Tanaka Tomoko, Togo Masaya, Okayama Kiminobu, Chihara Norio, Ueda Takehiro, Sekiguchi Kenji, Matsumoto Riki
Division of Neurology, Kobe University Graduate School of Medicine.
Rinsho Shinkeigaku. 2023 Jul 22;63(7):441-449. doi: 10.5692/clinicalneurol.cn-001724. Epub 2023 Jun 30.
We report two male patients who had a sensory seizure, which evolved into a focal impaired awareness tonic seizure, and after that, focal to bilateral tonic-clonic seizure. The first case, a 20-year-old man had been treated with steroids for anti-myelin oligodendrocyte glycoprotein (MOG) antibody-positive optic neuritis. His seizure started with abnormal sensation in the little finger of the left hand, which spread to the left upper and then to the left lower limb. The seizure then evolved into tonic seizures of the upper and lower limbs and he finally lost awareness. The second case, a 19-year-old man experienced floating dizziness while walking, followed by numbness and a pain-like electrical shock in the right upper limb. The right arm somatosensory seizure evolved into a right upper and lower limb tonic seizure, which spread to the bilateral limbs, and finally he lost awareness. Symptoms of both patients improved after the treatment with steroids. Both patients shared a similar high-intensity FLAIR lesion in the posterior midcingulate cortex. Both patients were diagnosed with MOG antibody-positive cerebral cortical encephalitis because of a positive titer of anti-MOG antibody in the serum. Several reports showed involvement of the cingulate gyrus in MOG antibody-positive cerebral cortical encephalitis, but only a few reported seizure semiology in detail. The semiology reported here is consistent with that of cingulate epilepsy or the findings of electrical stimulation of the cingulate cortex, namely, somatosensory (electric shock or heat sensation), motor (tonic posture), and vestibular symptoms (dizziness). Cingulate seizures should be suspected when patients show somatosensory seizures or focal tonic seizures. MOG antibody-positive cerebral cortical encephalitis should be considered as one of the differential diagnoses when the young patient shows the unique symptoms of an acute symptomatic cingulate seizure.
我们报告了两名男性患者,他们最初出现感觉性发作,随后演变为局灶性意识障碍性强直发作,之后又发展为从局灶性到双侧强直阵挛发作。第一例患者为一名20岁男性,曾因抗髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性的视神经炎接受类固醇治疗。他的发作始于左手小指的异常感觉,这种感觉蔓延至左上肢,然后再到左下肢。随后发作演变为上下肢的强直发作,最终他失去了意识。第二例患者为一名19岁男性,行走时出现漂浮性头晕,随后右上肢出现麻木和类似电击样疼痛。右上肢的体感发作演变为右上肢和下肢的强直发作,随后蔓延至双侧肢体,最终他也失去了意识。两名患者在接受类固醇治疗后症状均有所改善。两名患者在扣带回中部皮质均有类似的高强度液体衰减反转恢复(FLAIR)病变。由于血清中抗MOG抗体滴度呈阳性,两名患者均被诊断为MOG抗体阳性的大脑皮质脑炎。有几份报告显示扣带回参与了MOG抗体阳性的大脑皮质脑炎,但只有少数报告详细描述了发作症状学。这里报告的症状学与扣带回癫痫或扣带回皮质电刺激的结果一致,即体感(电击或热感)、运动(强直姿势)和前庭症状(头晕)。当患者出现体感发作或局灶性强直发作时,应怀疑扣带回发作。当年轻患者出现急性症状性扣带回发作的独特症状时,应将MOG抗体阳性的大脑皮质脑炎视为鉴别诊断之一。