Brain & Development最新文献

{"title":"Pediatric anti-neutral glycosphingolipid antibodies-positive encephalomyeloradiculoneuropathy presenting with prominent brain demyelination","authors":"Satoru Ochiai ,&nbsp;Itaru Hayakawa ,&nbsp;Tatsuro Mutoh ,&nbsp;Yuichi Abe","doi":"10.1016/j.braindev.2023.07.002","DOIUrl":"10.1016/j.braindev.2023.07.002","url":null,"abstract":"<div><h3>Background</h3><p><span>Encephalomyeloradiculoneuropathy (EMRN) is characterized by progressive neurological symptoms<span> in the central and peripheral nervous systems. The autoantibodies against neutral sphingolipids are disease-specific antibodies against EMRN. Although adults with EMRN typically present with symptoms of </span></span>peripheral nervous system involvement, the symptoms in pediatric patients are not well understood.</p></div><div><h3>Case</h3><p><span><span><span>A 4-year-old boy was admitted to our hospital on the 10th day of fever due to poor oral intake and hyponatremia. The day after admission, he developed </span>seizures and impaired consciousness and was transferred to our hospital. When he arrived at our hospital, he experienced disturbances in consciousness, neck rigidity, and </span>opisthotonus<span>. MRI of the head revealed scattered white matter lesions<span><span> and pleocytosis<span> in the cerebrospinal fluid (CSF). During </span></span>treatment with intravenous </span></span></span>methylprednisolone<span><span> (IVMP), the patient developed diminished deep tendon reflexes<span><span><span> in the lower extremities four days later, with no improvement in cervical stiffness or opisthotonos. Additional evaluations revealed enlarged cerebral white matter lesions on brain MRI, cauda equina enhancement on MRI of the spinal cord, axonal </span>neuropathy in the bilateral </span>tibial nerves, and positive anti-neutral glycosphingolipid (GSL) antibodies in both serum and CSF. Intensive immunomodulatory therapy, and </span></span>neurorehabilitation, led to substantial neurological recovery within three months of onset.</span></p></div><div><h3>Conclusion</h3><p>Pediatric<span> antineutral GSL antibody-positive EMRN may initially present with extensive cerebral white matter lesions and delayed onset of peripheral radiculoneuropathy. Our case extends the disease spectrum of EMRN and may aid in the early diagnosis of EMRN in the pediatric population.</span></p></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10210917","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Neurochemistry evaluated by magnetic resonance spectroscopy in a patient with FBXO28-related developmental and epileptic encephalopathy","authors":"Kentaro Sano ,&nbsp;Fuyuki Miya ,&nbsp;Mitsuhiro Kato ,&nbsp;Taku Omata ,&nbsp;Jun-ichi Takanashi","doi":"10.1016/j.braindev.2023.07.003","DOIUrl":"10.1016/j.braindev.2023.07.003","url":null,"abstract":"<div><h3>Background</h3><p>Mutations in the <em>FBXO28</em> gene, which encodes FBXO28, one of the F-box protein family, may cause developmental and epileptic encephalopathy (DEE). <em>FBXO28</em><span>-related DEE is radiologically characterized by cerebral atrophy<span><span>, delayed/abnormal myelination, and </span>brain malformation; however, no neurochemical analyses have been reported.</span></span></p><p><span><span>Case report: A female Japanese infant presented with severe psychomotor delay, epileptic spasms, and </span>visual impairment. Whole-exome sequencing revealed a de novo variant of the </span><em>FBXO28</em> gene, leading to the diagnosis of <em>FBXO28</em>-related DEE. Magnetic resonance (MR) spectroscopy at 6, 12, and 32 months revealed decreased <em>N</em><span>-acetylaspartate and choline-containing compounds and increased levels of myoinositol.</span></p></div><div><h3>Conclusion</h3><p>MR spectroscopy revealed neurochemical derangement in <em>FBXO28</em><span>-related DEE, that is, disturbed myelination secondary to neuronal damage with astrogliosis.</span></p></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10316368","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Severe and rare neurological manifestations following COVID-19 infection in children: A Malaysian tertiary centre experience","authors":"Muhamad Azamin Anuar ,&nbsp;Jun Xiong Lee ,&nbsp;Husna Musa ,&nbsp;Dianah Abd Hadi ,&nbsp;Elyssa Majawit ,&nbsp;Poorani Anandakrishnan ,&nbsp;Sumitha Murugesu ,&nbsp;Ahmad Rithauddin Mohamed ,&nbsp;Teik Beng Khoo","doi":"10.1016/j.braindev.2023.06.004","DOIUrl":"10.1016/j.braindev.2023.06.004","url":null,"abstract":"<div><h3>Introduction</h3><p><span>Since the emergence of COVID-19, we have experienced potent variants and sub-variants of the virus with non-specific </span>neurological manifestations. We observed a surge of the Omicron variant of COVID-19 patients with neurological manifestations where less cases of multisystem inflammatory syndrome in children (MIS-C) were reported. This article describes our experience of children with severe and rare neurological manifestations following COVID-19 infection.</p></div><div><h3>Methods</h3><p><span>This is a retrospective observational case series of patients under 18 years old who fulfilled the WHO COVID-19 case definition and were referred to our paediatric neurology unit at Hospital Tunku Azizah Kuala Lumpur. Their demographic data, </span>neurological symptoms<span>, laboratory and supporting investigations, neuroimaging, treatment and outcomes were collected and analysed.</span></p></div><div><h3>Results</h3><p><span><span>There were eleven patients with neurological manifestations who fulfilled the WHO COVID-19 case definition. Nine patients presented with seizures and/or encephalopathy, one patient with eye </span>opsoclonus<span> and another patient with persistent limbs myokymia. Based on the history, clinical, electrophysiological and </span></span>radiological findings<span>, two of them had febrile infection-related epilepsy syndrome<span>, two had acute disseminated encephalomyelitis<span>, two had acute necrotising encephalopathy of childhood, one each had hemiconvulsion-hemiplegia-epilepsy syndrome, acute encephalopathy with bilateral striatal necrosis, hemi-acute encephalopathy with biphasic seizures and reduced diffusion, infection-associated opsoclonus and myokymia.</span></span></span></p></div><div><h3>Conclusions</h3><p>This case series highlighted a wide spectrum of neurological manifestations of COVID-19 infection. Early recognition and prompt investigations are important to provide appropriate interventions. It is essential that these investigations should take place in a timely fashion and COVID-19 quarantine period should not hinder the confirmation of various presenting clinical syndromes.</p></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10500251","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cover","authors":"","doi":"10.1016/S0387-7604(23)00166-3","DOIUrl":"https://doi.org/10.1016/S0387-7604(23)00166-3","url":null,"abstract":"","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71783271","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of the neurofilament light chain as a biomarker in children with spinal muscular atrophy treated with nusinersen","authors":"Gigyo Seo ,&nbsp;Saeyoon Kim ,&nbsp;Jun Chul Byun ,&nbsp;Soonhak Kwon ,&nbsp;Yun Jeong Lee","doi":"10.1016/j.braindev.2023.07.005","DOIUrl":"10.1016/j.braindev.2023.07.005","url":null,"abstract":"<div><h3>Background</h3><p><span>This study aimed to evaluate the neurofilament<span> light chain (NfL) as a biomarker for treatment responses in children with a broad spectrum of </span></span>spinal muscular atrophy<span> (SMA) under nusinersen treatment.</span></p></div><div><h3>Method</h3><p><span>We measured NfL levels in serum (sNfL) and cerebrospinal fluid (cNfL) in nusinersen-treated patients with SMA and children without </span>neurologic disorders. Correlations between cNfL and sNfL levels and motor function scores were analyzed.</p></div><div><h3>Results</h3><p>sNfL and cNfL levels were measured in eight patients with SMA (SMA type 1, n = 3; SMA type 2, n = 5). sNfL levels were strongly correlated with cNfL levels regardless of the SMA subtype (r = 0.97, P &lt; 0.001). Patients with SMA type 1 had higher baseline cNfL and sNfL levels before treatment initiation than those with SMA type 2 and neurologically healthy children. In patients with acute stage of SMA type 1 and 2, the NfL level rapidly decreased during the nusinersen treatment loading phase followed by stabilization at a lower plateau level. In contrast, in a patient with a chronic stage of SMA type 2, the NfL level remained within the normal range with no apparent downward trend. Motor function scores showed a tendency toward an inverse correlation with NfL levels in patients with acute stage although not in patients with chronic stage.</p></div><div><h3>Conclusions</h3><p>cNfL and sNfL levels can be promising biomarkers for monitoring treatment response in patients within their acute stage, particularly in SMA type 1, although not in patients with a chronic stage of SMA type 2.</p></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9930833","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A boy with a progressive neurologic decline harboring two coexisting mutations in KMT2D and VPS13D","authors":"Yu-Ming Chang ,&nbsp;Yu-Wen Pan ,&nbsp;Yen-Yin Chou ,&nbsp;Wen-Hao Yu ,&nbsp;Meng-Che Tsai","doi":"10.1016/j.braindev.2023.08.001","DOIUrl":"10.1016/j.braindev.2023.08.001","url":null,"abstract":"<div><h3>Introduction</h3><p><span>Kabuki syndrome (KS) and </span>spinocerebellar ataxia (SCA) are both rare conditions with neurodevelopmental abnormalities. Approaching a patient with complex phenotypes and differentiating the role of mutations may be beneficial but challenging in predicting the disease prognosis.</p></div><div><h3>Case presentation</h3><p><span><span><span>A boy presented with progressive ataxia, developmental regression<span>, and myoclonus since 4 years of age. Additional features included growth hormone deficiency<span><span>, excessive body hair, dysmorphic facies, </span>hypoparathyroidism, and bilateral sensorineural </span></span></span>hearing impairment. Brain magnetic resonance imaging depicted T2-weighted hyperintensities over bilateral </span>globus pallidus<span>, thalamus<span><span>, subcortical white matter, and brainstem. The results of </span>tandem mass spectrometry<span><span>, mitochondrial deletion, and mitochondrial DNA sequencing were inconclusive. Whole-exome sequencing (WES) on </span>genomic DNA obtained from peripheral blood cells revealed a known pathogenic variant at </span></span></span></span><em>KMT2D</em> gene (c.5993A &gt; G, p.Tyr1998Cys) related to KS and two compound heterozygous, likely pathogenic variants at <em>VPS13D</em><span> gene (c.908G &gt; A, p.Arg303Gln and c.8561T &gt; G, p.Leu2854Arg) related to autosomal recessive SCA type 4 (SCAR4).</span></p></div><div><h3>Discussion</h3><p><span>SCAR4 is mainly adult-onset, but a few pediatric cases have recently been reported with progressive gait instability and developmental delay. The </span><em>VPS13D</em> gene has been suggested to play a role in mitochondrial size, autophagy, and clearance, thus explaining the clinical and imaging phenotypes.</p></div><div><h3>Conclusion</h3><p>Our case showed a rare co-existence of KS and SCAR4, highlighting the utility of WES in atypical cases that a single-gene disease cannot fully explain.</p></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10031091","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The gaze characteristics in preterm children: The appropriate timing for an eye-tracking tool","authors":"Soichi Yamase ,&nbsp;Wakako Ishii ,&nbsp;Nobuhiko Nagano ,&nbsp;Aya Okahashi ,&nbsp;Kimiko Deguchi ,&nbsp;Emiko Momoki ,&nbsp;Ichiro Morioka","doi":"10.1016/j.braindev.2023.08.003","DOIUrl":"10.1016/j.braindev.2023.08.003","url":null,"abstract":"<div><h3>Background</h3><p>An objective screening tool for autism spectrum disorder (ASD), also known as an eye-tracking tool, assesses the patient’s abnormal gaze patterns and detects the risk of ASD. As this tool is generally used for children born at term, this study aimed to clarify the appropriate timing for using the tool for preterm children, factors that influence the timing, and evaluate their gaze characteristics using the Gazefinder®.</p></div><div><h3>Method</h3><p>In 90 preterm children, a total of 125 eye-tracking tasks were completed and analyzed in 3–6, 7–9, 10–12, 13–18, and 19–32 months of corrected age (CA). The Gazefinder® was used to compare the mean fixation time percentage (MFP) in each CA and evaluate the gaze patterns. Perinatal factors associated with low MFP were also analyzed.</p></div><div><h3>Results</h3><p>Only 50% of the children scored ≥70% MFP at 3–6 months of CA. The MFP increased significantly after 7 months of CA (<em>p</em><span> = 0.0003), reached 90% at 13–18 months, and 100% at 19–32 months of CA. Chronic lung disease (CLD) was a clinical factor associated with low MFP (</span><em>p</em> = 0.036). Preterm children gazed more at eyes but gazed at mouths when the mouth moved.</p></div><div><h3>Conclusion</h3><p>It is necessary for preterm children to begin using Gazefinder® atleast at ≥13 months of age, especially those complicated with CLD. Preterm children prefer gazing at social information just as typically developing children.</p></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10124471","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Use of lacosamide for focal epilepsy in a child with kidney failure undergoing peritoneal dialysis","authors":"Yuki Ueda ,&nbsp;Ayako Furugen ,&nbsp;Masaki Kobayashi ,&nbsp;Yasuyuki Sato ,&nbsp;Yasuhiro Ueda ,&nbsp;Asako Hayashi ,&nbsp;Takeru Goto ,&nbsp;Shuhei Kimura ,&nbsp;Masashi Narugami ,&nbsp;Sachiko Nakakubo ,&nbsp;Midori Nakajima ,&nbsp;Kiyoshi Egawa ,&nbsp;Takayuki Okamoto ,&nbsp;Atsushi Manabe ,&nbsp;Hideaki Shiraishi","doi":"10.1016/j.braindev.2023.10.003","DOIUrl":"10.1016/j.braindev.2023.10.003","url":null,"abstract":"<div><h3>Background</h3><p><span><span>Lacosamide (LCM) has become commonly used for focal onset </span>seizures due to its high tolerability and low </span>drug<span><span><span> interactions. Unlike patients on hemodialysis (HD), </span>pharmacokinetic data and dosing recommendations for patients undergoing </span>peritoneal dialysis (PD) are scant.</span></p></div><div><h3>Case report</h3><p><span><span>A 2-year-old girl with end-stage kidney disease undergoing PD suffered prolonged focal onset seizures. The patient had congenital anomalies of the kidney and </span>urinary tract associated with branchio-oto-renal syndrome due to an </span><em>EYA1</em><span><span><span> gene mutation<span>. She also had neurological sequelae<span> from post-resuscitation encephalopathy at the age of one month. Antiseizure medication with few drug interactions, less impact on the neurodevelopmental state and possibility of </span></span></span>intravenous administration<span> was preferred. LCM met those criteria and was carefully administered. Although the patient had recurrent prolonged seizures during the titration periods, LCM could be continued without any apparent side effects. The blood levels of LCM increased linearly to the optimal level. We confirmed excretion of LCM in the </span></span>PD fluid<span>. Kidney transplantation was done three months after and her seizures were well controlled.</span></span></p></div><div><h3>Conclusions</h3><p>LCM might be a promising option for patients undergoing PD. Due to the lower removal efficacy in PD compared with in HD, close attention should be paid to possible drug excess.</p></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2023-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71429535","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnostic odyssey of Guillain-Barré syndrome in children","authors":"Yoko Kobayashi Takahashi ,&nbsp;Itaru Hayakawa ,&nbsp;Yuichi Abe","doi":"10.1016/j.braindev.2023.10.004","DOIUrl":"10.1016/j.braindev.2023.10.004","url":null,"abstract":"<div><h3>Background and Objectives</h3><p>A gap exists between difficulty in diagnosis and importance of early recognition and intervention in pediatric Guillain-Barré syndrome (GBS). Therefore, this study aimed to establish a diagnostic odyssey plot that allows “at-a-glance” overview of the diagnostic odyssey of GBS in children, including overall diagnostic delay, physician-related and patient-related diagnostic delays, and length and frequency of diagnostic errors.</p></div><div><h3>Methods</h3><p>In this single-center retrospective cohort study, standardized data were obtained from children with GBS from 2003 to 2020. Overall diagnostic delay (time between symptom onset and diagnosis), physician-related diagnostic delay (time between the first medical visit and diagnosis), and patient-related diagnostic delay (time between symptom onset and the first medical visit) were analyzed.</p></div><div><h3>Results</h3><p>The study examined a total of 21 patients (11 men, median age 4.5 years). Overall, there were 40 misdiagnoses among 17 patients, while four were diagnosed correctly at the first visit. The overall diagnostic delay was 9 days [interquartile range (IQR), 6–17 days]. Physician-related diagnostic delay, but not patient-related diagnostic delay, was correlated with the overall diagnostic delay. Patients in the late-diagnosed group were more frequently misdiagnosed during their diagnostic odyssey than patients in the other groups. Risk factors associated with diagnostic delay included delayed onset of weakness and sensory deficits, absence of swallowing problems, and misdiagnosis as orthopedic disorders or viral infections.</p></div><div><h3>Discussion</h3><p>A unique diagnostic odyssey exists in pedaitric GBS. Several clinical risk factors were associated with the diagnostic delay.</p></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2023-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71429534","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A nationwide survey of adenovirus-associated encephalitis/encephalopathy in Japan","authors":"Mika Nakazawa ,&nbsp;Shinpei Abe ,&nbsp;Mitsuru Ikeno ,&nbsp;Taiki Shima ,&nbsp;Toshiaki Shimizu ,&nbsp;Akihisa Okumura","doi":"10.1016/j.braindev.2023.10.002","DOIUrl":"10.1016/j.braindev.2023.10.002","url":null,"abstract":"<div><h3>Background</h3><p>Adenovirus is a major pathogen causing febrile illness among children. It may also cause acute encephalitis/encephalopathy. This study aimed to elucidate the clinical features of adenovirus-associated encephalitis/encephalopathy (AdVE) among children in Japan.</p></div><div><h3>Methods</h3><p>A nationwide survey of children with AdVE was conducted. An initial survey was distributed among pediatricians to obtain information about children with AdVE treated between January 2014 and March 2019. A second survey was used to obtain the clinical information of children with AdVE from hospitals that responded to the initial survey and those identified from a literature search of the reported cases. We collected demographic data and information about symptoms of infection, neurological symptoms, laboratory parameters, treatment, and outcomes. Outcomes were determined using the Pediatric Cerebral Performance Category Score.</p></div><div><h3>Results</h3><p>Clinical information was available for 23 children with a median age of 39 months. Two had preexisting neurological disorders and six had a history of febrile seizures. The outcome was good in 15 patients and poor in eight patients. Serum lactate dehydrogenase, glucose, and ammonia levels were higher among children with a poor outcome compared to those with a good outcome. Clinically mild encephalitis/encephalopathy with a reversible splenial lesion was the most common type (n = 8), followed by acute encephalopathy with biphasic seizures and late reduced diffusion (n = 7).</p></div><div><h3>Conclusion</h3><p>A prior history of febrile seizures was frequent in children with AdVE. Several different subtypes of acute encephalopathy were seen in children with AdVE, and the outcome was poor in those with acute encephalopathy with biphasic seizures and late reduced diffusion and hemorrhagic shock and encephalopathy syndrome. Elevated lactate dehydrogenase, glucose, and ammonia levels on admission were found to correlate with a poor outcome.</p></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":null,"pages":null},"PeriodicalIF":1.7,"publicationDate":"2023-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S0387760423001559/pdfft?md5=910b45893b7d0049f00d267ed5253cb1&pid=1-s2.0-S0387760423001559-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"54232440","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}