Neurology, IRCCS Institute of Neurological Sciences of Bologna, Bologna, Italy.
Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy.
Neurol Sci. 2022 Sep;43(9):5633-5636. doi: 10.1007/s10072-022-06186-6. Epub 2022 Jun 16.
Autoimmune encephalitis (AE) associated to antibodies against GABA A R is a rare form of encephalitis. On the other hand, thymoma has been linked to antibodies against both muscular and neuronal epitopes, even if concurrent positivity for more than one antibody is exceptional, and their contribution to the clinical course and treatment decision is unclear. We report a case of a 73-year-old male with AE associated with thymoma secreting both anti-GABAaR and anti-titin antibodies. Clinical presentation included status epilepticus, behavioural changes and cognitive decline. While the status was stopped with lacosamide, AE treatment included first- and second-line immunomodulation, in addition to thymoma's removal. Nonetheless, the patient experienced a worsening in cognitive and behavioural status.
自身免疫性脑炎(AE)与 GABA A R 抗体相关,是一种罕见的脑炎形式。另一方面,胸腺瘤与针对肌肉和神经元表位的抗体有关,尽管同时存在一种以上抗体的阳性情况很少见,但它们对临床病程和治疗决策的影响尚不清楚。我们报告了一例 73 岁男性,患有 AE 合并胸腺瘤,分泌抗 GABAaR 和抗 titin 抗体。临床表现包括癫痫持续状态、行为改变和认知能力下降。虽然使用拉科酰胺停止了癫痫状态,但 AE 的治疗包括一线和二线免疫调节,以及胸腺瘤的切除。然而,患者的认知和行为状况仍在恶化。