Chan Koon Ho, Tse C T, Chung C P, Lee Raymand L C, Kwan J S C, Ho P W L, Ho J W M
Queen Mary Hospital, LKS Faculty of Medicine, University Department of Medicine, The University of Hong Kong, 4/F Professorial Block, 102 Pokfulam Rd, Hong Kong.
Arch Neurol. 2011 Nov;68(11):1432-9. doi: 10.1001/archneurol.2011.249.
Neuromyelitis optica spectrum disorders (NMOSDs) are severe inflammatory demyelinating disorders of the central nervous system. Brain involvement is increasingly recognized.
To study brain involvement in NMOSDs among Hong Kong Chinese patients.
Retrospective study of patients with NMOSDs.
Tertiary medical center in Hong Kong. Patients Thirty-four Hong Kong Chinese patients with NMOSDs of 2 years or longer were recruited.
Brain and spinal cord magnetic resonance imaging was performed during NMOSD attacks and was repeated yearly for the first 3 years.
We evaluated clinical features of NMOSDs associated with brain involvement and brain lesions on magnetic resonance imaging.
Among 34 patients with NMOSDs of 2 years or longer, 20 (59%) had brain involvement. The mean age at onset among these 20 patients was 45.6 years (age range, 19-67 years); 18 were women. Eleven patients (32% of all the patients with NMOSDs) had clinical manifestation of brain involvement, 19 patients (56%) had brain abnormalities on magnetic resonance imaging consistent with inflammatory demyelination, and 2 patients (6%) fulfilled criteria for multiple sclerosis. Clinical manifestation of brain involvement included the following: trigeminal neuralgia; vomiting, vertigo, ataxia, dysphagia, and tetraparesis from lesions around the third and fourth ventricles and aqueduct; homonymous hemianopia, aphasia, hemiparesis, and cognitive impairment from extensive hemispheric white matter lesions; and ataxia, diplopia, hiccups, facial sensory loss, internuclear ophthalmoplegia, hemisensory loss, and hemiparesis from other lesions in the midbrain, pons, cerebellar peduncles, and medulla. Eight patients (24%) developed brainstem encephalitis clinically, and brainstem encephalitis was the initial clinical manifestation in 6 patients (18%). Brain abnormalities on magnetic resonance imaging were detected in brainstem in 15 patients (44%), hemispheric periventricular white matter in 7 patients (21%), deep white matter in 7 patients (21%), corpus callosum in 4 patients (12%), subcortical white matter in 3 patients (9%), thalamus in 2 patients (6%), hypothalamus in 1 patient (3%), basal ganglia in 1 patient (3%), internal capsule in 1 patient (3%), periaqueductal gray matter in 1 patient (3%), and around the third and fourth ventricles in 1 patient (3%); large confluent lesions were detected in 2 patients (6%).
Brain involvement manifesting clinically as brainstem encephalitis is common among Hong Kong Chinese patients with NMOSDs.
视神经脊髓炎谱系疾病（NMOSDs）是中枢神经系统严重的炎性脱髓鞘疾病。脑受累越来越受到认可。
研究香港华人NMOSDs患者的脑受累情况。
对NMOSDs患者进行回顾性研究。
香港的三级医疗中心。患者招募了34例病程2年及以上的香港华人NMOSDs患者。
在NMOSD发作期间进行脑和脊髓磁共振成像，并在最初3年内每年重复检查。
我们评估了与脑受累相关的NMOSDs临床特征以及磁共振成像上的脑病变。
在34例病程2年及以上的NMOSDs患者中，20例（59%）有脑受累。这20例患者的平均发病年龄为45.6岁（年龄范围19 - 67岁）；18例为女性。11例患者（占所有NMOSDs患者的32%）有脑受累的临床表现，19例患者（56%）磁共振成像显示脑异常符合炎性脱髓鞘，2例患者（6%）符合多发性硬化标准。脑受累的临床表现包括：三叉神经痛；第三和第四脑室及导水管周围病变导致的呕吐、眩晕、共济失调、吞咽困难和四肢轻瘫；广泛半球白质病变导致的同向性偏盲、失语、偏瘫和认知障碍；中脑、脑桥、小脑脚和延髓其他病变导致的共济失调、复视、呃逆、面部感觉丧失、核间性眼肌麻痹、半身感觉丧失和偏瘫。8例患者（24%）临床上发生脑干脑炎，6例患者（18%）脑干脑炎为首发临床表现。磁共振成像显示脑异常的部位有：15例患者（44%）在脑干，7例患者（21%）在半球脑室周围白质，7例患者（21%）在深部白质，4例患者（12%）在胼胝体，3例患者（9%）在皮质下白质，2例患者（6%）在丘脑，1例患者（3%）在下丘脑，1例患者（3%）在基底节，1例患者（3%）在内囊，1例患者（3%）在导水管周围灰质，1例患者（3%）在第三和第四脑室周围；2例患者（6%）检测到大片融合性病变。
在香港华人NMOSDs患者中，临床上以脑干脑炎表现的脑受累很常见。
