Le Dault E, Lagarde S, Guedj E, Dufournet B, Rey C, Kaphan E, Tanguy G, Bregigeon M, Sagui E, Brosset C
Service de pathologie infectieuse et tropicale, hôpital d'instruction des armées Laveran, 34, boulevard Alphonse-Laveran, 13014 Marseille, France.
Service de neurologie, hôpital d'instruction des armées Laveran, 34, boulevard Alphonse-Laveran, 13014 Marseille, France.
Rev Med Interne. 2016 Feb;37(2):127-30. doi: 10.1016/j.revmed.2015.06.007. Epub 2015 Jul 8.
Anti-leucine rich glioma inactivated 1 encephalitis is a common and a treatable etiology of autoimmune encephalitis. Its diagnosis is a challenge because the initial diagnostic work-up is often normal.
A 48-year-old man experienced cognitive and behavioral troubles, facio-brachial dystonic seizures and a syndrome of inappropriate antidiuretic hormone secretion. First line tests excluded infectious, neoplastic, systemic inflammatory, endrocrine or toxic etiologies. Cerebral (18)Fluoro-desoxy-glucose (FDG) position emission tomography and research of specific antibodies in cerebro-spinal fluid and serum led to diagnose an anti-leucine rich glioma inactivated 1 encephalitis. Intravenous immunoglobulins and corticosteroids were partially effective. Cyclophosphamid permitted a good recovery.
In the presence of acute neuropsychiatric disorders with a negative etiologic research, physician should think about dysimmune encephalitis. Facio-brachial dystonic seizures and syndrome of inappropriate antidiuretic hormone secretion are highly evocative of anti-leucine rich glioma inactivated 1 encephalitis. The diagnosis needs specific diagnostic tests (cerebral (18)FDG position emission tomography and antibodies research in cerebro-spinal fluid and in serum), after the exclusion of alternative diagnoses. Extensive and repeated diagnostic work-up for neoplasia is required. Immunosupressive therapies are effective in most cases.
抗富含亮氨酸胶质瘤失活1型脑炎是自身免疫性脑炎常见且可治疗的病因。其诊断具有挑战性，因为初始诊断检查通常正常。
一名48岁男性出现认知和行为障碍、面臂肌张力障碍性癫痫发作及抗利尿激素分泌不当综合征。一线检查排除了感染、肿瘤、全身性炎症、内分泌或中毒性病因。脑（18）氟脱氧葡萄糖（FDG）正电子发射断层扫描以及脑脊液和血清中特异性抗体的检测确诊为抗富含亮氨酸胶质瘤失活1型脑炎。静脉注射免疫球蛋白和皮质类固醇部分有效。环磷酰胺使病情得到良好恢复。
在病因检查为阴性的急性神经精神障碍患者中，医生应考虑自身免疫性脑炎。面臂肌张力障碍性癫痫发作及抗利尿激素分泌不当综合征高度提示抗富含亮氨酸胶质瘤失活1型脑炎。在排除其他诊断后，诊断需要进行特异性诊断检查（脑（18）FDG正电子发射断层扫描以及脑脊液和血清中的抗体检测）。需要对肿瘤进行广泛且反复的诊断检查。免疫抑制疗法在大多数情况下有效。
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