Mild encephalopathy with reversible splenial lesion associated with echovirus 6 infection: a case report and review of the literature

Background. Mild encephalopathy with reversible splenial lesion (MERS), a clinic-radiological syndrome distinguished by reversible encephalopathy onset, has been increasingly recognized in Caucasian children. Case. We describe a MERS case in a previously healthy 4-year-old girl admitted to the hospital with abnormal consciousness level, muscle weakness, dysphagia and dysarthria after a 4-day history of diarrhea and fever. Magnetic resonance imaging (MRI) of the brain showed hyperintensity in the corpus callosum splenium. Electroencephalogram was normal and cerebrospinal fluid (CSF) culture negative. The stool sample was positive for Echovirus 6 and serology test confirmed the infection. The child’s condition gradually improved and the MRI, after 15 days, depicted a normal brain. Only a mild speech impairment was persistent at discharge, which disappeared one month later. We performed a literature review about pediatric MERS cases demonstrating that infectious agents have been rarely isolated in CSF. Conclusion. MERS has an overall good prognosis independently from the treatment approach; this is confirmed by our case - one of the first reported with an Echovirus 6-related encephalopathy.

___

1. Takanashi JI, Imamura A, Hayakawa F, Terada H. Differences in the time course of splenial and white matter lesions in clinically mild encephalitis/ encephalopathy with a reversible splenial lesion (MERS). J Neurol Sci 2010; 292: 24-27.

2. Tada H, Takanashi J, Barkovich AJ, et al. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion. Neurology 2004; 63: 1854-1858.

3. Takanashi J, Barkovich AJ, Shiihara T, et al. Widening spectrum of a reversible splenial lesion with transiently reduced diffusion. AJNR Am J Neuroradiol 2006; 27: 836-838.

4. Takanashi JI. Two newly proposed encephalitis/ encephalopathy syndromes. Brain Dev 2009; 31: 521-528.

5. Kashiwagi M, Tanabe T, Shimakawa S, et al. Clinicoradiological spectrum of reversible splenial lesions in children. Brain Dev 2014; 36: 330-336.

6. Kobata R, Tsukahara H, Nakai A, et al. Transient MR signal changes in the splenium of the corpus callosum in rotavirus encephalopathy: value of diffusion-weighted imaging. J Comput Assist Tomogr 2002; 26: 825-828.

7. Karampatsas K, Spyridou C, Morrison IR, Tong CYW, Prendergast AJ. Rotavirus associated mild encephalopathy with a reversible splenial lesion (MERS)- case report and review of the literature. BMC Infect Dis 2015; 15: 446.

8. Yokoyama T, Yamada S, Doichi N, Kato E. Rotavirus-infected children with clinically mild encephalopathy with a reversible splenial lesion (MERS). BMJ Case Rep 2013: doi:10.1136/bcr-2013- 008644.

9. Fuchigami T, Goto K, Hasegawa M, et al. A 4-yearold girl with clinically mild encephalopathy with a reversible splenial lesion associated with rotavirus infection. J Infect Chemother 2013; 19: 149-153.

10. Mazur-Melewska K, Jonczyk-Potoczna, Szpura K, et al. Transient lesion in the splenium of the corpus callosum due to rotavirus infection. Childs Nerv Syst 2015; 31: 997-1000.

11. Fukuda S, Kishi K, Yasuda K, Sejima H, Yamaguchi S. Rotavirus-associated encephalopathy with a reversible splenial lesion. Pediatr Neurol 2009; 40: 131-133.

12. Kato Z, Orii KE, Morimoto M, et al. A transient lesion in the corpus callosum during rotavirus infection. Pediatr Neurol 2009; 41: 467-469.

13. Arawaka C, Fujita Y, Imai Y, et al. Detection of group A rotavirus RNA and antigens in serum and cerebrospinal fluid from two children with clinically mild encephalopathy with a reversible splenial lesion. Jpn J Infect Dis 2011; 64: 204-207.

14. Matsuoka T, Yodoshi T, Sugai M, et al. A case of mild encephalopathy with a reversible splenial lesion associated with G5P[6]rotavirus infection. Case Rep Pediatr 2013: doi: 10.1155/2013/197163.

15. Feraco P, Porretti G, Marchiò G, Bellizzi M, Recla M. Mild encephalitis/encephalopathy with reversible splenial lesion (MERS) due to cytomegalovirus: case report and review of the literature. Neuropediatrics 2018; 49: 68-71.

16. Ka A, Britton P, Troedson C, et al. Mild encephalopathy with reversible splenial lesion: an important differential of encephalitis. Eur J Paediatr Neurol 2015; 19: 377-382.

17. Fang Q, Chen L, Chen Q, Lin Z, Yang F. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion of corpus callosum in Chinese children. Brain Dev 2017; 39: 321-326.

18. Takanashi JI, Barkovich AJ, Yamaguchi KI, Kohno Y. Influenza-associated encephalitis/encephalopathy with a reversible lesion in the splenium of the corpus callosum: a case report and literature. AJNR Am J Neuroradiol 2004; 25: 798-802.

19. Britton PN, Dale RC, Blyth CC, et al; ACE study investigators and PAEDS network Influenzaassociated encephalitis/encephalopathy identified by the Australian childhood encephalitis study 2013- 2015. Pediatr Infect Dis J 2017; 36: 1021-1026.

20. Vanderschueren G, Schotsmans K, Maréchal E, Crols R. Mild encephalitis with reversible splenial (MERS) lesion syndrome due to Influenza B virus. Pract Neurol 2018; 18: 391-392.

21. Fluss J, Ferey S, Menache-Starobinski C, Delavelle J, Van Bogaert P, Vargas MI. Mild influenza-associated encephalopathy/encephalitis with a reversible splenial lesion in a Caucasian child with additional cerebellar features. Eur J Paediatr Neurol 2010; 14: 97-100.

22. Ganapathy S, Ey EH, Wolfson BJ, Khan N. Transient isolated lesion of the splenium associated with clinically mild influenza encephalitis. Pediatr Radiol 2008; 38: 1243-1245.

23. Matsubara K, Kodera M, Nigami H, Yura K, Fukaya T. Reversible splenial lesion in influenza virus encephalopathy Pediatr Neurol 2007; 37: 431-434.

24. Morichi S, Kawashima H, Ioi H, Yamanaka G, Kashiwagi Y, Hoshika A. High production of interleukin-10 and interferon-γ in influenzaassociated MERS in the early phase. Pediatr Int 2012; 54: 536-562.

25. Shah S, Keil A, Gara K, Gara K, Nagarajan L. Neurologic complication of influenza. J Child Neurol 2014; 29: NP49-NP53.

26. Iwata A, Matsubara K, Nigami H, Kamimura K, Fukaya T. Reversible splenial lesion associated with Novel Influenza A (H1N1) viral infection. Pediatr Neurol 2010; 42: 447-450.

27. Miyata R, Tanuma N, Hayashi M, et al. Oxidative stress in patients with clinically mild encephalitis/ encephalopathy with a reversible splenial lesion (MERS). Brain Dev 2012; 34: 124-127.

28. Chen WX, Liu HS, Yang SD, et al. Reversible splenial lesion syndrome in children: retrospective study and summary of case series. Brain Dev 2016; 38: 915-927.

29. Halpern LA, Agyeman P, Steinlin M, El-Koussy M, Grunt S. Mild encephalopathy with splenial lesion and parainfluenza virus infection. Pediatr Neurol 2013; 48: 252-254.

30. Hatanaka M, Kashiwagi M, Tanabe T, Nakahara H, Ohta K, Tamai H. Overlapping MERS and mild AESD caused by HHV-6 infection. Brain Dev 2015; 37: 334-338.

31. Hara M, Mizuochi T, Kawano G, et al. A case of clinically mild encephalitis with a reversible splenial lesion (MERS) after mumps vaccination. Brain Dev 2011; 33: 842-844.

32. Takanashi JI, Shiihara T, Hasegawa T, et al. Clinically mild encephalitis with a reversible splenial lesion (MERS) after mumps vaccination. J Neurol Sci 2015; 349: 226-228.

33. Suzuki H, Kusaka T, Okada H. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion caused by human parvovirus B19 infection: a case of two brothers with hereditary spherocytosis. Pediatr Neurol 2014; 51: 470-472.

34. Yagamguchi H, Ishida T, Yokoi T, et al. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion accompanied by Epstein-Barr virus hemophagocytic lymphohistiocytosis: a case report and review of the literature. J Pediatr Hematol Oncol 2017; 39: e92-e96.

35. Azuma J, Nabatame S, Katsura T, et al. Marked elevation of urinary β2-microglobulin in patients with reversible splenial lesions: a small case series. J Neurol Sci 2016; 368: 109-112.

36. Ueda N, Minami S, Akimoto M. Mycoplasma pneumoniae-associated mild encephalitis/ encephalopathy with a reversible splenial lesion: report of two pediatric cases and a comprehensive literature review. BMC Infect Dis 2016; 16: 671.

37. Yuan ZF, Shen J, Mao SS, et al. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion associated with Mycoplasma pneumoniae infection. BMC Infect Dis 2016; 16: 230.

38. Kawagoshi R, Ono J. A case of Mycoplasmaassociated encephalitis/encephalopathy with a reversible splenial lesion. Meiwa Igaku Zasshi 2015; 2: 35-40. [in Japanese].

39. Tokunaga Y. 1 girl case who showed transient of corpus callosum lesion with P-248 mycoplasmal diseases. No to Hattatsu 2008; 40(Suppl): 396. [in Japanese].

40. Ohgoshi Y, Sakai T, Nonaka S, Nakamura Y, Hosaki A, Bessho F. A case of mild encephalitis/ encephalopathy with a reversible splenial lesion associated with Mycoplasma pneumonia. Nihon Shonika Gakkai Zasshi 2009; 113: 350. [in Japanese].

41. Nakamoto T, Tanaka K, Koga H, Kan N, Takahashi S. A case of mild encephalitis/encephalopathy with a reversible splenial lesion during Mycoplasma pneumoniae infection. Nihon Shonika Gakkasi Zasshi 2012; 116: 1255. [in Japanese].

42. Uchida Y, Morita H, Miyazaki K, Adachi S, Tatebayashi K, Kaneko H. A case of menimgoencephalitis with a reversible splenial lesion due to Mycoplasma pneumoniae infection. Shoni Kansen Menneki 2013; 25: 201-202. [in Japanese].

43. Osuka S, Imai H, Ishikawa E, et al. Mild encephalitis/ encephalopathy with a reversible splenial lesion: evaluation by diffusion tensor imaging. Two case reports. Neorol Med Chir (Tokyo) 2010; 50: 1118- 1122.

44. Notebaert A, Willems J, Coucke L, Van Coster R, Verhelst H. Expanding the spectrum of MERS type 2 lesions, a particular form of encephalitis. Pediatr Neurol 2013; 48: 135-138.

45. Jain S, Patel B, Bhatt GC. Enteroviral encephalitis in children: clinical features, pathophysiology and treatment advances. Pathog Glob Health 2014; 108: 216-222.

46. Pallansch MA, Roos R. Enteroviruses: polioviruses, coxsackieviruses, echoviruses, and newer enteroviruses. . In: Knipe DM, Howley PM (eds). Fields Virology (5th edt).Philadelphia: Lippincott Williams & Wilkins, 2007: 839-894.

47. Hoshino A, Saitoh M, Oka A, et al. Epidemiology of acute encephalopathy in Japan, with emphasis on the association of viruses and syndromes. Brain Dev 2012; 34:337-343.

48. Kurokawa Y, Masuda H, Kobayashi H, et al. Effective therapy with infliximab for clinically mild encephalitis/encephalopathy with a reversible splenial lesion in an infant with Kawasaki disease. Nihon Rinsho Meneki Gakkai Kaishi 2017; 40: 190- 195.

49. Benschop KS, Geeraedts F, Beuvink B, et al; VIROTypeNed Increase in ECHOvirus 6 infections associated with neurological symptoms in the Netherlands, June to August 2016. Euro Surveil 2016; 21: 30351.

50. Hosoda A, Gatayama R, Moriyama S, et al. The first case of recurrent ultra late onset group B streptococcal sepsis in a 3-year-old child. IDCases 2017; 7: 16-18.

51. Hashimoto Y, Takanashi JI, Kaiho K, et al. A splenial lesion with transiently reduced diffusion in clinically mild encephalitis is not always reversible. A case report. Brain Dev 2009; 31 :710-712.

52. Ikeno M, Abe S, Kurahashi H, Takasu M, Shimizu T, Okumura A. Gastric perforation and critical illness polyneuropathy after steroid treatment in a patient with encephalitis/encephalopathy with transient splenial lesion. Brain Dev 2016; 39: 356-360.

53. Nozaki F, Kumada T, Miyajima T, et al. Reversible splenic lesion in a patient with febrile infection related epilepsy syndrome. Neuropediatrics 2013; 44: 291-294.

54. Pan JJ, Zhao YY, Lu C, Hu YH, Yang Y. Mild encephalitis/encephalopathy with a reversible splenial lesion: five cases and a literature review. Neurol Sci 2015; 36: 2043-2051.

55. Sun D, Chen WH, Baralc S, et al. Mild encephalopathy/ encephalitis with a reversible splenial lesion (MERS): a report of five neonatal cases. J Huazhong Univ Sci Technol [Med Sci] 2017; 37: 433-438.

56. Itamura S, Kamada M, Nakagawa N. Kawasaki disease complicated with reversible splenial lesion and acute myocarditis. Pediatr Cardiol 2011; 32: 696- 699.

57. Takanashi JI, Shirai K, Sugawara Y, Okamoto Y, Obonai T, Terada H. Kawasaki disease complicated by mild encephalopathy with a reversible splenial lesion (MERS). J Neurol Sci 2012; 315: 167-169.

58. Kometani H, Kawatani M, Ohta G, et al. 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 Dev 2013; 36: 551-553.

59. Imamura T, Takanashi JI, Yasugi J, Terada H, Nishimura A. Sisters with clinically mild encephalopathy with a reversible splenial lesion (MERS)-like features; familial MERS? J Neurol Sci 2010; 290: 153-156.

60. Yıldız AE, Maraş Genç H, Gürkaş E, Akmaz Ünlü H, Öncel İH, Güven A. Mild encephalitis/ encephalopathy with a reversible splenial lesion in children. Diagn Interv Radiol 2018; 24: 108-112.
Turkish Journal of Pediatrics-Cover
  • ISSN: 0041-4301
  • Yayın Aralığı: Yılda 6 Sayı
  • Başlangıç: 1958
  • Yayıncı: Hacettepe Üniversitesi Çocuk Sağlığı Enstitüsü Müdürlüğü
Sayıdaki Diğer Makaleler

Maternal and fetal tuberous sclerosis complex: a case report questioning clinical approach

Özge Sürmeli ONAY, Adviye ÇAKIL SAĞLIK, Pelin KOSGER, Zeynep SARAÇOĞLU, Uğur TOPRAK, Birsen UCAR, AYŞE NESLİHAN TEKİN

Tetanus; a forgotten infection disease: a report of two cases

Ülkem KOÇOĞLU BARLAS, Hasan Serdar KIHTIR, Osman YEŞİLBAŞ, Mey Talip PETMEZCİ, Nihal AKÇAY, Ercüment PETMEZCİ, Nevin HATİPOĞLU, Esra ŞEVKETOĞLU

Ambulatory arterial stiffness index is increased in obese children

Mehmet TAŞDEMİR, Ergin ERGİNÖZ, Özlem BOSTAN GAYRET, İlmay BİLGE

A rare case of syndromic severe congenital neutropenia: JAGN1 mutation

Funda ÇİPE, Çiğdem AYDOĞMUŞ, Avniye Kübra BASKIN, Gonca KESKİNDEMİRCİ, Wojciech GARNCARZ, Kaan BOZTUĞ

Congenital afibrinogenemia in a 4-year-old girl complicated with acute lymphoblastic leukemia

Alper OZCAN, Bahadır SAMUR, Şefika AKYOL, Arda ERDOGMUS, Turkan PATIROGLU, MUSA KARAKÜKCÜ, Ekrem ÜNAL

The corrected QT interval prolongation in adolescents with cardiac iron overload β-thalassemia major

Najib ADVANI, Ahmad KAUTSAR, Murti ANDRIASTUTI

Aortic balloon valvuloplasty and mid-term results in newborns: a single center experience

Birgül VARAN, Kahraman YAKUT, İlkay ERDOĞAN, Murat ÖZKAN, Kürşad TOKEL

Bone marrow involvement in pediatric malignancies: a comparison study of Positron emission tomographycomputed tomography and bone marrow biopsy

Özlem TEZOL, Fatih SAĞCAN, Pelin ÖZCAN KARA, Elvan Çaglar ÇITAK

Pediatric case of persistent hiccups associated with hypertrophic olivary degeneration

Pınar ARICAN, Özgür ÖZTEKİN, Dilek ÇAVUŞOĞLU, Sema BOZKAYA YILMAZ, Atilla ERSEN, Nihal OLGAÇ DÜNDAR, PINAR GENÇPINAR

Children with lymphoma presenting with hemophagocytic lymphohistiocytosis

Tekin AKSU, Turan BAYHAN, Belgin GÜLHAN, Selma ÇAKMAKÇI, Ayşe Selcen OĞUZ, Neşe YARALI, Namık Yaşar ÖZBEK, İnci ERGÜRHAN İLHAN