title: [Neurological manifestations of Behçet's disease].
abstract: Neurological manifestations of Behçet's disease (BD) occur in 5.3 to more than 50% of patients. They are divided into two major forms: "parenchymal" lesions, which include mainly meningoencephalitis as opposed to "extra-parenchymal" lesions (i.e. cerebral venous thrombosis and arterial aneurysms). Myelitis or peripheral neuropathy is exceptional. The neuro-Behçet syndrome (NBS) should be considered in the setting of neurological manifestations, particularly headache and pyramidal signs, in a young man diagnosed with BD. However, its recognition may be difficult when neurological manifestations are the presenting features of BD (one third of cases), and requires a thorough knowledge of clinical manifestations and morphological lesions. Thus, parenchymal NB lesions classically exhibit inflammatory characteristics on MRI and are located at the meso-diencephalic junction and in the brainstem, rarely with a supratentorial extension. Meningitis is not systematically associated, and may be absent in about 30% of cases. The pathogenesis of these lesions is incompletely understood, but inflammatory infiltrates include mainly neutrophils and activated T cells (mainly Th17). Differential diagnoses include infectious diseases (herpes, listeria, tuberculosis), and inflammatory diseases (i.e. multiple sclerosis and sarcoidosis). A prompt recognition of NBS should lead to initiate adequate therapies in order to limit the risk of sequelae, relapses or death.
authors.affiliation: Service de médecine interne 2, centre de référence des maladies autoimmunes rares, DHU i2B, immunopathologie, inflammation, biothérapie, université Pierre-et-Marie-Curie, Paris 6, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France.
authors.forename: N
authors.initials: N
authors.lastname: Noel
authors.affiliation: Service de neuroradiologie, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France.
authors.forename: A
authors.initials: A
authors.lastname: Drier
authors.affiliation: Service de médecine interne 2, centre de référence des maladies autoimmunes rares, DHU i2B, immunopathologie, inflammation, biothérapie, université Pierre-et-Marie-Curie, Paris 6, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France.
authors.forename: B
authors.initials: B
authors.lastname: Wechsler
authors.affiliation: Service de médecine interne 2, centre de référence des maladies autoimmunes rares, DHU i2B, immunopathologie, inflammation, biothérapie, université Pierre-et-Marie-Curie, Paris 6, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France; Faculté de médecine, université Pierre-et-Marie-Curie, Paris 6, 75013 Paris, France.
authors.forename: J-C
authors.initials: JC
authors.lastname: Piette
authors.affiliation: Service de neurologie, groupe hospitalier Pitié-Salpétrière, AP-HP, 75013 Paris, France.
authors.forename: R
authors.initials: R
authors.lastname: De Paz
authors.affiliation: Service de neuroradiologie, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France; Faculté de médecine, université Pierre-et-Marie-Curie, Paris 6, 75013 Paris, France.
authors.forename: D
authors.initials: D
authors.lastname: Dormont
authors.affiliation: Service de médecine interne 2, centre de référence des maladies autoimmunes rares, DHU i2B, immunopathologie, inflammation, biothérapie, université Pierre-et-Marie-Curie, Paris 6, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France; Faculté de médecine, université Pierre-et-Marie-Curie, Paris 6, 75013 Paris, France.
authors.forename: P
authors.initials: P
authors.lastname: Cacoub
authors.affiliation: Service de médecine interne 2, centre de référence des maladies autoimmunes rares, DHU i2B, immunopathologie, inflammation, biothérapie, université Pierre-et-Marie-Curie, Paris 6, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France; Faculté de médecine, université Pierre-et-Marie-Curie, Paris 6, 75013 Paris, France. Electronic address: david.saadoun@psl.aphp.fr.
authors.forename: D
authors.initials: D
authors.lastname: Saadoun
mesh_terms: D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D003937:Diagnosis, Differential; D018450:Disease Progression; D006801:Humans; D008297:Male; D008590:Meningoencephalitis / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D009187:Myelitis / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy; D009422:Nervous System Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000628:therapy
keywords: Behçet's disease; Maladie de Behçet; Manifestations neurologiques; Neuro-Behçet; Neurological manifestations