一名患有饮食失调症的青少年在脱髓鞘疾病的掩盖下合并韦尼克脑病和急性多发性神经病

E. O. Ovchinnikova, A. S. Kotov, M. V. Panteleeva, E. V. Mukhina
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摘要

韦尼克脑病是一种神经精神综合征,主要有三种症状:眼球运动障碍、小脑共济失调和精神障碍。这种病的死亡率和发病率都很高。韦尼克脑病最常见于缺乏维生素 B1 的青少年儿童。硫胺素缺乏症也可能导致多发性神经炎综合征,伴有或不伴有上述症状。这种病的特点是对称性感觉运动障碍、构音障碍、下肢瘫痪甚至瘫痪。本报告主要介绍了一个青少年病例,该病例表现为急性眼球运动麻痹、眼球震颤、腿部无力、步态受损、深部腱反射减弱、认知障碍,以及反复呕吐、长期饥饿和进食行为障碍等病史。磁共振成像扫描显示,在马肯迪中央孔周围区域有对称性病灶,T2强度增高。根据电神经肌电图数据,患者表现为影响双下肢的混合性运动感觉多神经病综合征,主要属于轴索病类型。通过硫胺素治疗,患者的眼球运动得到恢复,步态得到改善,小腿和双脚的肌肉力量得到增强,感觉障碍得到更好的控制。
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Wernicke encephalopathy in combination with acute polyneuropathy under the guise of a demyelinating disease in a teenager with an eating disorder
Wernicke encephalopathy is a neuropsychiatric syndrome characterized by three main symptoms: oculomotor disturbances, cerebellar ataxia, and psychiatric disturbances. The condition is associated with a high mortality and morbidity rate. Wernicke encephalopathy is most commonly seen in adolescent children presenting with a vitamin B1 deficiency. Thiamine deficiency may also cause polyneuritis syndrome, with or without the aforementioned symptoms. The condition is characterized by sensory-motor impairments in a symmetrical pattern, dysarthria, and paresis or even paralysis of the lower limbs. This report focuses on an adolescent case presenting acute oculomotor paresis, nystagmus, leg weakness, impaired gait, decreased deep tendon reflexes, cognitive impairment, and a history of recurrent vomiting, prolonged starvation, and eating behaviour disorders. The magnetic resonance imaging scan reveals symmetrical pathological foci of increased intensity in T2 in the periaqueductal region, at the Magendie’s central aperture. The patient displays a mixed motor-sensory polyneuropathic syndrome affecting both lower limbs, primarily of the axonopathy type, based on electroneuromyography data. Positive outcomes such as restored eyeball movement, enhanced gait, increased muscle strength in the lower legs and feet, and better management of sensory disorders have occurred due to thiamine treatment.
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