Abstract
Background: Acute encephalopathy during childhood represents a highly heterogeneous group of infectious and non-infectious pathologies. According to a recent nationwide survey on acute childhood encephalopathy in Japan, the combination of clinical and radiographic features left approximately half of the affected children unclassified, mainly because of the lack of disease-specific biomarkers.
 Case: Herein, we document a school-aged boy who manifested with acute encephalopathy that was characterized by a prolonged fever, altered mental status, urinary retention, and intention tremor lasting for more than a month. Accompanying features included syndrome of inappropriate secretion of antidiuretic hormone, pleocytosis with elevated interleukin-6 and interferon-gamma levels in the cerebrospinal fluid, and a transient splenial lesion on neuroimaging. No pathogens were identified, and C-reactive protein was negative throughout his clinical course. This constellation of clinical features was not compatible with any of the existing entities of acute pediatric encephalopathy.
 Discussion: Our retrospective literature review identified two additional school-aged male patients who exhibited highly similar clinical courses. The prolonged altered mental status with pleocytosis in the cerebrospinal fluid and a transient splenial lesion in the absence of serum inflammatory markers suggest a primary central nervous system pathology.
 Conclusion: This combination of features defines this presumably new group of acute childhood febrile encephalopathy with prolonged fever and ataxia in school-aged boys.
Highlights
DiscussionOur retrospective literature review identified two additional school-aged male patients who exhibited highly similar clinical courses
Acute childhood encephalopathy is predominantly seen in East Asian countries and comprises a heterogeneous group of infectious and non-infectious disorders [1]
As a result of a recent series of nationwide surveys conducted by Mizuguchi, et al, 2007 - 2010 [6], our understanding of this highly heterogeneous group of patients has progressed significantly to the point where up to 50% of affected children can be classified based on a combination of clinical and radiographic features, even in the absence of specific biomarkers
Summary
A previously healthy nine-year-old boy presented with a subacute onset of fever of 39 ◦C, frontal throbbing headache, and vomiting He was seen at a local hospital, where his physical examination and routine laboratory tests were uninformative. We report on a nine-year-old boy who presented with subacute onset of febrile encephalopathy, characterized by a prolonged high fever with altered mental status and ataxia lasting for more than one month. Our review of the three patients showed that they shared the following characteristics [see Table 1]: they were all school-aged boys who had a prolonged fever, altered mental status lasting for more than three weeks, ataxia lasting for more than one month, SIADH, pleocytosis in the CSF, negative blood cultures and C-reactive protein in peripheral blood, reversible splenial lesions on neuroimaging, and no residual neurological deficits at a remote follow-up. The presence of an altered mental status with pleocytosis in the CSF and a transient splenial lesion in the absence of serum inflammatory markers does not prove, but does
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callMore From: Journal of the International Child Neurology Association
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
