儿童川崎病合并轻微脑炎/脑病伴可逆性胼胝体压部病变综合征1例并文献复习

doi:10.19723/j.issn.1671-167X.2022.04.028

摘要/Abstract

摘要：

报道1例川崎病合并轻微脑炎/脑病伴可逆性胼胝体压部病变综合征(mild encephalitis/encephalopathy with a reversible splenial lesion，MERS)患儿的临床诊疗经过，并回顾相关文献报道，总结疾病特点，提高对该病的认识。本例患者为7岁余男孩，持续高热6 d，伴草莓舌、双眼球结膜充血、全身大片红斑样充血样皮疹和颈部淋巴结肿大，符合川崎病诊断标准。丙种球蛋白(2 g/kg)静脉滴注24 h后患儿仍有发热，且出现头痛、嗜睡表现，头颅磁共振成像(magnetic resonance imaging，MRI)显示胼胝体压部局限性梭形肿胀，T1稍低、T2稍高异常信号，扩散加权成像(diffusion-weighted images，DWI)呈明显高信号，表观扩散系数(apparent diffusion coefficient，ADC)图呈明显低信号，提示MERS，予甲泼尼龙2 mg/(kg·d)静脉滴注，数小时后患儿热退，头痛、嗜睡症状消失。1周后复查头颅MRI正常，出院时没有神经系统异常和冠状动脉扩张。共检索到符合条件的外文文献12篇，未检索到中文文献，共报道17例川崎病合并MERS患儿，中位年龄6.5岁(1~14岁)，其中5岁以上儿童11例，合并冠状动脉扩张者4例。所有患儿均有不同程度的意识障碍、幻视、惊厥等神经系统症状，头颅MRI符合MERS影像学改变，经积极治疗, 所有患儿的神经系统症状完全消失，其中13例患儿复查了头颅MRI，影像学改变均恢复正常，所有患儿均未遗留神经系统后遗症。川崎病合并MERS的病例报道非常少见，并发MERS的川崎病多发生在5岁以上年长儿童，头颅MRI检查有助于早期诊断，及时积极治疗可以使MERS病情短期内逆转，不留神经系统后遗症。

关键词: 黏膜皮肤淋巴结综合征, 轻微脑炎/脑病伴可逆性胼胝体压部病变综合征, 儿童

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

中图分类号:

R729

杜燕燕,王健,贺兰,季丽娜,徐樨巍. 儿童川崎病合并轻微脑炎/脑病伴可逆性胼胝体压部病变综合征1例并文献复习[J]. 北京大学学报（医学版）, 2022, 54(4): 756-761.

Yan-yan DU,Jian WANG,Lan HE,Li-na JI,Xi-wei XU. Kawasaki disease complicated with mild encephalitis/encephalopathy with a reversible splenial lesion: A case report and literature review[J]. Journal of Peking University (Health Sciences), 2022, 54(4): 756-761.

图/表 2

图1

表1

18例川崎病合并MERS患儿的临床资料"

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

表1

参考文献 26

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