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兰道-克莱夫纳综合征、注意力缺陷多动障碍(ADHD)与病毒/自身免疫性脑炎:一名六岁男孩诊断与管理中的挑战

Landau-Kleffner Syndrome, Attention-Deficit/Hyperactivity Disorder (ADHD), and Viral/Autoimmune Encephalitis: Challenges in the Diagnosis and Management of a Six-Year-Old Boy.

作者信息

Althagafi Lujain, Al Fuhayd Rahaf, Almeathem Fatimah K, Almeshal Razan A, Al-Amri Lma J, Mustafa Wessal A

机构信息

Psychiatry and Behavioral Sciences, King Abdullah bin Abdulaziz University Hospital (KAAUH), Riyadh, SAU.

出版信息

Cureus. 2024 Jan 11;16(1):e52133. doi: 10.7759/cureus.52133. eCollection 2024 Jan.

Abstract

In this case, we discuss the difficulties and challenges faced when diagnosing and treating a six-year-old boy presenting with abnormal behaviors and difficulty in concentration and inattentiveness, followed by regression of expressive language. These symptoms were then followed by hyperactivity, bouts of anger, and difficulty sleeping. The patient was seen by a psychiatrist, and he was diagnosed with attention-deficit/hyperactivity disorder (ADHD) initially and treated with little to no improvement. He was then recommended to see a neurologist by his psychiatrist and underwent a series of investigations, which included the following: brain MRI with contrast, magnetic resonance (MR) spectroscopy, cerebrospinal fluid (CSF) routine, CSF N-methyl-D-aspartate (NMDA) receptor antibody and glutamate decarboxylase (GAD) antibody analysis, and a CSF meningitis multiplex polymerase chain reaction, thyroid function, ammonia, lactate, creatine kinase, liver function, and metabolic screening of urine organic acids (UOAs), all of which revealed no abnormalities. Vitamin D was low at 38 ng/ml (>50). An electroencephalogram (EEG) done under standard conditions and provocative stimulation was abnormal with bilateral central and frontal discharge and more activity on the right side, revealing a background activity of moderately organized alpha waves (8-13 Hz) and bursts of sharp and slow wave activities that were accentuated by photic stimulation. Polysomnography showed poor sleep efficiency of 84.7%, and rapid eye movement (REM) stage was not reached due to interrupted sleep. He was then diagnosed with epileptic encephalopathy and Landau-Kleffner syndrome (LKS). The patient was prescribed sodium valproate, intravenous immunoglobulin (IVIG), and pulse steroids, with no major improvement, risperidone was added but was poorly tolerated, and the dose tapered off and eventually discontinued. Methylphenidate was the prescribed starting at 5 mg, and the dose was gradually increased to 20 mg/day given separately as 10 mg twice a day. A week later, melatonin 2 mg was added. Three months, later the EEG was repeated and was normal, and sodium valproate was tapered off and eventually discontinued. Later on that year, he was diagnosed with COVID-19 and developed acute myositis as a complication, methylphenidate was stopped, and only sertraline and melatonin were continued. MRI was repeated only this time, showing evidence of viral/autoimmune encephalitis (AIE) sequela, and IV corticosteroids were given alongside IVIG. He was discharged on prednisolone, and a month later, major improvements were seen in all aspects.

摘要

在本病例中,我们讨论了诊断和治疗一名六岁男孩时所面临的困难与挑战。该男孩出现行为异常、注意力不集中及易分心的情况,随后出现表达性语言能力倒退。这些症状之后又伴有多动、阵阵发怒及睡眠困难。一名精神科医生对该患者进行了诊治,最初诊断为注意力缺陷多动障碍(ADHD)并给予治疗,但几乎没有改善。随后其精神科医生建议他去看神经科医生,患者接受了一系列检查,包括:增强脑部磁共振成像(MRI)、磁共振波谱分析(MR)、脑脊液(CSF)常规检查、脑脊液N-甲基-D-天冬氨酸(NMDA)受体抗体和谷氨酸脱羧酶(GAD)抗体分析、脑脊液脑膜炎多重聚合酶链反应、甲状腺功能、血氨、乳酸、肌酸激酶、肝功能以及尿有机酸(UOAs)代谢筛查,所有这些检查均未发现异常。维生素D水平较低,为38 ng/ml(>50)。在标准条件下并给予诱发刺激进行的脑电图(EEG)检查结果异常,双侧中央区和额叶有放电,右侧活动更多,显示背景活动为中等程度有组织的阿尔法波(8 - 13 Hz)以及闪光刺激时加剧的尖波和慢波活动爆发。多导睡眠图显示睡眠效率较差,为84.7%,由于睡眠中断未进入快速眼动(REM)阶段。随后他被诊断为癫痫性脑病和Landau-Kleffner综合征(LKS)。给患者开了丙戊酸钠、静脉注射免疫球蛋白(IVIG)和冲击性类固醇药物,但没有明显改善,添加了利培酮但耐受性差,剂量逐渐减少并最终停用。开始服用哌甲酯,起始剂量为5 mg,剂量逐渐增加至20 mg/天,分两次服用,每次10 mg。一周后,添加了2 mg褪黑素。三个月后复查脑电图正常,丙戊酸钠逐渐减量并最终停用。同年晚些时候,他被诊断出感染了新冠病毒,并并发急性肌炎,哌甲酯停用,仅继续服用舍曲林和褪黑素。仅在此时再次进行MRI检查,显示有病毒/自身免疫性脑炎(AIE)后遗症的迹象,同时给予静脉注射皮质类固醇和IVIG。患者出院时服用泼尼松龙,一个月后,各方面均有显著改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b71f/10855006/ae3bb2114202/cureus-0016-00000052133-i01.jpg

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