title: Clinical features and prognostic factors of spinal cord sarcoidosis: a multicenter observational study of 20 BIOPSY-PROVEN patients.
abstract: Sarcoidosis of the spinal cord is a rare disease. The aims of this study are to describe the features of spinal cord sarcoidosis (SCS) and identify prognostic markers. We analyzed 20 patients over a 20-year period in 8 French hospitals. There were 12 men (60 %), mostly Caucasian (75 %). The median ages at diagnosis of sarcoidosis and myelitis were 34.5 and 37 years, respectively. SCS revealed sarcoidosis in 12 patients (60 %). Eleven patients presented with motor deficit (55 %) and 9 had sphincter dysfunction (45 %). The median initial Edmus Grading Scale (EGS) score was 2.5. The cerebrospinal fluid (CSF) showed elevated protein level (median: 1.00 g/L, interquartile range (IQR) 0.72-1.97), low glucose level (median 2.84 mmol/L, IQR 1.42-3.45), and elevated white cell count (median 22/mm(3), IQR 6-45). The cervical and thoracic cords were most often affected (90 %). All patients received steroids and an immunosuppressive drug was added in 10 cases (50 %). After a mean follow-up of 52.1 months (range 8-43), 18 patients had partial response (90 %), 7 displayed functional impairment (35 %), and the median final EGS score was 1. Six patients experienced relapse (30 %). There was an association between the initial and the final EGS scores (p = 0.006). High CSF protein level showed a trend toward an association with relapse (p = 0.076). The spinal cord lesion was often the presenting feature of sarcoidosis. Most patients experienced clinical improvement with corticosteroids and/or immunosuppressive treatment. The long-term functional prognosis was correlated with the initial severity.
authors.affiliation: Département de Médicine Interne, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, 69004, Lyon, France. cecile-audrey.durel@chu-lyon.fr.|Université de Lyon, Université Lyon 1, 69100, Villeurbanne, France. cecile-audrey.durel@chu-lyon.fr.
authors.forename: Cécile-Audrey
authors.initials: CA
authors.lastname: Durel
authors.affiliation: Département de Neurologie, Hôpital Pierre Wertheimer, Hospices Civils de Lyon, 69500, Bron, France.
authors.forename: Romain
authors.initials: R
authors.lastname: Marignier
authors.affiliation: Université de Lyon, Université Lyon 1, 69100, Villeurbanne, France.|Service de Biostatistique, Hospices Civils de Lyon, 69003, Lyon, France.|CNRS UMR 555, Laboratoire de Biométrie et Biologie Evolutive, Equipe Biostatistique Santé, 69100, Villeurbanne, France.
authors.forename: Delphine
authors.initials: D
authors.lastname: Maucort-Boulch
authors.affiliation: Université de Lyon, Université Lyon 1, 69100, Villeurbanne, France.|Service de Biostatistique, Hospices Civils de Lyon, 69003, Lyon, France.|CNRS UMR 555, Laboratoire de Biométrie et Biologie Evolutive, Equipe Biostatistique Santé, 69100, Villeurbanne, France.
authors.forename: Jean
authors.initials: J
authors.lastname: Iwaz
authors.affiliation: Département de Médicine Interne, CH Saint Luc Saint Joseph, 69007, Lyon, France.
authors.forename: Emilie
authors.initials: E
authors.lastname: Berthoux
authors.affiliation: Département de Médecine Interne, CHU de Clermont-Ferrand, CHU Estaing, 63003, Clermont-Ferrand, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Ruivard
authors.affiliation: Service de Médecine Interne, CHU Clermont-Ferrand, Hôpital Gabriel Montpied, 63003, Clermont-Ferrand, France.
authors.forename: Marc
authors.initials: M
authors.lastname: André
authors.affiliation: Département de Médecine Interne, CHU de Clermont-Ferrand, CHU Estaing, 63003, Clermont-Ferrand, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Le Guenno
authors.affiliation: Département de Médecine Interne, Hôpital Edouard Herriot, Hospices Civils de Lyon, 69008, Lyon, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Pérard
authors.affiliation: Département de Médecine Interne, Centre hospitalier Fleyriat, 01012, Bourg-en-Bresse, France.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Dufour
authors.affiliation: Département de Médecine Interne et Maladies Systémiques, CHU Dijon, 21079, Dijon, France.
authors.forename: Alin
authors.initials: A
authors.lastname: Turcu
authors.affiliation: Département de Neurologie, CHU de Saint-Etienne, Hôpital Nord, 42055, Saint Etienne Cedex 022, France.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Antoine
authors.affiliation: Département de Neurologie, CHU de Saint-Etienne, Hôpital Nord, 42055, Saint Etienne Cedex 022, France.
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Camdessanche
authors.affiliation: Département de Médecine Interne, CH Montluçon, 03100, Montluçon, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Delboy
authors.affiliation: Département de Médicine Interne, Hôpital de la Croix-Rousse, Hospices Civils de Lyon, 69004, Lyon, France.|Université de Lyon, Université Lyon 1, 69100, Villeurbanne, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Sève
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D005602:France / Q000453:epidemiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology*; D012720:Severity of Illness Index; D013116:Spinal Cord / Q000000981:diagnostic imaging / Q000187:drug effects; D013118:Spinal Cord Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology / Q000503:physiopathology*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents
keywords: Morbidity; Myelopathy; Neurosarcoidosis; Sarcoidosis; Sequelae; Spinal cord sarcoidosis