Photosensitive Posthypoxic Reflex Myoclonus in a Postcardiac Arrest Individual: An Interesting Finding.
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ABSTRACT:
SUBMITTER: Mahale RR
PROVIDER: S-EPMC5488589 | biostudies-literature | 2017 Jul-Sep
REPOSITORIES: biostudies-literature
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Photosensitive Posthypoxic Reflex Myoclonus in a Postcardiac Arrest Individual: An Interesting Finding.
Mahale Rohan R RR Mehta Anish A Srinivasa R R
Journal of neurosciences in rural practice 20170701 3
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Project description:Chronic posthypoxic action myoclonus is usually recognized as multifocal or generalized and, in most cases, causes severe disability. It is also commonly associated with other neurological symptoms, such as ataxia. We report two cases of focal predominant postanoxic action myoclonus and review the limited relevant literature. The first case presented with action and stimulus sensitive myoclonus in the lower limbs that caused him limited disability. He had not received any diagnosis in almost 2 years. The second patient exhibited predominantly focal facial myoclonus, although she was more limited by dysphasia and hemiplegia as a consequence of an ischemic stroke. Both patients responded to antiepileptic drugs. We found 9 of 159 previously reported cases with this focal distribution. Physicians should be aware of this limited phenotype given that the unusual focal nature of the movements may result in incorrect diagnosis and treatment.
| S-EPMC6353462 | biostudies-literature
Project description:ObjectivePosthypoxic myoclonus (PHM) in the first few days after resuscitation can be divided clinically into generalized and focal (uni- and multifocal) subtypes. The former is associated with a subcortical origin and poor prognosis in patients with postanoxic encephalopathy (PAE), and the latter with a cortical origin and better prognosis. However, use of PHM as prognosticator in PAE is hampered by the modest objectivity in its clinical assessment. Therefore, we aimed to obtain the anatomical origin of PHM with use of neurophysiological investigations, and relate these to its clinical presentation.MethodsThis study included 20 patients (56 ± 18 y/o, 68% M, 2 survived, 1 excluded) with EEG-EMG-video recording. Three neurologists classified PHM into generalized or focal PHM. Anatomical origin (cortical/subcortical) was assessed with basic and advanced neurophysiology (Jerk-Locked Back Averaging, coherence analysis).ResultsClinically assessed origin of PHM did not match the result obtained with neurophysiology: cortical PHM was more likely present in generalized than in focal PHM. In addition, some cases demonstrated co-occurrence of cortical and subcortical myoclonus. Patients that recovered from PAE had cortical myoclonus (1 generalized, 1 focal).InterpretationHypoxic damage to variable cortical and subcortical areas in the brain may lead to mixed and varying clinical manifestations of myoclonus that differ of those patients with myoclonus generally encountered in the outpatient clinic. The current clinical classification of PHM is not adequately refined to play a pivotal role in guiding treatment decisions to withdraw care. Our neurophysiological characterization of PHM provides specific parameters to be used in designing future comprehensive studies addressing the potential role of PHM as prognosticator in PAE.
| S-EPMC5899907 | biostudies-literature
Project description:End-of-life care and decisions on withdrawal of life-sustaining therapies vary across countries, which may affect the feasibility of future multicenter cardiac arrest trials. In Brazil, withdrawal of life-sustaining therapy is reportedly uncommon, allowing the natural history of postcardiac arrest hypoxic-ischemic brain injury to present itself. We aimed to characterize approaches to neuroprognostication of cardiac arrest survivors among physicians in Brazil.DesignCross-sectional study.SettingBetween August 2, 2019, and July 31, 2020, we distributed a web-based survey to physicians practicing in Brazil.SubjectsPhysicians practicing in Brazil and members of the Brazilian Association of Neurointensive Care, who care for patients resuscitated following cardiac arrest.InterventionsNot applicable.Measurements and main resultsResponses from 185 physicians were obtained. Pupillary reflexes, corneal reflexes, and motor responses were considered critical to prognostication, whereas neuroimaging and electroencephalography were also regarded as important. For patients without targeted temperature management, absent pupillary and corneal reflexes at 24 hours postarrest were considered strongly predictive of poor neurologic outcome by 31.8% and 33.0%, respectively. For targeted temperature management-treated patients, absent pupillary and corneal reflexes at 24-hour postrewarming were considered prognostic by 22.9% and 20.0%, respectively. Physicians felt comfortable making definitive prognostic recommendations at day 6 postarrest or later (34.2%) for nontargeted temperature management-treated patients, and at day 6 postrewarming (20.4%) for targeted temperature management-treated patients. Over 90% believed that improving neuroprognostic accuracy would affect end-of-life decision-making.ConclusionsThere is significant variability in neuroprognostic approaches to postcardiac arrest patients and timing of prognostic studies among Brazilian physicians, with practices frequently deviating from current guidelines, underscoring a need for greater neuroprognostic accuracy. Nearly all physicians believed that improving neuroprognostication will impact end-of-life decision-making. Given the tendency to delay prognostic recommendations while using similar neuroprognostic tools, Brazil offers a unique cohort in which to examine the natural history of hypoxic-ischemic brain injury in future studies.
| S-EPMC7803669 | biostudies-literature
Project description:Pediatric cardiac arrests carry significant morbidity and mortality. With increasing rates of return of spontaneous circulation, it is vital to optimize recovery conditions to decrease morbidity.MethodsWe evaluated all patients who presented to a large quaternary pediatric intensive care unit with return of spontaneous circulation. We compared patient-specific postcardiac arrest care preimplementation and postimplementation of a standardized postcardiac arrest resuscitation pathway. We implemented evidence-based best practices using the Translating Research into Practice framework and Plan-Do-Study-Act cycles. Our primary aim was to increase the percent of postcardiac arrest care events meeting guideline targets for blood pressure and temperature within the first 12 hours by 50% within 18 months.ResultsEighty-one events occurred in the preintervention group (August 1, 2016-April 30, 2018) and 64 in the postintervention group (May 1, 2018-December 1, 2019). The percent of postcardiac arrest events meeting guideline targets for the entirety of their postarrest period improved from 10.9% for goal mean arterial blood pressure to 26.3%, P = 0.03, and increased from 23.4% for temperature to 71.9%, P < 0.0001.ConclusionsImplementing a postcardiac arrest standardized care plan improved adherence to evidence-based postcardiac arrest care metrics, specifically preventing hypotension and hyperthermia. Future multicenter research is needed to link guideline adherence to patient outcomes.
| S-EPMC7952102 | biostudies-literature
Project description:BackgroundThiamine administration has been shown to improve survival in a postcardiac arrest animal study. We aimed to evaluate the efficacy of thiamine in comatose out-of-hospital cardiac arrest (OHCA) patients following return of spontaneous circulation.MethodsA randomized, double-blinded, placebo-controlled study was conducted. Thirty-seven OHCA patients were randomly assigned to receive either thiamine 100 mg every 8 hours or a placebo. The primary outcome was 28-day all-cause mortality.ResultsOver the course of 2 years, 37 patients were randomized to either receive thiamine (n = 20) or a placebo (n = 17). The primary outcome was not different between the groups: 10/20 (50%) in the thiamine group vs. 8/17 (47.1%) in the placebo group (P=0.93 by the log-rank test). There were no significant differences in secondary outcomes between the groups (good neurological outcome, lactate level, and S100B level).ConclusionsIn this study, there were no significant differences in survival outcome. Further studies with a larger population are necessary to confirm these results.
| S-EPMC7298263 | biostudies-literature
Project description:Prognostication after cardiac arrest often depends primarily on neurological function, and characterizing the extent of neurological injury hinges on neurophysiological testing and clinical neurological examination. The presence of early posthypoxic myoclonus (PHM) following cardiac arrest had been invariably associated with poor outcome, but more recent studies have shown that those with early PHM may survive with good neurological function. Electroencephalographic patterns suggestive of severe brain injury may be more valuable than the presence of PHM itself in portending poor functional status, and phenotyping PHM may also be useful in delineating benign and malignant forms. Patients with early PHM should be evaluated similarly to others who suffer cardiac arrest by using a multimodal approach in determining prognosis until further studies are performed that better characterize early PHM subtypes and their outcomes.
| S-EPMC5716491 | biostudies-literature
Project description:ObjectiveWe tested the hypothesis that there are readily classifiable electroencephalographic (EEG) phenotypes of early postanoxic multifocal myoclonus (PAMM) that develop after cardiac arrest.MethodsWe studied a cohort of consecutive comatose patients treated after cardiac arrest from January 2012 to February 2015. For patients with clinically evident myoclonus before awakening, 2 expert physicians reviewed and classified all EEG recordings. Major categories included: Pattern 1, suppression-burst background with high-amplitude polyspikes in lockstep with myoclonic jerks; and Pattern 2, continuous background with narrow, vertex spike-wave discharges in lockstep with myoclonic jerks. Other patterns were subcortical myoclonus and unclassifiable. We compared population characteristics and outcomes across these EEG subtypes.ResultsOverall, 401 patients were included, of whom 69 (16%) had early myoclonus. Among these patients, Pattern 1 was the most common, occurring in 48 patients (74%), whereas Pattern 2 occurred in 8 patients (12%). The remaining patients had subcortical myoclonus (n = 2, 3%) or other patterns (n = 7, 11%). No patients with Pattern 1, subcortical myoclonus, or other patterns survived with favorable outcome. By contrast, 4 of 8 patients (50%) with Pattern 2 on EEG survived, and 4 of 4 (100%) survivors had favorable outcomes despite remaining comatose for 1 to 2 weeks postarrest.InterpretationEarly PAMM is common after cardiac arrest. We describe 2 distinct patterns with distinct prognostic significances. For patients with Pattern 1 EEGs, it may be appropriate to abandon our current clinical standard of aggressive therapy with conventional antiepileptic therapy in favor of early limitation of care or novel neuroprotective strategies. Ann Neurol 2016;80:175-184.
| S-EPMC4982787 | biostudies-literature
Project description:BackgroundHemizygous mutations in GRIA3 encoding the GluA3 subunit of the amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor are known to be associated with neurodevelopmental disorders, including intellectual disability, hypotonia, an autism spectrum disorder, sleep disturbances, and epilepsy in males.ObjectiveTo describe a new and consistent phenotype in 4 affected male patients associated with an undescribed deleterious variant in GRIA3.MethodsWe evaluated a large French family in which segregate a singular phenotype according to an apparent X-linked mode of inheritance. Molecular analyses using next generation sequencing and in vitro functional studies using 2-electrode voltage clamp recordings on Xenopus laevis oocytes and a β-lactamase reporter assay in transfected human embryonic kidney (HEK293) cells were performed.ResultsIn addition to mild intellectual disability and dysarthria, affected patients presented a tightly consistent early-onset movement disorder combining an exaggerated startle reflex with generalized chorea and multifocal myoclonus. The unreported GRIA3 missense variant c.2477G > A; p.(Gly826Asp) affecting the fourth transmembrane domain of the protein was identified in index patients and their unaffected mothers. Functional studies revealed that variant receptors show decreased current response evoked by agonist (ie, kainic acid and glutamate) and reduced expression on the cell surface in favor of pathogenicity by a loss-of-function mechanism.ConclusionsTaken together, our results suggest that apart from known GRIA3-related disorders, an undescribed mutation-specific singular movement disorder does exist. We thus advocate considering GRIA3 mutations in the differential diagnosis of hyperekplexia and generalized chorea with myoclonus. © 2020 International Parkinson and Movement Disorder Society.
| S-EPMC9190290 | biostudies-literature
Project description:Rapid induction and maintaining a target temperature of 32.0-36.0°C within a narrow range for <24 hours are essential, but those are very hard to perform in postcardiac arrest syndrome (PCAS) patients. We investigated the usability of an intravascular temperature management (IVTM) system with neurolept-anesthesia (NLA; droperidol and fentanyl). Single-arm, prospective multicenter trial was carried out in the seven university and the three affiliated hospitals. In the 24 comatose PCAS patients, the target temperature (33.0°C) was rapidly induced and maintained for 24 hours using an IVTM system with NLA. The rewarming speed was 0.1°C/h until 36.5°C and was maintained for 24 hours. The primary end point was the ability to achieve ≤34.0°C for <3 hours after starting cooling, and the secondary end points were the cooling rate, deviation from the target temperature, and adverse events. Cerebral Performance Category (CPC) score at 14 days was also evaluated. Statistical analyses were performed by SPSS software, using the intention-to-treat data sets. The target temperature of ≤34.0°C was reached by 45 minutes (35-73 minutes) and was within 3 hours in all patients. The cooling rate from 36.4°C to 33.0°C was 2.7°C/h (2.4-3.6°C/h). The temperature of 33.1°C (33.1-33.1°C) and 36.7°C (36.6-36.9°C) for 24 hours each was held during the maintenance and the after rewarming phases, respectively. Temperature deviations >0.2°C from 33.0°C in the maintenance phase occurred once each in two patients. The favorable neurological outcomes (CPC1, 2) were relatively good (50%). Five patients experienced serious adverse events; none was device related. We rapidly achieved therapeutic hypothermia within a narrow temperature range without major complications using the IVTM system with NLA in PCAS patients.
| S-EPMC7482718 | biostudies-literature
Project description:Primary cardiac tumors are rare and approximately half of them are atrial myxomas. They rarely remain asymptomatic, especially if large. The imaging of a myxoma by contrast dye during coronary angiography is an infrequent sign, which clarifies the vascular supply of the tumor. We report herein an interesting and rare case of a left atrial myxoma hypervascularized from the right coronary artery.
| S-EPMC4737441 | biostudies-literature