Annals of the Child Neurology Society最新文献

{"title":"Isolated peripheral neuroleukemiosis mimicking Guillain-Barré syndrome in an adolescent with relapsed B-lymphoblastic leukemia","authors":"Ethan Edmondson,&nbsp;Paisley Pauli,&nbsp;Jyotinder Punia,&nbsp;Daniel G. Calame","doi":"10.1002/cns3.20095","DOIUrl":"https://doi.org/10.1002/cns3.20095","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Neuroleukemiosis, leukemic infiltration of the peripheral nervous system (PNS), is rare. Very few cases of isolated neuroleukemiosis, PNS leukemic infiltration without leukemia blasts in the blood or bone marrow, have been reported in pediatrics. Most cases have occurred in adults with acute myelogenous leukemia (AML) in remission who presented with peripheral neuropathy. We describe a pediatric patient presenting with isolated neuroleukemiosis mimicking Guillain-Barré syndrome.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Patient description</h3>\u0000 \u0000 <p>A 15-year-old boy presented 1 month after IVIG treatment for Guillain-Barre syndrome with worsening ataxia and weakness. He had a past medical history of B-cell acute lymphoblastic leukemia (B-ALL) in remission for 8 years. Examination revealed distal greater than proximal weakness of the bilateral lower extremities, areflexia at the Achilles tendon, and 1+ patellar and upper extremity reflexes. Initial magnetic resonance imaging (MRI) of the brain and spine were normal, and lumbar puncture revealed increased protein with uninterpretable white count due to excessive red blood cells. Repeat MRI brain and spinal cord showed extensive enlargement of all nerve roots and enhancement of the cauda equina and portions of cranial nerve VII bilaterally. Repeat lumbar puncture with cytology revealed leukemic blast cells. Blasts were not detected in peripheral blood smear or by flow cytometry. Bone marrow biopsy was also free of blast cells, confirming the diagnosis of relapsed B-ALL restricted to the nervous system.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Neuroleukemiosis is a rare entity with typical clinical features of mono- or polyneuropathy. It most often occurs in adults in remission from AML. However, neuroleukemiosis should also be on the differential for pediatric patients in remission from ALL presenting with neuropathy.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"2 4","pages":"315-318"},"PeriodicalIF":0.0,"publicationDate":"2024-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.20095","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142868023","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Altered cerebral oxygen extraction and metabolism in preterm neonates and the relationship to anemia: A noncontrast MRI study","authors":"Zixuan Lin,&nbsp;Dan Wu,&nbsp;Dengrong Jiang,&nbsp;Hanzhang Lu,&nbsp;Ying Qi","doi":"10.1002/cns3.20081","DOIUrl":"https://doi.org/10.1002/cns3.20081","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>The preterm brain is susceptible to structural injuries, which may be related to an imbalance between blood supply and oxygen metabolism. However, the effect of preterm birth on cerebral oxygen metabolism and its underlying mechanism have not been fully elucidated. The present study measured cerebral oxygen extraction and metabolism using noncontrast magnetic resonance imaging (MRI) methods in preterm neonates and examined its relationship with anemia of prematurity.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Fifty neonates with a gestational age of 28–42 weeks were enrolled. Cerebral oxygen extraction fraction (OEF) and cerebral metabolic rate of oxygen (CMRO₂) were measured with T₂-relaxation-under-spin-tagging (TRUST) MRI, together with cerebral blood flow (CBF).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We showed that CBF (<i>p</i> = 0.00021) and CMRO₂ (<i>p</i> &lt; 0.0001) increased with gestational age while OEF increased with postnatal age (<i>p</i> = 0.0013). Higher OEF was also associated with a higher Apgar score at birth (<i>p</i> = 0.039). Furthermore, hematocrit significantly mediates the increase of OEF with postnatal age (<i>p</i> &lt; 0.001). Structural equation modeling analysis suggested a bidirectional relationship between CBF and CMRO₂; both contributed to the changes in OEF.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>These findings demonstrated an altered cerebral oxygen metabolism in preterm brain, suggesting a potential role of MRI–based oxygenation measurement in the assessment of transfusion and intervention for preterm neonates.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"2 4","pages":"269-280"},"PeriodicalIF":0.0,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.20081","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142869180","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acute onset of unilateral movements?","authors":"Devan J. Peterson,&nbsp;Olivia S. Yale,&nbsp;Janetta L. Arellano","doi":"10.1002/cns3.20089","DOIUrl":"https://doi.org/10.1002/cns3.20089","url":null,"abstract":"<p>This previously healthy neurotypical 3-year-old boy presented for frequent stereotyped episodes of left facial and arm twitching with maintained awareness. An electroencephalogram (EEG) and brain magnetic resonance imaging were normal. However, due to a high concern for focal motor seizures, he was discharged on daily levetiracetam. Six days later, he was readmitted for increased irritability and continued left hemibody movements while awake and asleep. Continuous EEG captured the episodes of concern without an EEG correlate. The evaluation for toxic, metabolic, and infectious conditions, including cerebrospinal fluid studies, was unremarkable. His agitation was concerning for an adverse effect of levetiracetam, so he was switched to lacosamide. The evaluation was broadened to include autoimmune disorders.</p><p>His agitation did not improve, and then he developed low-grade fevers with continued irritability. His left hemibody movements evolved to orofacial dyskinesia and choreoathetoid movements (Video S1). He was started on intravenous immunoglobulins and methylprednisolone for probable autoimmune etiology. He was later found to have antibodies to the NMDA receptor in the cerebrospinal fluid (titer 1:5), confirming the diagnosis of anti-<i>N</i>-methyl-<span>d</span>-aspartate (NMDA)-receptor encephalitis, consistent with his behavioral change and movement disorder.</p><p>This boy illustrates the clinical presentation of anti-NMDA encephalitis in a child presenting with stereotypical hemibody movements unresponsive to antiseizure medication. His findings highlight the importance of a comprehensive evaluation and early treatment of potential autoimmune causes of acute behavioral and motor changes without signs of infection or previous seizures.</p><p><b>Devan J. Peterson</b>: Conceptualization; investigation; supervision; visualization; writing— original draft; writing—review and editing. <b>Olivia S. Yale</b>: Conceptualization; investigation; writing—original draft; writing—review and editing. <b>Janetta L. Arellano</b>: Conceptualization; investigation; supervision; writing—original draft; writing—review and editing.</p><p>The authors declare no conflicts of interest.</p>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"2 4","pages":"319"},"PeriodicalIF":0.0,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.20089","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142869181","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Complex epilepsy phenotype associated with chromosome 2q24.2-q24.3 deletion involving sodium channel gene cluster","authors":"Rima Madan,&nbsp;Fiorella S. Guido,&nbsp;Nicole Brescia","doi":"10.1002/cns3.20087","DOIUrl":"https://doi.org/10.1002/cns3.20087","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>The 2q24.2-24.3 chromosome region encodes sodium channel genes important in severe childhood epilepsy, notably <i>SCN1A</i> linked to Dravet syndrome (DS). However, the roles of other genes, either within the SCN cluster or in the segments proximal to it, have not been clearly delineated. The combination of ketogenic diet and fenfluramine is known to provide substantial benefits to patients with DS, but there is a paucity of literature regarding its role in other developmental and epileptic encephalopathies (DEE). This report aims to further explore clinical findings and treatment outcomes in a patient with a complex epilepsy phenotype.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Our patient's extensive diagnostic evaluation revealed 14 deleted genes within the 2q24.2-24.3 chromosome region.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The patient initially presented with clusters of focal motor seizures with apnea and cyanosis requiring intubation as well as prolonged hospitalizations for status epilepticus. He has baseline hypotonia, dysphagia, and developmental delay. He had a &gt;50% reduction in his seizures following a combination of ketogenic diet and fenfluramine. His seizures are now responsive to rescue midazolam, and he no longer has status epilepticus.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Our patient's remarkable clinical improvement suggests that this dual therapy may be beneficial in patients with DEE exhibiting pathogenic variations in this region of chromosome 2, beyond just DS.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"2 4","pages":"295-298"},"PeriodicalIF":0.0,"publicationDate":"2024-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.20087","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142869162","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Witnessing abusive head trauma: Accidents show higher rates of intracranial pathologies than shaking","authors":"Chris Brook","doi":"10.1002/cns3.20084","DOIUrl":"https://doi.org/10.1002/cns3.20084","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>This study aims to determine whether intracranial injuries, such as seizures, encephalopathy, bilateral subdural hematoma (SDH), and severe bilateral retinal hemorrhage (RH), are indicators of abusive head trauma (AHT), particularly in cases involving shaking.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Data comprising 54 witnessed shaking cases were drawn from two studies in the literature. Data of 100 witnessed accidents comes from the pediBIRN collaboration. Rates of intracranial injuries in cases of unconflicted witnessed accidents are compared to rates in cases of witnessed shaking and also to cases of unconflicted witnessed shaking. Unconflicted is defined as observed by an independent, unbiased witness, or by a potentially biased witness (such as partner) if reported prior to medical examinations.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>When all witnessed shaking cases were considered, including potentially biased witnesses, there are higher rates of findings commonly associated with AHT in witnessed accidents than in cases of witnessed shaking, although the difference is only statistically significant for seizures and encephalopathy. When restricted to cases when the witness was unconflicted, the rates of all findings are significantly more common in accidents than in shaking.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Accidents result in more severe intracranial pathologies than shaking, aligning with biomechanical studies that have shown that impact exerts greater force on the brain than violent shaking.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"2 3","pages":"206-211"},"PeriodicalIF":0.0,"publicationDate":"2024-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.20084","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142320574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Rett syndrome: The Natural History Study journey","authors":"Alan K. Percy,&nbsp;Timothy A. Benke,&nbsp;Eric D. Marsh,&nbsp;Jeffrey L. Neul","doi":"10.1002/cns3.20086","DOIUrl":"https://doi.org/10.1002/cns3.20086","url":null,"abstract":"<p>Understanding clinical features and disease progression of Rett syndrome (RTT) and establishing clinical trial readiness was enhanced by the RTT Natural History Study (NHS). The NHS benefited from two key developments: one, the Orphan Drug Act passed by Congress in 1983 defining criteria for rare disorders in the United States and creating opportunities for pharmaceutical companies to develop products for individuals with rare disorders, and two, the Rare Diseases Act of 2002, which established the National Institutes of Health Office of Rare Diseases and provided research funding. Funding for the RTT and related disorders NHS was obtained in 2003, creating a broad network of experienced clinical investigators across the United States and producing critical results not only for RTT but also for related disorders: <i>CDKL5</i> deficiency disorder, <i>FOXG1</i> disorder, and <i>MECP2</i> duplication syndrome. Longitudinal information from over 1800 participants (more than 1600 diagnosed with RTT) led to multiple reports describing their clinical features and natural progression and identified putative biomarkers and clinical outcome measures. Establishing clinical trial readiness assisted in evaluating the first FDA-approved medication for RTT in 2023 and continues to provide opportunities to develop potentially life-altering therapies. The experiences of the RTT NHS journey provide informative guidance for studying other rare neurological disorders. These lessons include positive features of developing productive collaborations focused on improving lives of people and families with RTT and related disorders, as well as lessons learned through retrospective analysis for improving overall conduct of natural history studies in rare disorders.</p>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"2 3","pages":"189-205"},"PeriodicalIF":0.0,"publicationDate":"2024-08-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.20086","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142320630","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hereditary sensory autonomic neuropathy type VI in the age of genetic testing","authors":"Lekshmi Peringassery Sateesh,&nbsp;Pavani Chitamanni,&nbsp;Danielle Akinsanmi,&nbsp;Suman Ghosh,&nbsp;Steven G. Pavlakis,&nbsp;Alexandra Reznikov","doi":"10.1002/cns3.20085","DOIUrl":"https://doi.org/10.1002/cns3.20085","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Hereditary sensory and autonomic neuropathy type VI (HSAN VI) is a rare recessive genetic disorder caused by mutations in the human dystonin (<i>DST</i>) gene. We report a novel homozygous alternate transcript mutation in the <i>DST</i> gene causing a severe neonatal form of HSAN VI.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Patient Description</h3>\u0000 \u0000 <p>This baby boy was born with severe hypotonia, respiratory distress, dysmorphic features, and bilateral club feet. Imaging, karyotyping, Prader–Willi assay, spinal muscular atrophy genetic panel and myotonic dystrophy genetic panel were all negative. A comprehensive neuropathy panel detected a homozygous pathogenic variant in the <i>DST</i> gene—alternate transcript NM_015546.4:c.1357G&gt;A (p.Trp4525*). Nerve conduction studies revealed mixed axonal and demyelinating sensorimotor neuropathy, suggesting the possibility of motor involvement in severe forms of this rare condition. The infant ultimately developed sepsis and died from cardiorespiratory arrest. Neuropathological findings of focal and mild spinal nerve axonal degeneration were nonspecific.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>Collective analysis of these patients would help to further characterize the spectrum of disease pathology and could provide insight into the neurophysiology and neuropathology of this rare condition.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"2 4","pages":"290-294"},"PeriodicalIF":0.0,"publicationDate":"2024-08-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.20085","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142868384","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Ictal shushing behavior in a child with a pilocytic astrocytoma","authors":"Liliana Ladner,&nbsp;Mebratu Daba","doi":"10.1002/cns3.20083","DOIUrl":"https://doi.org/10.1002/cns3.20083","url":null,"abstract":"<p>Pilocytic astrocytomas (PA) are slow-growing gliomas that account for 15% of all central nervous system tumors.<span>¹</span> Focal seizures are well-reported sequelae of PAs but present heterogeneously.<span>²</span> We describe a unique ictal “shushing” behavior in a pediatric patient with a PA of the isthmus of the cingulate gyrus.</p><p>This 14-year-old right-handed boy with no significant past medical history presented due to concern for seizures. Three to four months prior, he began experiencing daily episodes of an unusual gesture. During these episodes, he would walk toward his family or friends, place his pointer finger on his lips, and tell them to “shush” without any other vocalizations. He was aware of these episodes as they occurred but incapable of stopping them. Each episode was preceded by abdominal pain, nausea, and dizziness and followed by headaches and sleeping. Vomiting occurred after several episodes. During one episode that occurred while sleeping, his eyes rolled back for one minute, and then he awoke and promptly returned to sleep, but there were no additional seizure manifestations.</p><p>A subsequent 2-hour electroencephalogram (EEG) and continuous EEG monitoring were normal. Due to high clinical suspicion of focal seizures with impaired awareness, an MRI of the brain was ordered, and he was prescribed levetiracetam. The MRI demonstrated a lesion within the isthmus of the right cingulate gyrus that was cystic and contained a heterogeneously enhancing mural nodule (Figure 1).</p><p>Two days later, the patient underwent surgical resection of the lesion, a PA with eosinophilic granular bodies and Rosenthal fibers on histology. After the operation, he was alert but exhibited left-sided hemineglect and homonymous hemianopsia. Seven months after surgery, he stopped taking his levetiracetam, and the “shushing” behavior did not recur and there were no other seizure manifestations. Subsequent imaging demonstrated no tumor recurrence.</p><p>This patient exhibited an unusual ictal “shushing” behavior due to tumor-related epilepsy. Ictal shushing as a manifestation of a focal seizure has not been previously described in a pediatric patient. For pediatric patients with similar behaviors, both focal seizures and PAs should be on the differential.</p><p>The location of this patient's lesion in the isthmus of the right cingulate gyrus and its proximity to the temporal lobe may have contributed to his semiology. In patients with mesial temporal sclerosis, seizures arise in the hippocampus and propagate to the cortex.<span>³</span> Specifically, impulses from the temporal lobe may propagate through the supplementary motor area to produce index finger pointing in localization-related epilepsy.<span>⁴</span> Although similar ictal shushing has been reported as a “hush sign” in two adult patients, this report highlights the first presentation in a pediatric patient with a focal lesion.<span><su","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"2 3","pages":"248-250"},"PeriodicalIF":0.0,"publicationDate":"2024-07-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.20083","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142320626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Opioid neurotoxicity: A case series and review from members of the Child Neurology Society Neurocritical Care Special Interest Group","authors":"Varina L. Boerwinkle,&nbsp;Imani H. Sweatt,&nbsp;Aniela Grzezulkowska,&nbsp;William R. Reuther,&nbsp;Aaron Gelinne,&nbsp;Emilio G. Cediel,&nbsp;Divakar S. Mithal,&nbsp;Carolyn S. Quinsey,&nbsp;Scott W. Elton","doi":"10.1002/cns3.20077","DOIUrl":"10.1002/cns3.20077","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>The Child Neurology Society 2023 Annual Meeting Neurocritical Care Special Interest Group discussed pediatric opioid use–associated neurotoxicity with cerebellar edema (POUNCE). Inspired by the discussion and the suspicion of an underrecognized severe form of the disorder, we provide a case series and literature review on this important and emerging topic.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>The meeting was moderated by coauthor DSM, with formal presentation by coauthor AG, and supplemented with a supporting case by coauthor VLB. The attendees, by show of hand, were queried for experience with direct care of children in the critical care unit with neurotoxicity from opioid exposure. These meeting elements informed our literature review and case series.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>A key focus of the meeting was the importance of interdisciplinary communication regarding POUNCE, emphasizing the necessity for neurosurgical assessment due to mass effect. Approximately 10 of 40 attendees, representing different US hospitals, reported caring for children with opioid neurotoxicity and concern for increased intracranial pressure. Described during the meeting was a 2-year-old girl with opioid exposure, rapidly worsening neurological exam, and transforaminal herniation concerning for severe POUNCE syndrome and impact on brain networks by resting-state functional magnetic resonanance imaging (rs-MRI). After surgical decompression did not improve her neurological function, she underwent rs-MRI, electroencephalogram, and MRI. The networks indicated better neurological function than the exam, consistent with outcome. In contrast, the second patient, was an 11-month-old boy with fentanyl exposure who was treated for opioid overdose and closely monitored clinically. He did not require surgical intervention and has recovered well.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>These patients add to the few publications documenting the management of POUNCE, which may require urgent posterior cranial fossa decompression, and highlight the potential for good outcomes. Additionally, this is the first report documenting rs-fMRI for this condition, which was consistent with the patient's outcome.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"2 3","pages":"225-234"},"PeriodicalIF":0.0,"publicationDate":"2024-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.20077","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141657849","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Saccade and pupil changes in children recovering from opsoclonus-myoclonus ataxia syndrome reveal midbrain alterations in oculomotor circuits","authors":"Douglas P. Munoz,&nbsp;Brian J. White,&nbsp;Donald C. Brien,&nbsp;Kajaal Parbhoo,&nbsp;Carmen Yea,&nbsp;E. Ann Yeh","doi":"10.1002/cns3.20078","DOIUrl":"10.1002/cns3.20078","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>This study measured eye movements in children with a history of opsoclonus-myoclonus ataxia syndrome in order to identify abnormalities in saccade and pupil behavior that map onto specific alterations in brainstem pathways.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We used video-based eye tracking while participants freely viewed 10 min of short (2–4 s) video clips without instructions. Clip transitions represented a large visual perturbation and we quantified multiple characteristics of saccade and pupil responses following these transitions in 13 children recovering from opsoclonus-myoclonus and 13 healthy, age-matched control participants.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>The frequency of saccades and distribution of fixation durations differed between the groups. Following the clip transitions, children recovering from opsoclonus-myoclonus ataxia syndrome exhibited longer time to initiate saccades, leading to a delay in harvesting visual information. Clip transitions to lighter clips produced similar pupil constriction responses in the two groups. However, clip transitions to darker clips produced dilation responses that were initiated earlier and of greater magnitude in opsoclonus-myoclonus ataxia syndrome, suggesting removal or suppression of a signal that delays dilation.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>Children with a history of opsoclonus-myoclonus ataxia syndrome demonstrated key abnormalities in saccade and pupil metrics. We propose a novel hypothesis in which dysfunction in the pathway from the superior colliculus to the mesencephalic and pontine reticular formation that houses the saccade and pupil premotor circuits could produce these results.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"2 3","pages":"212-224"},"PeriodicalIF":0.0,"publicationDate":"2024-07-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.20078","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141671789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}