Lorazepam for post-operative pediatric cerebellar mutism syndrome: A case report.

IF 1 Q4 PEDIATRICS
Son Nguyen, Denesh Ratnasingam, Kimberly C Hartman
{"title":"Lorazepam for post-operative pediatric cerebellar mutism syndrome: A case report.","authors":"Son Nguyen, Denesh Ratnasingam, Kimberly C Hartman","doi":"10.1177/18758894261461379","DOIUrl":null,"url":null,"abstract":"<p><p>Case DiagnosisAn 11-year-old male with medulloblastoma underwent gross total resection of a posterior fossa tumor, complicated by post-operative pediatric cerebellar mutism syndrome (ppCMS), exhibiting mutism, emotional dysregulation, impaired volitional movement initiation, and ataxia.Case DescriptionWhile ppCMS's pathophysiology remains incompletely understood, prevailing hypotheses implicate cerebellothalamocortical dysregulation, functional diaschisis, and inhibitory-excitatory network imbalance. This case explores the use of lorazepam, a benzodiazepine that enhances GABA-A receptor activity, as a potential neuromodulator to facilitate recovery in ppCMS.SettingTertiary care children's hospital.Assessments/ResultsPharmacologic trials with bromocriptine, risperidone, and gabapentin yielded minimal benefit. However, administration of intranasal midazolam (5 mg) during radiation simulation resulted in transient resolution of mutism and improved initiation of movement. Based on this response, lorazepam (2 mg daily, 0.06 mg/kg) was initiated, leading to sustained improvements in speech, motor function, and therapy participation. Functional outcome assessments, including the WeeFIM, revealed a 40-unit gain within one week of lorazepam initiation compared to a 1-unit gain in the preceding week. Immediate regression was noted upon medication discontinuation and reintroduction resulted in subsequent recovery.Discussion/RelevanceThe differential response to benzodiazepines compared to other medications suggests potential for inhibitory-excitatory network imbalances. Future research should explore whether less sedating benzodiazepines, such as clobazam, or other GABAergic modulators could provide similar benefits without the cognitive side effects associated with lorazepam.ConclusionsThere is compelling evidence that benzodiazepine-mediated GABAergic modulation may facilitate CMS recovery by restoring cerebellothalamocortical network function. The observed improvements in motor initiation and speech fluency underscore the potential for pharmacologic neuromodulation as an adjunct to traditional rehabilitative therapies.An 11-year-old male with medulloblastoma underwent gross total resection of a posterior fossa tumor, complicated by ppCMS, exhibiting mutism, emotional dysregulation, impaired volitional movement initiation, and ataxia. Amid ongoing neurorehabilitation, a pharmacologic trial with lorazepam led to a reproducible improvement in speech and engagement. Though transient, the response suggested reversible disruption within functional circuits rather than fixed structural damage. This case report uncovers latent functional capacity in ppCMS, which can help distinguish ppCMS from other overlapping neurologic or psychiatric conditions throughout the course of rehabilitation.</p>","PeriodicalId":16692,"journal":{"name":"Journal of pediatric rehabilitation medicine","volume":" ","pages":"18758894261461379"},"PeriodicalIF":1.0000,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13438969/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of pediatric rehabilitation medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1177/18758894261461379","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"PEDIATRICS","Score":null,"Total":0}
引用次数: 0

Abstract

Case DiagnosisAn 11-year-old male with medulloblastoma underwent gross total resection of a posterior fossa tumor, complicated by post-operative pediatric cerebellar mutism syndrome (ppCMS), exhibiting mutism, emotional dysregulation, impaired volitional movement initiation, and ataxia.Case DescriptionWhile ppCMS's pathophysiology remains incompletely understood, prevailing hypotheses implicate cerebellothalamocortical dysregulation, functional diaschisis, and inhibitory-excitatory network imbalance. This case explores the use of lorazepam, a benzodiazepine that enhances GABA-A receptor activity, as a potential neuromodulator to facilitate recovery in ppCMS.SettingTertiary care children's hospital.Assessments/ResultsPharmacologic trials with bromocriptine, risperidone, and gabapentin yielded minimal benefit. However, administration of intranasal midazolam (5 mg) during radiation simulation resulted in transient resolution of mutism and improved initiation of movement. Based on this response, lorazepam (2 mg daily, 0.06 mg/kg) was initiated, leading to sustained improvements in speech, motor function, and therapy participation. Functional outcome assessments, including the WeeFIM, revealed a 40-unit gain within one week of lorazepam initiation compared to a 1-unit gain in the preceding week. Immediate regression was noted upon medication discontinuation and reintroduction resulted in subsequent recovery.Discussion/RelevanceThe differential response to benzodiazepines compared to other medications suggests potential for inhibitory-excitatory network imbalances. Future research should explore whether less sedating benzodiazepines, such as clobazam, or other GABAergic modulators could provide similar benefits without the cognitive side effects associated with lorazepam.ConclusionsThere is compelling evidence that benzodiazepine-mediated GABAergic modulation may facilitate CMS recovery by restoring cerebellothalamocortical network function. The observed improvements in motor initiation and speech fluency underscore the potential for pharmacologic neuromodulation as an adjunct to traditional rehabilitative therapies.An 11-year-old male with medulloblastoma underwent gross total resection of a posterior fossa tumor, complicated by ppCMS, exhibiting mutism, emotional dysregulation, impaired volitional movement initiation, and ataxia. Amid ongoing neurorehabilitation, a pharmacologic trial with lorazepam led to a reproducible improvement in speech and engagement. Though transient, the response suggested reversible disruption within functional circuits rather than fixed structural damage. This case report uncovers latent functional capacity in ppCMS, which can help distinguish ppCMS from other overlapping neurologic or psychiatric conditions throughout the course of rehabilitation.

劳拉西泮治疗小儿术后小脑性缄默综合征1例。
病例诊断:一例11岁男性髓母细胞瘤患者行后颅窝肿瘤全切除术,术后并发小儿小脑缄默症(ppCMS),表现为缄默症、情绪失调、意志运动启动障碍和共济失调。虽然ppCMS的病理生理机制尚不完全清楚,但普遍的假设涉及小脑丘脑皮质失调、功能失稳和抑制性-兴奋性网络失衡。本病例探讨了劳拉西泮(一种增强GABA-A受体活性的苯二氮卓类药物)作为促进ppCMS恢复的潜在神经调节剂的使用。三级保健儿童医院。评估/结果溴隐亭、利培酮和加巴喷丁的药理学试验显示,疗效微乎其微。然而,在辐射模拟期间鼻内给予咪达唑仑(5mg)可导致失聪的短暂消退和运动启动的改善。基于这种反应,开始使用劳拉西泮(每天2 mg, 0.06 mg/kg),导致言语、运动功能和治疗参与的持续改善。功能结果评估,包括WeeFIM,显示劳拉西泮起始一周内增加40个单位,而前一周增加1个单位。停药后立即恢复,重新用药后恢复。与其他药物相比,苯二氮卓类药物的不同反应表明可能存在抑制-兴奋网络失衡。未来的研究应该探索是否镇静作用较弱的苯二氮卓类药物,如氯巴唑,或其他gaba能调节剂可以提供类似的益处,而没有与劳拉西泮相关的认知副作用。结论苯二氮卓类药物介导的gaba能调节可能通过恢复小脑丘脑皮质网络功能促进CMS的恢复。观察到的运动启动和语言流畅性的改善强调了药理学神经调节作为传统康复治疗的辅助疗法的潜力。一例11岁男性髓母细胞瘤患者行后窝肿瘤全切除术,并发ppCMS,表现为失语、情绪失调、意志运动启动受损和共济失调。在正在进行的神经康复中,劳拉西泮的药理学试验导致言语和参与的可重复改善。虽然是短暂的,但反应表明功能电路的可逆破坏,而不是固定的结构损伤。本病例报告揭示了ppCMS的潜在功能能力,这有助于在整个康复过程中将ppCMS与其他重叠的神经或精神疾病区分开来。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
CiteScore
2.30
自引率
5.30%
发文量
139
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信
小红书