Gao Cong, Wu Linzhan, Chen Xiaohui, Long Youming, Zhong Rong, Yang Ning, Chen Yaotang
a 1 Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and The Ministry of Education of China , Institute of Neuroscience and the Second Affiliated Hospital of GuangZhou Medical University , Guangzhou , China.
b 2 Department of Neurology , the Second Affiliated Hospital of GuangZhou Medical University , Guangzhou , China.
Int J Neurosci. 2016 Nov;126(11):1036-43. doi: 10.3109/00207454.2015.1114484. Epub 2016 Feb 5.
Hypothalamic lesions in neuromyelitis optica (NMO) patients might be more specific for NMO than multiple sclerosis (MS). However, this is controversial.
To characterize clinical features of patients with inflammatory demyelinating disorders (IDDs) with visible hypothalamic lesions using magnetic resonance imaging (MRI).
Patients with IDDs (n = 429) were recruited retrospectively.
Of 52 patients with hypothalamic images enrolled, 42 were positive for aquaporin-4 (AQP4) antibodies, including 28 patients with NMO, 6 with recurrent transverse myelitis, 3 with recurrent optic neuritis, and 5 with brainstem and brain syndrome. The remaining 10 patients were anti-AQP4-negative, including 3 with MS, 3 with acute disseminated encephalomyelitis, and 4 with other disorders. In the AQP4-positive group, manifestations, including ataxia, intractable hiccup and nausea, syndrome of inappropriate antidiuretic hormone secretion and encephalopathy were more frequent in those with hypothalamic lesions than those without. Cell counts of cerebrospinal fluid in patients with hypothalamic lesions differed from patients without lesions. Brain MRI abnormalities were more frequent in brainstem and hemisphere of the hypothalamic lesion group.
Hypothalamic lesions were observed frequently in patients with AQP4 antibodies. Clinical manifestations and paraclinical features in AQP4-positive patients with hypothalamic lesions differed from those without lesions.
视神经脊髓炎（NMO）患者的下丘脑病变对NMO而言可能比多发性硬化（MS）更具特异性。然而，这一点存在争议。
利用磁共振成像（MRI）对有可见下丘脑病变的炎性脱髓鞘疾病（IDDs）患者的临床特征进行描述。
回顾性招募IDDs患者（n = 429）。
纳入的52例有下丘脑影像的患者中，42例水通道蛋白4（AQP4）抗体呈阳性，包括28例NMO患者、6例复发性横贯性脊髓炎患者、3例复发性视神经炎患者以及5例脑干和脑综合征患者。其余10例患者抗AQP4阴性，包括3例MS患者、3例急性播散性脑脊髓炎患者以及4例其他疾病患者。在AQP4阳性组中，有下丘脑病变的患者出现共济失调、顽固性呃逆和恶心、抗利尿激素分泌不当综合征及脑病等表现的频率高于无下丘脑病变者。有下丘脑病变患者的脑脊液细胞计数与无病变患者不同。下丘脑病变组脑干和半球的脑部MRI异常更为常见。
AQP4抗体阳性患者中常观察到下丘脑病变。有下丘脑病变的AQP4阳性患者的临床表现和副临床特征与无病变者不同。
