Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India.
Department of Neuropathology, National Institute of Mental Health and Neurosciences (NIMHANS), Bengaluru, Karnataka, India.
Neurol India. 2024 Mar 1;72(2):375-378. doi: 10.4103/ni.ni_692_22. Epub 2024 Apr 30.
Morvan's syndrome is a rare, complex autoimmune syndrome comprising peripheral nerve hyperexcitability, dysautonomia, insomnia, and encephalopathy. In this case report, we highlight the clinical and pathological findings of an elderly gentleman who presented to us with clinical features of Morvan's syndrome associated with anti-contactin-associated protein 2 (CASPR-2) antibodies. Histopathology [Figure 3] revealed cortical atrophy with gliosis and mild microglial proliferation. Microglial activation and gliosis were observed in the hippocampus, hypothalamus, and thalamus. Brainstem showed multifocal inflammation. Mild inflammation was observed in the leptomeninges. Morvan's syndrome is an autoimmune disease with antibodies targeted against CASPR within the voltage-gated potassium channel (VGKC) complex. Early diagnosis and treatment play a key role in the management of patients. Most patients show a good response when treated with plasmapheresis and steroids. This patient presented to us late into the illness and succumbed.
莫旺综合征是一种罕见的、复杂的自身免疫性综合征,包括周围神经兴奋性过高、自主神经功能紊乱、失眠和脑病。在本病例报告中,我们重点介绍了一位老年绅士的临床和病理发现,他表现出莫旺综合征的临床特征,伴有抗接触蛋白相关蛋白 2(CASPR-2)抗体。组织病理学[图 3]显示皮质萎缩伴神经胶质增生和轻度小胶质细胞增生。海马体、下丘脑和丘脑观察到小胶质细胞激活和神经胶质增生。脑干呈现多灶性炎症。软脑膜有轻度炎症。莫旺综合征是一种自身免疫性疾病,抗体针对电压门控钾通道(VGKC)复合物中的 CASPR。早期诊断和治疗对患者的管理起着关键作用。大多数患者在接受血浆置换和类固醇治疗后反应良好。这位患者在疾病晚期就诊,最终去世。