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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.
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Copyright (c) 2019 Rena Okada, Yuri Sakaguchi, Takeshi Matsushige, Isamu Kamimaki, Toshiki Takenouchi, Takao Takahashi
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References
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- Chen, W.-X., Liu, H.-S., Yang, S.-D., Zeng, S.-H., Gao, Y.-Y., Du, Z.-H., … Mai, J.-N. (2016). Reversible splenial lesion syndrome in children: Retrospective study and summary of case series. Brain and Development, 38(10), 915–927. https://doi.org/10.1016/j.braindev.2016.04.011
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- Nukui, M., Kawawaki, H., Inoue, T., Kuki, I., Okazaki, S., Amo, K., … Shiomi, M. (2018). Clinical characteristics of acute encephalopathy with acute brain swelling: A peculiar type of acute encephalopathy. Brain and Development, 40(9), 792–798. https://doi.org/10.1016/j.braindev.2018.05.004
- Sakuma, H., Awaya, Y., Shiomi, M., Yamanouchi, H., Takahashi, Y., Saito, Y., … Sasaki, M. (2010). Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. Acta Neurologica Scandinavica, 121(4), 251–256. https://doi.org/10.1111/j.1600-0404.2009.01198.x
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- Mizuguchi, M. (1997). Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain and Development, 19(2), 81–92. https://doi.org/10.1016/S0387-7604(96)00063-0
- Yamanouchi, H., Shiomi, M., Awaya, Y., & Mizuguchi, M. (2009). [Current topics of acute encephalitis and encephalopathy in Japanese children]. No to Hattatsu = Brain and Development, 41(2), 124–126. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/19517777
- M Mizuguchi M AOMKYS A Oka, Takanashi J. Nation-wide Survey of Acute Childhood Encephalopathy: research on rare and intractable diseases (in Japanese).Ministry of Health, Labour and Welfare. 2012
- The Japanese Society of Child Neurology e super-vised Committee on Evaluation Guidelines for Pediatric Acute Encephalopathy. Evidence-based PracticeGuidelines for the Treatment of Pediatric Acute Encephalopathy 2016 (in Japanese). Shindan to Chiryo-sha. 2016;p. 115–9
- Tsuruta M UJNK Mori H. An atypical course of mildencephalopathy with reversible splenial lesion accompanied by prolonged fever and involuntary movement(in Japanese). No to Hattatsu (Tokyo). 2012;44(347)
- Takahashi R TMKJSSCSea Takayanagi M.EA 14-year-old boy with encephalopathy with diverse symptoms and transient splenial lesion (in Japanese). The Journal of Sendai City Hospital (Sendai). 2012;32(53-7):115–9
References
Kometani, H., Kawatani, M., Ohta, G., Okazaki, S., Ogura, K., Yasutomi, M., … Ohshima, Y. (2014). Marked elevation of interleukin-6 in mild encephalopathy with a reversible splenial lesion (MERS) associated with acute focal bacterial nephritis caused by Enterococcus faecalis. Brain and Development, 36(6), 551–553. https://doi.org/10.1016/j.braindev.2013.07.012
Chen, W.-X., Liu, H.-S., Yang, S.-D., Zeng, S.-H., Gao, Y.-Y., Du, Z.-H., … Mai, J.-N. (2016). Reversible splenial lesion syndrome in children: Retrospective study and summary of case series. Brain and Development, 38(10), 915–927. https://doi.org/10.1016/j.braindev.2016.04.011
Ka, A., Britton, P., Troedson, C., Webster, R., Procopis, P., Ging, J., … Dale, R. C. (2015). Mild encephalopathy with reversible splenial lesion: An important differential of encephalitis. European Journal of Paediatric Neurology, 19(3), 377–382. https://doi.org/10.1016/j.ejpn.2015.01.011
Nukui, M., Kawawaki, H., Inoue, T., Kuki, I., Okazaki, S., Amo, K., … Shiomi, M. (2018). Clinical characteristics of acute encephalopathy with acute brain swelling: A peculiar type of acute encephalopathy. Brain and Development, 40(9), 792–798. https://doi.org/10.1016/j.braindev.2018.05.004
Sakuma, H., Awaya, Y., Shiomi, M., Yamanouchi, H., Takahashi, Y., Saito, Y., … Sasaki, M. (2010). Acute encephalitis with refractory, repetitive partial seizures (AERRPS): a peculiar form of childhood encephalitis. Acta Neurologica Scandinavica, 121(4), 251–256. https://doi.org/10.1111/j.1600-0404.2009.01198.x
Takanashi, J. (2009). Two newly proposed infectious encephalitis/encephalopathy syndromes. Brain and Development, 31(7), 521–528. https://doi.org/10.1016/j.braindev.2009.02.012
Mizuguchi, M. (1997). Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain and Development, 19(2), 81–92. https://doi.org/10.1016/S0387-7604(96)00063-0
Yamanouchi, H., Shiomi, M., Awaya, Y., & Mizuguchi, M. (2009). [Current topics of acute encephalitis and encephalopathy in Japanese children]. No to Hattatsu = Brain and Development, 41(2), 124–126. Retrieved from http://www.ncbi.nlm.nih.gov/pubmed/19517777
M Mizuguchi M AOMKYS A Oka, Takanashi J. Nation-wide Survey of Acute Childhood Encephalopathy: research on rare and intractable diseases (in Japanese).Ministry of Health, Labour and Welfare. 2012
The Japanese Society of Child Neurology e super-vised Committee on Evaluation Guidelines for Pediatric Acute Encephalopathy. Evidence-based PracticeGuidelines for the Treatment of Pediatric Acute Encephalopathy 2016 (in Japanese). Shindan to Chiryo-sha. 2016;p. 115–9
Tsuruta M UJNK Mori H. An atypical course of mildencephalopathy with reversible splenial lesion accompanied by prolonged fever and involuntary movement(in Japanese). No to Hattatsu (Tokyo). 2012;44(347)
Takahashi R TMKJSSCSea Takayanagi M.EA 14-year-old boy with encephalopathy with diverse symptoms and transient splenial lesion (in Japanese). The Journal of Sendai City Hospital (Sendai). 2012;32(53-7):115–9