Glial Fibrillary Acidic Protein Autoimmunity A French Cohort Study

被引：88

作者：

Gravier-Dumonceau, Alice ^{[1
,2
]}

Ameli, Roxana ^{[3
]}

Rogemond, Veronique ^{[4
,6
,7
,8
]}

Ruiz, Anne ^{[9
,10
]}

Joubert, Bastien ^{[4
,6
,7
,8
]}

Muniz-Castrillo, Sergio ^{[4
,6
,7
,8
]}

Vogrig, Alberto ^{[4
,6
,7
,8
]}

Picard, Geraldine ^{[4
,6
,7
,8
]}

Ambati, Aditya ^{[11
]}

Benaiteau, Marie ^{[12
]}

Rulquin, Florence ^{[13
]}

Ciron, Jonathan ^{[13
]}

Deiva, Kumaran ^{[14
]}

de Broucker, Thomas ^{[15
]}

Kremer, Laurent ^{[16
]}

Kerschen, Philippe ^{[17
]}

Sellal, Francois ^{[18
,20
]}

Bouldoires, Bastien ^{[19
]}

Genet, Roxana ^{[21
]}

Biberon, Julien ^{[22
]}

Bigot, Adrien ^{[23
]}

Duval, Fanny ^{[24
]}

Issa, Nahema ^{[25
]}

Rusu, Elena-Camelia ^{[26
]}

Goudot, Mathilde ^{[27
]}

Dutray, Anais ^{[28
]}

Devoize, Jean Louis ^{[29
]}

Hopes, Lucie ^{[30
]}

Kaminsky, Anne-Laure ^{[31
]}

Philbert, Marion ^{[32
]}

Chanson, Eve ^{[33
]}

Leblanc, Amelie ^{[34
]}

Morvan, Erwan ^{[35
]}

Andriuta, Daniela ^{[36
,37
,38
]}

Diraison, Philippe ^{[39
]}

Mirebeau, Gabriel ^{[40
]}

Derollez, Celine ^{[41
]}

Bourg, Veronique ^{[42
]}

Bodard, Quentin ^{[43
]}

Fort, Clementine ^{[5
]}

Grigorashvili-Coin, Irina ^{[44
]}

Rieul, Guillaume ^{[45
]}

Molinier-Tiganas, Daniela ^{[46
]}

Bonnan, Mickael ^{[47
]}

Tchoumi, Thierry ^{[48
]}

Honnorat, Jerome ^{[4
,6
,7
,8
]}

Marignier, Romain ^{[1
,2
,9
,10
]}

机构：

[1] Hop Neurol & Neurochirurg P Wertheimer, Serv Neurol, Sclerose Plaques Pathol Myeline & Neuroinflammat, Bron, France

[2] Hop Neurol & Neurochirurg P Wertheimer, Ctr Reference Malad Inflammat Rares Cerveau & Moe, Bron, France

[3] Hop Neurol & Neurochirurg P Wertheimer, Serv Imagerie Med, Bron, France

[4] Hop Neurol & Neurochirurg P Wertheimer, Ctr Reference Syndromes Neurol Paraneoplas & Ence, Bron, France

[5] Hosp Civils Lyon, Hop Femme Mere Enfant, Serv Neurol Pediat, Lyon, France

[6] INSERM 1217, Inst NeuroMyoGene, Paris, France

[7] CNRS, UMR5310, Paris, France

[8] Univ Claude Bernard Lyon 1, Fac Med Lyon Est, Lyon, France

[9] INSERM 1028, Ctr Rech Neurosci Lyon, Paris, France

[10] CNRS, UMR5292, Paris, France

[11] Stanford Univ, Ctr Sleep Sci & Med, Palo Alto, CA 94304 USA

[12] Hop Toulouse, Hop Pierre Paul Riquet, Serv Neurol Cognit Epilepsie Sommeil & Mouvements, Toulouse, France

[13] Hop Toulouse, Hop Pierre Paul Riquet, Serv Neurol Inflammatoire & Neurooncol, Toulouse, France

[14] Hop Bicetre, AP HP, Serv Neuropediat, Le Kremlin Bicetre, France

[15] Hop Delafontaine, Ctr Hosp St Denis, Serv Neurol, St Denis, France

[16] Hop Univ Strasbourg, Hop Hautepierre, Serv Neurol, Strasbourg, France

[17] Ctr Hosp Luxembourg, Serv Neurol, Luxembourg, Luxembourg

[18] Hop Civils Colmar, Serv Neurol, Colmar, Switzerland

[19] Hop Civils Colmar, Serv Med Interne, Colmar, Switzerland

[20] Univ Strasbourg, Fac Med, Unite INSERM, U 1118, Strasbourg, France

[21] Hop Instruct Armees Legouest, Serv Med Interne, Metz, France

[22] Ctr Hosp Reg Univ Tours, Serv Neurol, Tours, France

[23] Ctr Hosp Reg Univ Tours, Serv Med Interne & Immunol Clin, Tours, France

[24] Hop Bordeaux, Grp Hosp Pellegrin, Serv Neurol & Malad Neuromusculaires, Bordeaux, France

[25] Hop St Andre, Serv Med Intens & Reanimat, Bordeaux, France

[26] Hop St Musse, Ctr Hosp Intercommunal Toulon, Serv Neurol, Toulon, France

[27] Hop Emile Muller, Serv Neurol, Mulhouse, France

[28] Ctr Hosp Perpignan, Serv Neurol, Perpignan, France

[29] Ctr Hosp Cayenne, Pole Cardiovasc & Metabol, Cayenne, French Guiana

[30] CHRU Nancy, Hop Cent, Serv Neurol, Nancy, France

[31] CHU St Etienne, Serv Neurol, St Priest En Jarez, France

[32] CHU Martinique, Serv Neuropediat, Site Mere Enfant, Fort De France, France

[33] CHU Clermont Ferrand, CHU Gabriel Montpied, Serv Neurol, Clermont Ferrand, France

[34] Hop Instruct Armees Clermont Tonnerre, Serv Neurol, Brest, France

[35] Hop Fdn Adolphe Rothschild, Serv Neurol, Paris, France

[36] CHU Amiens, Serv Neurol, Amiens, France

[37] CHU Amiens, Lab Neurosci Fonct & Pathol, Amiens, France

[38] Univ Picardie Jules Verne, Amiens, France

[39] Ctr Hosp Cornouaille, Hop Laennec, Serv Neurol, Quimper, France

[40] CHU La Reunion, Serv Neurol, St Pierre, France

[41] CHU Lille, Hop Roger Salengro, Serv Neurol, Lille, France

[42] CHU Nice, Hop Pasteur 2, Serv Neurol, Nice, France

[43] Ctr Hosp Angouleme, Serv Med Interne & Malad Infect, Angouleme, France

[44] Ctr Hosp St Brieuc, Serv Neurol, St Brieuc, France

[45] Ctr Hosp St Brieuc, Serv Reanimat, St Brieuc, France

[46] Ctr Hosp Le Mans, Serv Med Polyvalente & Med Interne, Le Mans, France

[47] Ctr Hosp Pau, Serv Neurol, Pau, France

[48] Ctr Hosp Saintonge, Serv Neurol, UNV, Saintes, France

来源：

NEUROLOGY | 2022年 / 98卷 / 06期

关键词：

SCLEROSIS-LIKE ILLNESS; MILD ENCEPHALITIS/ENCEPHALOPATHY; LIMBIC ENCEPHALITIS; CASE SERIES; ASTROCYTOPATHY; COMPLICATIONS; INFECTION;

D O I：

10.1212/WNL.0000000000013087

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Background and Objectives To report the clinical, biological, and imaging features and clinical course of a French cohort of patients with glial fibrillary acidic protein (GFAP) autoantibodies. Methods We retrospectively included all patients who tested positive for GFAP antibodies in the CSF by immunohistochemistry and confirmed by cell-based assay using cells expressing human GFAP alpha since 2017 from 2 French referral centers. Results We identified 46 patients with GFAP antibodies. Median age at onset was 43 years, and 65% were men. Infectious prodromal symptoms were found in 82%. Other autoimmune diseases were found in 22% of patients, and coexisting neural autoantibodies in 11%. Tumors were present in 24%, and T-cell dysfunction in 23%. The most frequent presentation was subacute meningoencephalitis (85%), with cerebellar dysfunction in 57% of cases. Other clinical presentations included myelitis (30%) and visual (35%) and peripheral nervous system involvement (24%). MRI showed perivascular radial enhancement in 32%, periventricular T2 hyperintensity in 41%, brainstem involvement in 31%, leptomeningeal enhancement in 26%, and reversible splenial lesions in 4 cases. A total of 33 of 40 patients had a monophasic course, associated with a good outcome at last follow-up (Rankin Score <= 2: 89%), despite a severe clinical presentation. Adult and pediatric features are similar. Thirty-two patients were treated with immunotherapy. A total of 11/22 patients showed negative conversion of GFAP antibodies. Discussion GFAP autoimmunity is mainly associated with acute/subacute meningoencephalomyelitis with prodromal symptoms, for which tumors and T-cell dysfunction are frequent triggers. The majority of patients followed a monophasic course with a good outcome.

引用

页码：E653 / E668

页数：16

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