Sadighi Zsila, Sabin Noah D, Hayden Randall, Stewart Elizabeth, Pillai Asha
Department of Neurology, St Jude Children's Research Hospital, Memphis, TN, USA
St Jude Children's Research Hospital, Memphis, TN, USA.
J Child Neurol. 2015 Sep;30(10):1307-14. doi: 10.1177/0883073814560628. Epub 2015 Jan 6.
Human herpesvirus 6 (HHV6) encephalitis and Wernicke encephalopathy are treatable yet frequently undiagnosed causes of encephalopathy in pediatric recipients of allogeneic and autologous hematopoietic cell transplantation. Here we review representative cases of both conditions to highlight specific and relevant neurologic features that prompted effective diagnosis and treatment. Two patients with confusion accompanied by seizures, memory changes, or specific visual hallucinations and HHV6 detectable by polymerase chain reaction (PCR) in cerebrospinal fluid had improvement in viral load with ganciclovir or foscarnet treatment. Two patients had confusion, ataxia, or ocular changes and low serum thiamine levels, which resolved with parenteral thiamine. In all cases, definitive diagnosis and treatment were facilitated by a high index of suspicion and search for specific pathognomonic neurologic deficits accompanying the confusional state. It is critical to clinically differentiate these 2 conditions from other common neurologic syndromes occurring after transplant, allowing potentially improved patient outcomes by prompt diagnosis and effective treatment.
人疱疹病毒6型（HHV6）脑炎和韦尼克脑病是异基因和自体造血细胞移植儿科受者中可治疗但常未被诊断的脑病病因。在此，我们回顾这两种病症的典型病例，以突出促使有效诊断和治疗的特定且相关的神经学特征。两名伴有癫痫发作、记忆改变或特定视幻觉且脑脊液中通过聚合酶链反应（PCR）可检测到HHV6的意识模糊患者，经更昔洛韦或膦甲酸钠治疗后病毒载量有所改善。两名有意识模糊、共济失调或眼部改变且血清硫胺素水平低的患者，经胃肠外补充硫胺素后症状得到缓解。在所有病例中，高度怀疑并寻找意识模糊状态伴随的特定特征性神经功能缺损有助于明确诊断和治疗。临床上将这两种病症与移植后出现的其他常见神经综合征区分开来至关重要，通过及时诊断和有效治疗可能改善患者预后。
