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Journal of Peking University (Health Sciences) ›› 2022, Vol. 54 ›› Issue (4): 756-761. doi: 10.19723/j.issn.1671-167X.2022.04.028

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Kawasaki disease complicated with mild encephalitis/encephalopathy with a reversible splenial lesion: A case report and literature review

Yan-yan DU1,Jian WANG1,Lan HE1,Li-na JI1,*(),Xi-wei XU1,2   

  1. 1. Department of Pediatrics, Beijing Tsinghua Changgung Hospital Affiliated to Tsinghua University, School of Clinical Medicine of Tsinghua University, Beijing 102218, China
    2. Department of Gastroenterology, Beijing Children' s Hospital affiliated to Capital Medical University & National Center for Children' s Health, Beijing 100045, China
  • Received:2021-12-08 Online:2022-08-18 Published:2022-08-11
  • Contact: Li-na JI E-mail:jlna01103@btch.edu.cn

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Abstract:

We reported a pediatric case of Kawasaki disease complicated with mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) from Beijing Tsinghua Changgung Hospital. The clinical data were retrospectively analyzed and the related literature was reviewed. The clinical features, treatment and prognosis of the disease were summarized to improve recognition of Kawasaki disease complicated with MERS. A 7-year-old boy was diagnosed with Kawasaki disease due to continuous high fever for 6 d, accompanied by strawberry tongue, conjunctival congestion, erythema-like hyperemia rash, and cervical enlarged lymph nodes. And treatment was started with intravenous immunoglobulin (IVIG: 2 g/kg) and oral aspirin [40 mg/(kg·d)]. Twenty-four hours after the treatment of IVIG, the patient' s fever persisted and in addition he developed headache and drowsiness. His cranial magnetic resonance imaging (MRI) demonstrated a localized lesion in the splenium of the corpus callosum with high intensity signal on diffusion-weighted images (DWI) and T2-weighted, and low intensity signal on apparent diffusion coefficient (ADC) and T1-weighted. Based on these findings, he was diagnosed with MERS-complicated Kawasaki disease. Methylprednisolone [2 mg/(kg·d)] treatment was started intravenously, and within several hours he was afebrile and the neurological symptoms disappeared. A follow-up MRI was conducted after 1 week was normal. He was discharged without any neurological sequelae and coronary artery lesions. A total of 12 qualified foreign literature were retrieved, with no Chinese literature searched. Seventeen children were reported, the median age was 6.5 years (range: 1-14 years), among them 11 cases were children over 5 years old, and 4 cases were complicated with coronary artery lesions. All children had neurological symptoms, such as consciousness disorder, visual hallucination or convulsion. MRI conformed to MERS imaging changes. After active treatment, the neurological manifestations and radiological abnormalities completely disappeared, leaving no neurological sequelae. Kawasaki disease complicated with MERS had not been reported in China by now. Literature that identified Kawasaki disease complicated with MERS mostly occurred in children over 5 years old. Cranial MRI examination is helpful for early diagnosis. Timely treatment can reverse MERS in a short time, without neurological sequelae left.

Key words: Mucocutaneous lymph node syndrome, Mild encephalitis/encephalopathy with a reversible splenial lesion, Child

CLC Number: 

  • R729

Figure 1

Cranial MRI before and after treatment A, diffusion-weighted images (DWI) revealed area of high intensity in the splenium of the corpus callosum (arrow) before treatment; B, apparent diffusion coefficient (ADC) revealed area of low intensity in the splenium of the corpus callosum (arrow) before treatment; C, D showed that the abnormal signal lesions of the splenium of the corpus callosum disappeared after treatment."

Table 1

Clinical data of 18 children diagnosed as Kawasaki disease complicated with MERS"

Case no. Study Age/years Gender IVIG Other therapies Na/(mmol/L) Consciousness disturbance Neurological symptom onset Neurological prognosis Coronary sequelae
1 Itamura et al[4] 14 Female 1.8 g/kg - 128 Headache, visual hallucination, consciousness disorder Day 5 CR RCA 8 mm LAD 5.1 mm
2 Sato et al[5] 7 Female 2g/kg - 131 Drowsiness, illusion, emotional changes Day 2 CR No
3 Takanashi et al[6] 2 Female 2 g/kg×2 d PSL 134 Drowsiness, visual hallucination Day 10 CR No
4 Takanashi et al[6] 7 Female 2 g/kg - 129 Drowsiness, visual hallucination, incoherent speech, impulsive behavior Day 3 CR No
5 Takanashi et al[6] 8 Male 2 g/kg×3 d CsA, IFX 119 Drowsiness, incoherentspeech, unresponsiveness Day 1 CR 5 mm(NA)
6 Takanashi et al[6] 10 Female 2 g/kg×2 d - 127 Drowsiness, visual hallucination, emotional changes, incoherent speech Day 3 CR No
7 Kurokawa et al[2] 2 Female 2 g/kg×2 d IVMP, IFX 133 Convulsion, consciousness disorder Day 2 CR No
8 Takahashi et al[7] 2 Male 2 g/kg×2 d IVMP 133 Convulsion, visual hallucination, consciousness disorder Day 1 CR No
9 Tsukamoto et al[8] 1 Female 2 g/kg×2 d IVMP 125 Convulsion, cnsciousness disorder Day 2 CR No
10 松村et al[9] 5 Male 2 g/kg PSL NA Consciousness disorder Day 6 CR No
11 松村et al[9] 8 Male NA - NA Consciousness disorder Day 5 CR RCA 3 mm LAD 5 mm
12 八木et al[10] 11 Female NA - 143 Aphasia, ataxia Day 3 CR No
13 森et al[11] 2 Male NA IVMP, PE NA Drowsiness, consciousness disorder Day 2 CR Yes (NA)
14 Yoshihara et al[12] 2 Female 2 g/kg IVMP 131 Consciousness disorder, incoherent speech Day 4 CR No
15 Kashiwagi et al[13] 10 Female NA IVMP 127 Visual hallucination, incoherent speech, impulsive behavior Day 2 CR NA
16 Kashiwagi et al[13] 6 Male NA IVMP 129 Visual hallucination, emotional changes, impulsive behavior, incoherent speech Day 3 CR NA
17 Ka et al[14] 6 Female 2d (NA) - NA Emotional changes, drowsiness Day 7 CR NA
18 This study 7 Male 2 g/kg PSL 128 Headache, drowsiness Day 8 CR No
1 Newburger JW , Takahashi M , Gerber MA , et al. Diagnosis, treatment, and long-term management of Kawasaki disease: A statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association[J]. Pediatrics, 2004, 114 (6): 1708- 1733.
doi: 10.1542/peds.2004-2182
2 Kurokawa Y , Masuda H , Kobayashi T , et al. Effective therapy with infliximab for clinically mild encephalitis/encephalopathy with a reversible splenial lesion in an infant with Kawasaki disease[J]. Nihon Rinsho Meneki Gakkai Kaishi, 2017, 40 (3): 190- 195.
doi: 10.2177/jsci.40.190
3 Kobayashi T , Inoue Y , Takeuchi K , et al. Prediction of intravenous immunoglobulin unresponsiveness in patients with Kawasaki disease[J]. Circulation, 2006, 113 (22): 2606- 2612.
doi: 10.1161/CIRCULATIONAHA.105.592865
4 Itamura S , Kamada M , Nakagawa N , et al. Kawasaki disease complicated with reversible splenial lesion and acute myocarditis[J]. Pediatr Cardiol, 2011, 32 (5): 696- 699.
doi: 10.1007/s00246-011-9937-4
5 Sato T , Ushiroda Y , Oyama T , et al. Kawasaki disease-associated MERS: Pathological insights from SPECT findings[J]. Brain Dev, 2012, 34 (7): 605- 608.
doi: 10.1016/j.braindev.2011.09.015
6 Takanashi J , Shirai K , Sugawara Y , et al. Kawasaki disease complicated by mild encephalopathy with a reversible splenial lesion (MERS)[J]. J Neurol Sci, 2012, 315 (1/2): 167- 169.
7 Takahashi KI , Homma S , Suzuki K , et al. A case of Kawasaki disease complicated by clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS)[J]. J Tokyo Wom Med Univ, 2017, 87 (Suppl 1): 118- 124.
8 Tsukamoto Y , Majima H , Inukai S . A girl of Kawasaki disease associated with MERS after diagnosis of febrile seizure[J]. J Jpn Soc Pediatr Radiol, 2021, 37 (1): 113- 118.
9 松村雄, 阿久津裕子, 倉信大, ほか. 川崎病に合併した脳症の症例報告[J]. 心臓, 2014, 46 (12): 1648- 1649.
10 八木文子, 中田桂, 川上睦美, ほか. Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS)2型の2例[J]. 臨床放射線, 2018, 63 (8): 911- 918.
11 森秀洋, 澤田真理子, 佐藤一寿, ほか. 可逆性脳梁膨大部病変を有する軽症脳炎·脳症を合併した川崎病に対する血漿交換療法[J]. 日本小児科学会雑誌, 2020, 124 (10): 1514- 1519.
12 Yoshihara S , Fujita Y , Miyamoto K , et al. Kawasaki disease with mild encephalitis/encephalopathy with reversible splenial lesion in a 2-year-old girl[J]. Indian J Pediatr, 2021, 88 (7): 718.
doi: 10.1007/s12098-021-03779-5
13 Kashiwagi M , Tanabe T , Shimakawa S , et al. Clinico-radiological spectrum of reversible splenial lesions in children[J]. Brain Dev, 2014, 36 (4): 330- 336.
doi: 10.1016/j.braindev.2013.05.007
14 Ka A , Britton P , Troedson C , et al. Mild encephalopathy with reversible splenial lesion: An important differential of encephalitis[J]. Eur J Paediatr Neurol, 2015, 19 (3): 377- 382.
doi: 10.1016/j.ejpn.2015.01.011
15 Tada H , Takanashi J , Barkovich AJ , et al. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion[J]. Neurology, 2004, 63 (10): 1854- 1858.
doi: 10.1212/01.WNL.0000144274.12174.CB
16 Takanashi J . Two newly proposed infectious encephalitis/encephalopathy syndromes[J]. Brain Dev, 2009, 31 (7): 521- 528.
doi: 10.1016/j.braindev.2009.02.012
17 Takanashi J , Imamura A , Hayakawa F , et al. Differences in the time course of splenial and white matter lesions in clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS)[J]. J Neurol Sci, 2010, 292 (1/2): 24- 27.
18 Starkey J , Kobayashi N , Numaguchi Y , et al. Cytotoxic lesions of the corpus callosum that show restricted difusion: Mechanisms, causes, and manifestations[J]. Radiographics, 2017, 37 (2): 562- 576.
doi: 10.1148/rg.2017160085
19 Takanashi J , Tada H , Maeda M , et al. Encephalopathy with a reversible splenial lesion is associated with hyponatremia[J]. Brain Dev, 2009, 31 (3): 217- 220.
doi: 10.1016/j.braindev.2008.04.002
20 Watanabe T , Abe Y , Sato S , et al. Hyponatremia in Kawasaki disease[J]. Pediatr Nephrol, 2006, 21 (6): 778- 781.
doi: 10.1007/s00467-006-0086-6
21 Rife E , Gedalia A . Kawasaki Disease: An Update[J]. Curr Rheumatol Rep, 2020, 22 (10): 75.
doi: 10.1007/s11926-020-00941-4
22 Terai M , Honda T , Yasukawa K , et al. Prognostic impact of vascular leakage in acute Kawasaki disease[J]. Circulation, 2003, 108 (3): 325- 330.
doi: 10.1161/01.CIR.0000079166.93475.5F
23 Miyata R , Tanuma N , Hayashi M , et al. Oxidative stress in patients with clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS)[J]. Brain Dev, 2012, 34 (2): 124- 127.
doi: 10.1016/j.braindev.2011.04.004
24 Ichiyama T , Nishikawa M , Hayashi T , et al. Cerebral hypoperfusion during acute Kawasaki disease[J]. Stroke, 1998, 29 (7): 1320- 1321.
doi: 10.1161/01.STR.29.7.1320
25 Hikita T , Kaminaga T , Wakita S , et al. Regional cerebral blood flow abnormalities in patients with kawasaki disease[J]. Clin Nucl Med, 2011, 36 (8): 643- 649.
doi: 10.1097/RLU.0b013e318217adfc
26 多田弘子, 高梨潤一. 可逆性脳梁膨大部病変を有する軽症脳炎脳症―up-to-date[J]. 日本小児科学会雑誌, 2019, 123 (5): 814- 823.
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