TY - JOUR
T1 - Diagnostic Clues to Human Herpesvirus 6 Encephalitis and Wernicke Encephalopathy after Pediatric Hematopoietic Cell Transplantation
AU - Sadighi, Zsila
AU - Sabin, Noah D.
AU - Hayden, Randall
AU - Stewart, Elizabeth
AU - Pillai, Asha
N1 - Funding Information:
The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: This research was supported by K08 #HL-088260-06 (NHLBI) (AP), the V Scholar Award of the V Foundation for Cancer Research (AP) and the American Lebanese Syrian Association of Charities (ALSAC), the funding arm of St Jude Children’s Research Hospital (ZS and AP).
PY - 2015/9/18
Y1 - 2015/9/18
N2 - 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.
AB - 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.
KW - Wernicke encephalopathy
KW - central nervous system
KW - hematopoietic cell transplant
KW - human herpesvirus 6 encephalitis
KW - thiamine
UR - http://www.scopus.com/inward/record.url?scp=84939540496&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84939540496&partnerID=8YFLogxK
U2 - 10.1177/0883073814560628
DO - 10.1177/0883073814560628
M3 - Article
C2 - 25564483
AN - SCOPUS:84939540496
VL - 30
SP - 1307
EP - 1314
JO - Journal of Child Neurology
JF - Journal of Child Neurology
SN - 0883-0738
IS - 10
ER -