儿童Wernicke’s脑病1例并35例文献复习  

Severe sepsis as an initial presentation in children with Wernicke＇s encephalopathy： report of a case and literature review

作　　者：辛毅[1] 万代红[1] 初清[1] 李爱敏[1] 高兴娟[1] 

机构地区：[1]青岛大学医学院附属烟台毓璜顶医院PICU,烟台264000

出　　处：《中华儿科杂志》2011年第8期612-617,共6页Chinese Journal of Pediatrics

摘　　要：目的探讨儿童Wemicke’s脑病临床特点以及诊断与治疗，提高对该病的认识。方法总结1例以严重脓毒症为首发表现的儿童Wemicke’s脑病的临床表现、诊断及治疗特点，并对国内外近10年报道的35例儿童病例进行文献复习。结果包括本例患儿共36例，男22／36例，年龄2个月～16岁。除本例外均有基础疾病：喂养不当25／35例、长期呕吐5／35例、免疫抑制治疗4／35例、长期完全胃肠外营养（未添加维生素）3／35例以及神经性厌食1／35例；有典型临床“三联征（精神状态改变、眼部体征以及共济失调）”6／36例，其他临床表现：意识障碍24／36例、感染22／36例、神经病理征及肌张力改变18／36例、惊厥17／36例，发育迟缓4／36例、生长停滞2／36例。行脑脊液常规检测31／36例，异常7／31例（蛋白略增高）；检测脑脊液乳酸水平4／36例，均增高；血清乳酸水平检测7／36例、6／7例增高；血清丙酮酸检测4／36例，均增高；硫胺素焦磷酸盐活力（TPPE）检测9／36例，均增高；血清硫胺素水平检测2／36例、1／2例降低；行颅脑CT检查20／36例，16／20例双基底节区低密度、1／20例弥漫性脑皮质萎缩；行颅脑MRI检查13／36例，均表现为双侧对称性乳头体、基底节区异常信号、7／13例还表现中脑被盖、中央导水管及Ⅲ、Ⅳ脑室周围自质区异常信号。诊断方法：在行颅脑MRI的13例中，根据MRI确立诊断12／13例、“三联征”＋MRI确立3／13例、根据尸检1／13例；行TPPE和（或）乳酸测定11例中，确立诊断9／11例。初始治疗：33／36例经胃肠外补充硫胺素、1／36例给药方式不详、1／36例经口服给药、1／36例未补充；硫胺素剂量：29／35例每日100mg、3／35例剂量不详、2／35例每日50mg、1／35例每日600mg。初始治疗后34／35例临床症状在24h-1周内缓解，1／36例无反应死亡。存活34例中，19例�Objective Wernicke＇s encephalopathy （WE） is an acute neuropsychiatric syndrome resulting from thiamine deficiency, which is associated with significant morbidity and mortality. The disorder is still greatly underdiagnosed in children because of either a relatively non-specific clinical presentation in some cases or unrecognized clinical setting. The aim of this literature review was to provide knowledge of pediatric WE in an effort to assist in early diagnosis, thereby reducing the morbidity and mortality. Methods The clinical manifestations, characteristic magnetic resonance imaging （MRI）, diagnosis and treatment of one case and the other 35 cases reported in the last decade in children were summarized. Results Thirtysix cases （22 boys and 14 girls, 2-month to 16-year-old） were analyzed. All the other 35 cases except for our case had underlying diseases： improper feeding in 25/35 cases, long-time vomiting in 5/35 cases, immunosuppressive therapy in 4/35 cases, long-time total parenteral nutrition without multivitamin preparations supplementation in 3/35 cases and anorexia nervosa in 1/35 case. The classic triad （mentalstatus changes, nystagmus and opthalmoplegia, and ataxia） was seen in 6/36 cases. The other clinical manifestations included consciousness disturbance in 24/36 cases, infection in 22/36 cases, pathological reflex and muscular tension changes in 18/36 cases, convulsion in 17/36 cases, developmental delay in 4/36 cases and failure to thrive in 2/36 cases. Cerebrospinal fluid examination was performed in 31/36 cases, and a slightly raised protein concentration was seen in 7/31 cases. The cerebrospinal fluid lactate levels were detected in 4/36 cases （ all increased） , serum lactic acid levels in 7/36 cases （ 6/7 cases increased）, serum pyruvate in 4/36 cases （ all increased）, thiamine pyrophosphate effect （TPPE） in 9/36 cases （ all increased）, and serum thiamine in 2/36 cases （increased in 1/2 cases）. The brain computed tomography （CT） scan was conducted

关 键 词：Wemicke’s脑病 儿童 严重脓毒症 

分 类 号：R747.9[医药卫生—神经病学与精神病学]