Hospital Queen Elizabeth, Kota Kinabalu, Sabah, Malaysia.
Universiti Malaysia Sarawak (UNIMAS), Kota Samarahan, Sarawak, Malaysia.
J Med Case Rep. 2023 Mar 6;17(1):99. doi: 10.1186/s13256-022-03699-8.
Multiple sclerosis is a diffuse chronic demyelinating disease of the central nervous system. It is relatively uncommon in the Asian population and even more so in males. Despite the usual involvement of the brainstem, eight-and-a-half syndrome remains a rare first presentation in multiple sclerosis. Only a few cases have been reported previously, but none involving the Asian population. Eight-and-a-half syndrome, a neuro-ophthalmological condition, is characterized by one-and-a-half syndrome with ipsilateral lower facial nerve palsy, which localizes lesions to the pontine tegmentum. This case report demonstrates the first case of eight-and-a-half syndrome as the first presentation of multiple sclerosis in an Asian male.
A healthy 23-year-old Asian man presented with sudden onset of diplopia followed by left-sided facial asymmetry for 3 days. Assessment of extraocular movement revealed left conjugate horizontal gaze palsy. On right gaze, there was limited left eye adduction and horizontal nystagmus of the right eye. These findings were consistent with a left-sided one-and-a-half syndrome. Prism cover test revealed left esotropia of 30 prism diopters. Cranial nerve examination showed left lower motor neuron facial nerve palsy, while other neurological examination was normal. Magnetic resonance imaging brain showed multifocal T2 fluid attenuated inversion recovery hyperintense lesions, involving bilateral periventricular, juxtacortical, and infratentorial regions. A focal gadolinium contrast-enhanced lesion with open ring sign on T1 sequence was seen at the left frontal juxtacortical region. Multiple sclerosis was diagnosed on the basis of the clinical and radiological evidence, which fulfilled the 2017 McDonald criteria. Positive oligoclonal bands in cerebrospinal fluid analysis further confirmed our diagnosis. He had a complete resolution of symptoms 1 month after a course of pulsed corticosteroid therapy, and was subsequently placed on maintenance therapy with interferon beta-1a.
This case illustrates eight-and-a-half syndrome as the first presentation of a diffuse central nervous system pathology. A wide range of differential diagnoses needs to be considered in such a presentation as based on the patient's demographics and risk factors.
多发性硬化症是一种弥漫性中枢神经系统慢性脱髓鞘疾病。它在亚洲人群中相对少见，在男性中更为罕见。尽管脑干通常会受到影响，但 8 又 1/2 综合征仍然是多发性硬化症的罕见首发表现。以前仅报道过少数病例，但都不涉及亚洲人群。8 又 1/2 综合征是一种神经眼科疾病，其特征为同侧下颅神经麻痹的 1 又 1/2 综合征，病变定位于脑桥被盖。本病例报告首次展示了亚洲男性多发性硬化症首发表现为 8 又 1/2 综合征。
一位健康的 23 岁亚裔男性，突发复视，伴左侧面部不对称 3 天。眼外肌评估显示左侧共轭水平凝视麻痹。向右凝视时，左眼内收受限，右眼出现水平眼球震颤。这些发现与左侧 1 又 1/2 综合征一致。棱镜遮盖试验显示左侧 30 棱镜度内斜视。颅神经检查显示左侧下运动神经元性面神经病，其他神经系统检查正常。脑磁共振成像显示多发性 T2 液衰减反转恢复高信号病灶，累及双侧脑室周围、皮质下和颅后窝区域。左额皮质下区域可见局灶性钆增强病变，T1 序列呈开环征。根据临床和影像学证据，诊断为多发性硬化症，符合 2017 年 McDonald 标准。脑脊液寡克隆带阳性进一步证实了我们的诊断。他在接受脉冲皮质类固醇治疗 1 个月后症状完全缓解，随后接受干扰素 β-1a 维持治疗。
本病例说明了 8 又 1/2 综合征是弥漫性中枢神经系统病变的首发表现。在这种表现中，需要根据患者的人口统计学和危险因素考虑广泛的鉴别诊断。
