location: Franciscus Gasthuis), Rotterdam, the Netherlands; Department of Neurology (W.W., N.K.), University of Vermont Medical Centre, Burlington; Department of Neurology (H.C.L.), University Hospital of Cologne, Germany; Department of Neurology (V.G., B.S.), Montefiore Medical Center, Bronx, NY; Department of Neurology (G.C.), University Milano-Bicocca, Monza, Italy; Department of Neurology (G.G.-G.), Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Spain; Department of Neurology (F.A.B.), Instituto de Investigaciones Neurológicas Raúl Carrea, FLENI, Buenos Aires, Argentina; Department of Neurology (H.D.K.), University Health Network, University of Toronto, Ontario, Canada; Department of Neurology (E.D.), University Hospital of Larissa, Greece; Department of Neurology (S. Attarian), Reference Centre for NMD, CHU Timone ERN NMD, Marseille, France; Department of Neurology (A.J.v.d.K., F.E.), Amsterdam University Medical Centre, University of Amsterdam, Neuroscience Institute; Department of Neurology (J.P.A.S.), Maasstad Hospital, Rotterdam; Department of Neurology (H.J.G.), Reinier de Graaf Hospital, Reinier de Graafweg, Delft, the Netherlands; Department of Neurology (R.D.M.H.), King's College Hospital, Denmark Hill, London; Department of Neurology (J.K.L.H.), The Walton Centre, Liverpool, United Kingdom; Department of Neurology (K.A.S.), University of Texas Health Science Centre at Houston; Department of Biology (N.K., S. Karafiath), Utah Valley University, Orem; Department of Neurology (M.V.), Lahey Hospital and Medical Center, Tufts University School of Medicine, Burlington, MA; Department of Neurology, Mental Health and Sensory Organs (NESMOS) (G.A.), Faculty of Medicine and Psychology, University of Rome "Sapienza," Sant' Andrea Hospital, Italy; Department of Clinical Neurosciences (T.E.F.), University of Calgary, Alberta, Canada; Department of Neurology (C.F.), Maastricht University Medical Centre, the Netherlands; Department of Neurology (M.B.), Leeds Teaching Hospitals; Department of Neurology (R.C.R.), Addenbrooke's Hospital, Cambridge, United Kingdom; Department of Neurology (N.J.S.), University at Buffalo Jacobs School of Medicine and Biomedical Sciences, NY; Department of Neurology (R.F.), Scientific Institute San Raffaele, Milano, Italy; Department of Neurology (G.W.v.D.), Canisius Wilhelmina Hospital, Nijmegen; Department of Neurology (M.P.J.G.), Jeroen Bosch Hospital, 's-Hertogenbosch; Department of Neurology (J.V.), Leiden University Medical Centre; and Department of Immunology (B.C.J.), Erasmus MC, University Medical Centre Rotterdam, the Netherlands.

出版信息

Neurology. 2023 Jun 6;100(23):e2386-e2397. doi: 10.1212/WNL.0000000000207282. Epub 2023 Apr 19.

Abstract

BACKGROUND AND OBJECTIVES

To investigate CSF findings in relation to clinical and electrodiagnostic subtypes, severity, and outcome of Guillain-Barré syndrome (GBS) based on 1,500 patients in the International GBS Outcome Study.

METHODS

Albuminocytologic dissociation (ACD) was defined as an increased protein level (>0.45 g/L) in the absence of elevated white cell count (<50 cells/μL). We excluded 124 (8%) patients because of other diagnoses, protocol violation, or insufficient data. The CSF was examined in 1,231 patients (89%).

RESULTS

In 846 (70%) patients, CSF examination showed ACD, which increased with time from weakness onset: ≤4 days 57%, >4 days 84%. High CSF protein levels were associated with a demyelinating subtype, proximal or global muscle weakness, and a reduced likelihood of being able to run at week 2 (odds ratio [OR] 0.42, 95% CI 0.25-0.70; = 0.001) and week 4 (OR 0.44, 95% CI 0.27-0.72; = 0.001). Patients with the Miller Fisher syndrome, distal predominant weakness, and normal or equivocal nerve conduction studies were more likely to have lower CSF protein levels. CSF cell count was <5 cells/μL in 1,005 patients (83%), 5-49 cells/μL in 200 patients (16%), and ≥50 cells/μL in 13 patients (1%).

DISCUSSION

ACD is a common finding in GBS, but normal protein levels do not exclude this diagnosis. High CSF protein level is associated with an early severe disease course and a demyelinating subtype. Elevated CSF cell count, rarely ≥50 cells/μL, is compatible with GBS after a thorough exclusion of alternative diagnoses.

CLASSIFICATION OF EVIDENCE

This study provides Class IV evidence that CSF ACD (defined by the Brighton Collaboration) is common in patients with GBS.

摘要

背景和目的

通过对国际吉兰-巴雷综合征(GBS)预后研究中的 1500 名患者进行研究,探讨脑脊液(CSF)检查结果与临床和电诊断亚型、严重程度和结局的关系。

方法

白蛋白细胞分离(ACD)定义为蛋白水平升高(>0.45g/L)而白细胞计数正常(<50 个细胞/μL)。由于其他诊断、违反方案或数据不足,我们排除了 124 名(8%)患者。共对 1231 名(89%)患者进行了 CSF 检查。

结果

846 名(70%)患者的 CSF 检查结果显示 ACD,随着发病时间的推移,这种情况的发生率逐渐增加:发病 4 天内为 57%,4 天后为 84%。高 CSF 蛋白水平与脱髓鞘亚型、近端或全身肌无力以及在第 2 周(比值比 [OR]0.42,95%CI0.25-0.70;P=0.001)和第 4 周(OR0.44,95%CI0.27-0.72;P=0.001)时无法跑步的可能性降低有关。米勒-费舍尔综合征、远端为主的肌无力以及正常或不确定的神经传导研究更可能导致较低的 CSF 蛋白水平。1005 名患者(83%)的 CSF 细胞计数<5 个/μL,200 名患者(16%)为 5-49 个/μL,13 名患者(1%)≥50 个/μL。

讨论

ACD 是 GBS 的常见表现,但正常蛋白水平并不能排除该诊断。高 CSF 蛋白水平与疾病早期严重程度和脱髓鞘亚型有关。升高的 CSF 细胞计数(罕见≥50 个/μL),在排除其他诊断后,与 GBS 一致。

证据分类

本研究提供了 IV 级证据,表明 GBS 患者的 CSF ACD(由布莱顿协作组织定义)很常见。

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