Hospital Israelita Albert Einstein, São Paulo, Brazil.
Hospital Municipal Dr Moysés Deutsch, São Paulo, Brazil.
Pract Neurol. 2024 Jul 16;24(4):306-309. doi: 10.1136/pn-2024-004089.
A 30-year-old woman had 5 days of visual hallucinations, nystagmus, memory impairment and mutism. On examination, she was disorientated with reduced attention span, gaze-evoked nystagmus, paratonia and abnormal frontal reflexes. Cerebrospinal fluid (CSF) showed 80 cells, protein 0.41 g/L and glucose 3.2 mmol/L (plasma glucose 5.0 mmol/L). MR scan of the brain showed involvement of limbic and extra-limbic regions and brainstem. Commercial cell-based assays were negative, but tissue-based assays showed neuropil staining, and cell-based assays for anti-metabotropic glutamate receptor 5 (mGluR5) antibodies were positive in serum and CSF. Six months later, she was diagnosed with Hodgkin's lymphoma. This case emphasises the broader clinical spectrum of anti-mGluR5 encephalitis, challenging its initial characterisation as Ophelia syndrome. It underscores the significance of interpreting commercial cell-based assays and advocates for tissue-based assay testing followed by cell-based assay testing in serum and CSF for diagnosing rare autoimmune encephalitis.
一位 30 岁女性出现 5 天的幻视、眼球震颤、记忆力减退和缄默症。体格检查发现患者定向力障碍、注意力不集中、眼球震颤、张力障碍和额反射异常。脑脊液检查显示 80 个细胞，蛋白 0.41g/L，葡萄糖 3.2mmol/L（血浆葡萄糖 5.0mmol/L）。脑磁共振成像显示边缘和边缘外区域以及脑干受累。商业细胞检测呈阴性，但组织检测显示神经毡染色，血清和脑脊液中抗代谢型谷氨酸受体 5（mGluR5）抗体的细胞检测呈阳性。6 个月后，她被诊断为霍奇金淋巴瘤。本病例强调了抗 mGluR5 脑炎更广泛的临床表现，挑战了其最初作为 Ophelia 综合征的特征。它强调了解释商业细胞检测的重要性，并提倡进行组织检测，然后在血清和脑脊液中进行细胞检测，以诊断罕见的自身免疫性脑炎。
