Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 17 de 17
Filter
1.
JAMA Neurol ; 81(5): 507-514, 2024 May 01.
Article in English | MEDLINE | ID: mdl-38587858

ABSTRACT

Importance: Guidelines recommend seizure prophylaxis for early posttraumatic seizures (PTS) after severe traumatic brain injury (TBI). Use of antiseizure medications for early seizure prophylaxis after mild or moderate TBI remains controversial. Objective: To determine the association between seizure prophylaxis and risk reduction for early PTS in mild and moderate TBI. Data Sources: PubMed, Google Scholar, and Web of Science (January 1, 1991, to April 18, 2023) were systematically searched. Study Selection: Observational studies of adult patients presenting to trauma centers in high-income countries with mild (Glasgow Coma Scale [GCS], 13-15) and moderate (GCS, 9-12) TBI comparing rates of early PTS among patients with seizure prophylaxis with those without seizure prophylaxis. Data Extraction and Synthesis: The Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) reporting guidelines were used. Two authors independently reviewed all titles and abstracts, and 3 authors reviewed final studies for inclusion. A meta-analysis was performed using a random-effects model with absolute risk reduction. Main Outcome Measures: The main outcome was absolute risk reduction of early PTS, defined as seizures within 7 days of initial injury, in patients with mild or moderate TBI receiving seizure prophylaxis in the first week after injury. A secondary analysis was performed in patients with only mild TBI. Results: A total of 64 full articles were reviewed after screening; 8 studies (including 5637 patients) were included for the mild and moderate TBI analysis, and 5 studies (including 3803 patients) were included for the mild TBI analysis. The absolute risk reduction of seizure prophylaxis for early PTS in mild to moderate TBI (GCS, 9-15) was 0.6% (95% CI, 0.1%-1.2%; P = .02). The absolute risk reduction for mild TBI alone was similar 0.6% (95% CI, 0.01%-1.2%; P = .04). The number needed to treat to prevent 1 seizure was 167 patients. Conclusion and Relevance: Seizure prophylaxis after mild and moderate TBI was associated with a small but statistically significant reduced risk of early posttraumatic seizures after mild and moderate TBI. The small absolute risk reduction and low prevalence of early seizures should be weighed against potential acute risks of antiseizure medications as well as the risk of inappropriate continuation beyond 7 days.


Subject(s)
Anticonvulsants , Brain Injuries, Traumatic , Seizures , Humans , Brain Injuries, Traumatic/complications , Anticonvulsants/therapeutic use , Seizures/prevention & control , Seizures/etiology
2.
Brain Sci ; 14(2)2024 Jan 23.
Article in English | MEDLINE | ID: mdl-38391685

ABSTRACT

Despite recent medical therapeutic advances, approximately one third of patients do not attain seizure freedom with medications. This drug-resistant epilepsy population suffers from heightened morbidity and mortality. In appropriate patients, resective epilepsy surgery is far superior to continued medical therapy. Despite this efficacy, there remain drawbacks to traditional epilepsy surgery, such as the morbidity of open neurosurgical procedures as well as neuropsychological adverse effects. SEEG-guided Radiofrequency Thermocoagulation (SgRFTC) is a minimally invasive, electrophysiology-guided intervention with both diagnostic and therapeutic implications for drug-resistant epilepsy that offers a convenient adjunct or alternative to ablative and resective approaches. We review the international experience with this procedure, including methodologies, diagnostic benefit, therapeutic benefit, and safety considerations. We propose a framework in which SgRFTC may be incorporated into intracranial EEG evaluations alongside passive recording. Lastly, we discuss the potential role of SgRFTC in both delineating and reorganizing epilepsy networks.

3.
Resuscitation ; 188: 109823, 2023 07.
Article in English | MEDLINE | ID: mdl-37164175

ABSTRACT

BACKGROUND: Patients resuscitated from cardiac arrest have variable severity of primary hypoxic ischemic brain injury (HIBI). Signatures of primary HIBI on brain imaging and electroencephalography (EEG) include diffuse cerebral edema and burst suppression with identical bursts (BSIB). We hypothesize distinct phenotypes of primary HIBI are associated with increasing cardiopulmonary resuscitation (CPR) duration. METHODS: We identified from our prospective registry of both in-and out-of-hospital CA patients treated between January 2010 to January 2020 for this cohort study. We abstracted CPR duration, neurological examination, initial brain computed tomography gray to white ratio (GWR), and initial EEG pattern. We considered four phenotypes on presentation: awake; comatose with neither BSIB nor cerebral edema (non-malignant coma); BSIB; and cerebral edema (GWR ≤ 1.20). BSIB and cerebral edema were considered as non-mutually exclusive outcomes. We generated predicted probabilities of brain injury phenotype using localized regression. RESULTS: We included 2,440 patients, of whom 545 (23%) were awake, 1,065 (44%) had non-malignant coma, 548 (23%) had BSIB and 438 (18%) had cerebral edema. Only 92 (4%) had both BSIB and edema. Median CPR duration was 16 [IQR 8-28] minutes. Median CPR duration increased in a stepwise manner across groups: awake 6 [3-13] minutes; non-malignant coma 15 [8-25] minutes; BSIB 21 [13-31] minutes; cerebral edema 32 [22-46] minutes. Predicted probability of phenotype changes over time. CONCLUSIONS: Brain injury phenotype is related to CPR duration, which is a surrogate for severity of HIBI. The sequence of most likely primary HIBI phenotype with progressively longer CPR duration is awake, coma without BSIB or edema, BSIB, and finally cerebral edema.


Subject(s)
Brain Edema , Brain Injuries , Cardiopulmonary Resuscitation , Heart Arrest , Hypoxia-Ischemia, Brain , Out-of-Hospital Cardiac Arrest , Humans , Cardiopulmonary Resuscitation/adverse effects , Cardiopulmonary Resuscitation/methods , Cohort Studies , Brain Edema/etiology , Coma/complications , Heart Arrest/complications , Hypoxia-Ischemia, Brain/etiology , Brain Injuries/complications , Out-of-Hospital Cardiac Arrest/therapy
4.
Epilepsia ; 64(7): 1842-1852, 2023 07.
Article in English | MEDLINE | ID: mdl-37073101

ABSTRACT

OBJECTIVE: Posttraumatic epilepsy (PTE) develops in as many as one third of severe traumatic brain injury (TBI) patients, often years after injury. Analysis of early electroencephalographic (EEG) features, by both standardized visual interpretation (viEEG) and quantitative EEG (qEEG) analysis, may aid early identification of patients at high risk for PTE. METHODS: We performed a case-control study using a prospective database of severe TBI patients treated at a single center from 2011 to 2018. We identified patients who survived 2 years postinjury and matched patients with PTE to those without using age and admission Glasgow Coma Scale score. A neuropsychologist recorded outcomes at 1 year using the Expanded Glasgow Outcomes Scale (GOSE). All patients underwent continuous EEG for 3-5 days. A board-certified epileptologist, blinded to outcomes, described viEEG features using standardized descriptions. We extracted 14 qEEG features from an early 5-min epoch, described them using qualitative statistics, then developed two multivariable models to predict long-term risk of PTE (random forest and logistic regression). RESULTS: We identified 27 patients with and 35 without PTE. GOSE scores were similar at 1 year (p = .93). The median time to onset of PTE was 7.2 months posttrauma (interquartile range = 2.2-22.2 months). None of the viEEG features was different between the groups. On qEEG, the PTE cohort had higher spectral power in the delta frequencies, more power variance in the delta and theta frequencies, and higher peak envelope (all p < .01). Using random forest, combining qEEG and clinical features produced an area under the curve of .76. Using logistic regression, increases in the delta:theta power ratio (odds ratio [OR] = 1.3, p < .01) and peak envelope (OR = 1.1, p < .01) predicted risk for PTE. SIGNIFICANCE: In a cohort of severe TBI patients, acute phase EEG features may predict PTE. Predictive models, as applied to this study, may help identify patients at high risk for PTE, assist early clinical management, and guide patient selection for clinical trials.


Subject(s)
Brain Injuries, Traumatic , Epilepsy, Post-Traumatic , Humans , Case-Control Studies , Brain Injuries, Traumatic/complications , Brain Injuries, Traumatic/diagnosis , Epilepsy, Post-Traumatic/diagnosis , Epilepsy, Post-Traumatic/etiology , Electroencephalography , Glasgow Coma Scale
5.
Neurology ; 100(19): e1967-e1975, 2023 05 09.
Article in English | MEDLINE | ID: mdl-36948595

ABSTRACT

BACKGROUND AND OBJECTIVE: Nearly one-third of patients with severe traumatic brain injury (TBI) develop posttraumatic epilepsy (PTE). The relationship between PTE and long-term outcomes is unknown. We tested whether, after controlling for injury severity and age, PTE is associated with worse functional outcomes after severe TBI. METHODS: We performed a retrospective analysis of a prospective database of patients with severe TBI treated from 2002 through 2018 at a single level 1 trauma center. Glasgow Outcome Scale (GOS) was collected at 3, 6, 12, and 24 months postinjury. We used repeated-measures logistic regression predicting GOS, dichotomized as favorable (GOS 4-5) and unfavorable (GOS 1-3), and a separate logistic model predicting mortality at 2 years. We used predictors as defined by the International Mission for Prognosis and Analysis of Clinical Trials in TBI (IMPACT) base model (i.e., age, pupil reactivity, and GCS motor score), PTE status, and time. RESULTS: Of 392 patients who survived to discharge, 98 (25%) developed PTE. The proportion of patients with favorable outcomes at 3 months did not differ between those with and without PTE (23% [95% Confidence Interval [CI]: 15%-34%] vs 32% [95% CI: 27%-39%]; p = 0.11) but was significantly lower at 6 (33% [95% CI: 23%-44%] vs 46%; [95% CI: 39%-52%] p = 0.03), 12 (41% [95% CI: 30%-52%] vs 54% [95% CI: 47%-61%]; p = 0.03), and 24 months (40% [95% CI: 47%-61%] vs 55% [95% CI: 47%-63%]; p = 0.04). This was driven by higher rates of GOS 2 (vegetative) and 3 (severe disability) outcomes in the PTE group. By 2 years, the incidence of GOS 2 or 3 was double in the PTE group (46% [95% CI: 34%-59%]) compared with that in the non-PTE group (21% [95% CI: 16%-28%]; p < 0.001), while mortality was similar (14% [95% CI: 7%-25%] vs 23% [95% CI: 17%-30%]; p = 0.28). In multivariate analysis, patients with PTE had lower odds of favorable outcome (odds radio [OR] 0.1; 95% CI: 0.1-0.4; p < 0.001), but not mortality (OR 0.9; 95% CI: 0.1-1.9; p = 0.46). DISCUSSION: Posttraumatic epilepsy is associated with impaired recovery from severe TBI and poor functional outcomes. Early screening and treatment of PTE may improve patient outcomes.


Subject(s)
Brain Injuries, Traumatic , Epilepsy , Humans , Retrospective Studies , Brain Injuries, Traumatic/therapy , Prognosis , Glasgow Outcome Scale , Epilepsy/complications
6.
J Neurosurg ; 138(4): 992-1001, 2023 04 01.
Article in English | MEDLINE | ID: mdl-36087323

ABSTRACT

OBJECTIVE: In mesial temporal lobe epilepsy (MTLE), the ideal surgical approach to achieve seizure freedom and minimize morbidity is an unsolved question. Selective approaches to mesial temporal structures often result in suboptimal seizure outcomes. The authors report the results of a pilot study intended to evaluate the clinical feasibility of using an endoscopic anterior transmaxillary (eATM) approach for minimally invasive management of MTLEs. METHODS: The study is a prospectively collected case series of four consecutive patients who underwent the eATM approach for the treatment of MTLE and were followed for a minimum of 12 months. All participants underwent an epilepsy workup and surgical care at a tertiary referral comprehensive epilepsy center and had medically refractory epilepsy. The noninvasive evaluations and intracranial recordings of these patients confirmed the presence of anatomically restricted epileptogenic zones located in the mesial temporal structures. Data on seizure freedom at 1 year, neuropsychological outcomes, diffusion tractography, and adverse events were collected and analyzed. RESULTS: By applying the eATM technique and approaching the far anterior temporal lobe regions, mesial-basal resections of the temporal polar areas and mesial temporal structures were successfully achieved in all patients (2 with left-sided approaches, 2 with right-sided approaches). No neurological complications or neuropsychological declines were observed. All 4 patients achieved Engel class Ia outcome up to the end of the follow-up period (19, 15, 14, and 12 months). One patient developed hypoesthesia in the left V2 distribution but there were no other adverse events. The low degree of white matter injury from the eATM approach was analyzed using high-definition fiber tractography in 1 patient as a putative mechanism for preserving neuropsychological function. CONCLUSIONS: The described series demonstrates the feasibility and potential safety profile of a novel approach for medically refractory MTLE. The study affirms the feasibility of performing efficacious mesial temporal lobe resections through an eATM approach.


Subject(s)
Epilepsy, Temporal Lobe , Epilepsy , Humans , Epilepsy, Temporal Lobe/diagnostic imaging , Epilepsy, Temporal Lobe/surgery , Feasibility Studies , Pilot Projects , Treatment Outcome , Temporal Lobe/diagnostic imaging , Temporal Lobe/surgery , Hippocampus/surgery
7.
Brain ; 145(11): 3901-3915, 2022 11 21.
Article in English | MEDLINE | ID: mdl-36412516

ABSTRACT

Over 15 million epilepsy patients worldwide have drug-resistant epilepsy. Successful surgery is a standard of care treatment but can only be achieved through complete resection or disconnection of the epileptogenic zone, the brain region(s) where seizures originate. Surgical success rates vary between 20% and 80%, because no clinically validated biological markers of the epileptogenic zone exist. Localizing the epileptogenic zone is a costly and time-consuming process, which often requires days to weeks of intracranial EEG (iEEG) monitoring. Clinicians visually inspect iEEG data to identify abnormal activity on individual channels occurring immediately before seizures or spikes that occur interictally (i.e. between seizures). In the end, the clinical standard mainly relies on a small proportion of the iEEG data captured to assist in epileptogenic zone localization (minutes of seizure data versus days of recordings), missing opportunities to leverage these largely ignored interictal data to better diagnose and treat patients. IEEG offers a unique opportunity to observe epileptic cortical network dynamics but waiting for seizures increases patient risks associated with invasive monitoring. In this study, we aimed to leverage interictal iEEG data by developing a new network-based interictal iEEG marker of the epileptogenic zone. We hypothesized that when a patient is not clinically seizing, it is because the epileptogenic zone is inhibited by other regions. We developed an algorithm that identifies two groups of nodes from the interictal iEEG network: those that are continuously inhibiting a set of neighbouring nodes ('sources') and the inhibited nodes themselves ('sinks'). Specifically, patient-specific dynamical network models were estimated from minutes of iEEG and their connectivity properties revealed top sources and sinks in the network, with each node being quantified by source-sink metrics. We validated the algorithm in a retrospective analysis of 65 patients. The source-sink metrics identified epileptogenic regions with 73% accuracy and clinicians agreed with the algorithm in 93% of seizure-free patients. The algorithm was further validated by using the metrics of the annotated epileptogenic zone to predict surgical outcomes. The source-sink metrics predicted outcomes with an accuracy of 79% compared to an accuracy of 43% for clinicians' predictions (surgical success rate of this dataset). In failed outcomes, we identified brain regions with high metrics that were untreated. When compared with high frequency oscillations, the most commonly proposed interictal iEEG feature for epileptogenic zone localization, source-sink metrics outperformed in predictive power (by a factor of 1.2), suggesting they may be an interictal iEEG fingerprint of the epileptogenic zone.


Subject(s)
Epilepsy , Seizures , Humans , Retrospective Studies , Electrocorticography/methods , Epilepsy/diagnosis , Epilepsy/surgery , Biomarkers
8.
Resuscitation ; 172: 17-23, 2022 03.
Article in English | MEDLINE | ID: mdl-35041875

ABSTRACT

INTRODUCTION: Guidelines recommend use of computerized tomography (CT) and electroencephalography (EEG) in post-arrest prognostication. Strong associations between CT and EEG might obviate the need to acquire both modalities. We quantified these associations via deep learning. METHODS: We performed a single-center, retrospective study including comatose patients hospitalized after cardiac arrest. We extracted brain CT DICOMs, resized and registered each to a standard anatomical atlas, performed skull stripping and windowed images to optimize contrast of the gray-white junction. We classified initial EEG as generalized suppression, other highly pathological findings or benign activity. We extracted clinical information available on presentation from our prospective registry. We trained three machine learning (ML) models to predict EEG from clinical covariates. We used three state-of-the-art approaches to build multi-headed deep learning models using similar model architectures. Finally, we combined the best performing clinical and imaging models. We evaluated discrimination in test sets. RESULTS: We included 500 patients, of whom 218 (44%) had benign EEG findings, 135 (27%) showed generalized suppression and 147 (29%) had other highly pathological findings that were most commonly (93%) burst suppression with identical bursts. Clinical ML models had moderate discrimination (test set AUCs 0.73-0.80). Image-based deep learning performed worse (test set AUCs 0.51-0.69), particularly discriminating benign from highly pathological findings. Adding image-based deep learning to clinical models improved prediction of generalized suppression due to accurate detection of severe cerebral edema. DISCUSSION: CT and EEG provide complementary information about post-arrest brain injury. Our results do not support selective acquisition of only one of these modalities, except in the most severely injured patients.


Subject(s)
Deep Learning , Brain/diagnostic imaging , Electroencephalography/methods , Humans , Neuroimaging , Prognosis , Retrospective Studies
9.
J Clin Neurophysiol ; 39(2): 159-165, 2022 Feb 01.
Article in English | MEDLINE | ID: mdl-32639251

ABSTRACT

PURPOSE: Neurologic manifestations of coronavirus disease (COVID-19) such as encephalopathy and seizures have been described. To our knowledge, detailed EEG findings in COVID-19 have not yet been reported. This report adds to the scarce body of evidence. METHODS: We identified eight COVID-19 positive patients who underwent EEG monitoring in our hospital system. RESULTS: EEGs were most commonly ordered for an altered level of consciousness, a nonspecific neurologic manifestation. We observed generalized background slowing in all patients and generalized epileptiform discharges with triphasic morphology in three patients. Focal electrographic seizures were observed in one patient with a history of focal epilepsy and in another patient with no such history. Five of eight patients had a previous diagnosis of epilepsy, suggesting that pre-existing epilepsy can be a potential risk factor for COVID-19-associated neurological manifestations. Five of eight patients who underwent EEG experienced a fatal outcome of infection. CONCLUSIONS: Our findings underscore previous observations that neurologic manifestations are common in severe cases. COVID-19 patients with epilepsy may have an increased risk of neurological manifestations and abnormal EEG.


Subject(s)
COVID-19 , Epilepsies, Partial , Electroencephalography , Humans , SARS-CoV-2 , Seizures/diagnosis , Seizures/etiology
10.
J Neurol Neurosurg Psychiatry ; 93(2): 133-143, 2022 02.
Article in English | MEDLINE | ID: mdl-34321344

ABSTRACT

BACKGROUND: Approximately 1/3 of patients with epilepsy have drug-resistant epilepsy (DRE) and require surgical interventions. This meta-analysis aimed to review the effectiveness of MRI-guided laser interstitial thermal therapy (MRgLITT) in DRE. METHODS: The Population, Intervention, Comparator and Outcome approach and Preferred Reporting Items for Systematic Reviews and Meta-Analyses were followed. PubMed, MEDLINE and EMBASE databases were systematically searched for English language publications from 2012 to Nov 2020. Data on the prevalence outcome using the Engel Epilepsy Surgery Outcome Scale (Class I-IV), and postoperative complications were analysed with 95% CIs. RESULTS: Twenty-eight studies that included a total of 559 patients with DRE were identified. The overall prevalence of Engel class I outcome was 56% (95% CI 0.52% to 0.60%). Hypothalamic hamartomas (HH) patients had the highest seizure freedom rate of 67% (95% CI 0.57% to 0.76%) and outcome was overall comparable between mesial temporal lobe epilepsy (mTLE) (56%, 95% CI 0.50% to 0.61%) and extratemporal epilepsy (50% 95% CI 0.40% to 0.59%). The mTLE cases with mesial temporal sclerosis had better outcome vs non-lesional cases of mTLE. The prevalence of postoperative adverse events was 19% (95% CI 0.14% to 0.25%) and the most common adverse event was visual field deficits. The reoperation rate was 9% (95% CI 0.05% to 0.14%), which included repeat ablation and open resection. CONCLUSION: MRgLITT is an effective and safe intervention for DRE with different disease aetiologies. The seizure freedom outcome is overall comparable in between extratemporal and temporal lobe epilepsy; and highest with HH. TRAIL REGISTRATION NUMBER: The study protocol was registered with the National Institute for Health Research (CRD42019126365), which serves as a prospective register of systematic reviews. It is an international database of prospectively registered systematic reviews with a focus on health-related outcomes. Details about the protocol can be found at https://wwwcrdyorkacuk/PROSPERO/.


Subject(s)
Drug Resistant Epilepsy/surgery , Hamartoma/surgery , Hypothalamic Diseases/surgery , Laser Therapy/methods , Magnetic Resonance Imaging/methods , Adult , Epilepsies, Partial/surgery , Epilepsy, Temporal Lobe/surgery , Female , Humans , Male , Middle Aged , Postoperative Period , Treatment Outcome
11.
Resusc Plus ; 7: 100151, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34386780

ABSTRACT

BACKGROUND: Electroencephalography (EEG) is commonly used after cardiac arrest. Burst suppression with identical bursts (BSIB) has been reported as a perfectly specific predictor of poor outcome but published case series are small. We describe two patients with BSIB who awakened from coma after cardiac arrest. METHODS: We identified two out-of-hospital cardiac arrest (OHCA) patients with coma and BSIB. We determined the etiology of arrest, presenting neurological examination, potential confounders to neurological assessment, neurodiagnostics and time to awakening. We reviewed and interpreted EEGs using 2021 American Clinical Neurophysiology Society guidelines. We quantified identicality of bursts by calculating pairwise correlation coefficients between the first 500 ms of each aligned burst. RESULTS: In case one we present a 62-year-old man with OHCA secondary to septic shock. EEG showed burst suppression pattern, with bursts consisted of high amplitude generalized spike waves in lock-step with myoclonus (inter-burst correlation = 0.86). He followed commands 3 days after arrest, when repeat EEG showed a continuous, variable and reactive background without epileptiform activity. Case two was a 49-year-old woman with OHCA secondary to polysubstance overdose. Initial EEG revealed burst suppression with high amplitude generalized polyspike-wave bursts with associated myoclonus. She followed commands on post-arrest day 4, when repeat EEG showed a continuous, variable and reactive background with frequent runs of bifrontal predominant sharply contoured rhythmic delta activity. CONCLUSION: These cases highlight the perils of prognosticating with a single modality in comatose cardiac arrest patients.

12.
Oper Neurosurg (Hagerstown) ; 21(4): 258-264, 2021 09 15.
Article in English | MEDLINE | ID: mdl-34293155

ABSTRACT

BACKGROUND: Stereoelectroencephalography (SEEG) is an effective method to define the epileptogenic zone (EZ) in patients with medically intractable epilepsy. Typical placement requires passing and anchoring electrodes through native skull. OBJECTIVE: To describe the successful placement of SEEG electrodes in patients without native bone. To the best of our knowledge, the use of SEEG in patients with nonautologous cranioplasties has not been described. METHODS: We describe 3 cases in which SEEG was performed through nonautologous cranioplasty. The first is a 30-yr-old male with a titanium mesh cranioplasty following a left pterional craniotomy for aneurysm clipping. The second is a 51-yr-old female who previously underwent lesionectomy of a ganglioglioma with mesh cranioplasty and subsequent recurrence of her seizures. The third is a 31-yr-old male with a polyether ether ketone cranioplasty following decompressive hemicraniectomy for trauma. RESULTS: SEEG was performed successfully in all three cases without intraoperative difficulties or complications and with excellent electroencephalogram recording and optimal localization of the seizure focus. The EZ was successfully localized in all three patients. There were no limitations related to drilling or inserting the guiding bolt/electrode through the nonautologous cranioplasties. CONCLUSION: SEEG through nonautologous cranioplasties was clinically feasible, safe, and effective in our series. The presence of nonautologous bone cranioplasty should not preclude such patients from undergoing SEEG explorations.


Subject(s)
Brain Neoplasms , Electroencephalography , Electrodes, Implanted , Female , Humans , Male , Skull/surgery , Stereotaxic Techniques
13.
Epileptic Disord ; 22(5): 654-658, 2020 Oct 01.
Article in English | MEDLINE | ID: mdl-32972906

ABSTRACT

Progressive myoclonic epilepsy (PME) is characterized by prominent myoclonus and generalized or focal seizures. A recently described novel KCNC1 mutation is associated with a specific phenotype of progressive myoclonic epilepsy, which has been defined as myoclonic epilepsy and ataxia due to potassium channel mutation (MEAK). Our case illustrates a typical presentation of this disease and the potential for misdiagnosis as idiopathic generalized epilepsy during the early phase of the disease. Unique findings that may suggest an alternative diagnosis are a progressive myoclonus, prominent ataxia/dysmetria on examination, and abnormally high amplitude in the sensory evoked potential recording. We also report a brief review of the existing literature on MEAK. Early and accurate diagnosis with genetic testing may significantly help in counseling patients and families.


Subject(s)
Myoclonic Epilepsies, Progressive/diagnosis , Myoclonic Epilepsies, Progressive/physiopathology , Shaw Potassium Channels/genetics , Adolescent , Ataxia/diagnosis , Ataxia/genetics , Ataxia/physiopathology , Electroencephalography , Epilepsy, Generalized/diagnosis , Genetic Testing , Humans , Magnetic Resonance Imaging , Male , Myoclonic Epilepsies, Progressive/genetics
14.
Neurosurg Clin N Am ; 31(3): 319-324, 2020 Jul.
Article in English | MEDLINE | ID: mdl-32475482

ABSTRACT

Epilepsy affects about 1% of the general population. Frontal lobe epilepsy is the second most common focal epilepsy accounting for nearly 25% of medically refractory epilepsies. This paper reviews frontal lobe epilepsy from a perspective of a network disease that may help us to understand epilepsy from the microscale of genes, to local neuronal circuits, to the macrolevel of a whole-brain network. Surgical interventions, such as ablation and resection act by removing the active target nodes in the network, while responsive neurostimulation and vagus nerve stimulation act by modulating networks at the local neuronal circuit level and whole-brain level.


Subject(s)
Brain/physiopathology , Epilepsy, Frontal Lobe/genetics , Epilepsy, Frontal Lobe/physiopathology , Models, Neurological , Seizures/genetics , Seizures/physiopathology , Humans , Neural Pathways/physiopathology , Neurons/physiology , Signal Transduction
15.
Clin Auton Res ; 29(2): 151-160, 2019 04.
Article in English | MEDLINE | ID: mdl-30456432

ABSTRACT

Sudden unexpected death in epilepsy (SUDEP) is a major cause of epilepsy-related mortality. SUDEP is highly linked to seizures, with most deaths occurring after convulsive seizures in sleep. In most cases of SUDEP, convulsive seizures appear to directly trigger catastrophic cardiorespiratory dysfunction leading to death. In the last few decades, many pathophysiological mechanisms have been proposed to explain the sequence of events leading to death. Patients with epilepsy often have underlying autonomic dysfunction, as measured by heart rate variability and other testing modalities. Additionally, seizures often trigger acute cardiac and respiratory dysfunction. While sinus tachycardia is the most common cardiac finding during seizures, asystole and malignant tachyarrhythmias may also occur. Seizures can also lead to respiratory dysfunction, including central ictal and obstructive apnea related to laryngospasm. Available data suggest that there could be underlying autonomic dysfunction, potentially related to genetic, medication, and other factors that might predispose individuals to sudden catastrophic cardio-respiratory dysfunction in the setting of a seizure, resulting in SUDEP. Further exploration of this possible link is needed. Patients with medically refractory epilepsy are at the highest risk, and adequate management via medical therapy to control convulsive seizures, or surgical intervention may decrease the SUDEP risk. Recently, many automated seizure detection systems have been developed to detect convulsive seizures, which may enable caregivers to more closely monitor individuals with epilepsy. Improved identification of seizures may be important for patients with refractory epilepsy as close supervision and timely intervention after a seizure could potentially reduce the risk of SUDEP.


Subject(s)
Autonomic Nervous System Diseases/physiopathology , Autonomic Nervous System/physiopathology , Epilepsy/physiopathology , Sudden Unexpected Death in Epilepsy , Humans
16.
Epilepsy Res Treat ; 2016: 7982494, 2016.
Article in English | MEDLINE | ID: mdl-27069682

ABSTRACT

Objective. We investigated the longitudinal outcome of resective epilepsy surgery to identify the predictors of seizure recurrence. Materials and Methods. We retrospectively analyzed patients who underwent resections for intractable epilepsy over a period of 7 years. Multiple variables were investigated as potential predictors of seizure recurrence. The time to first postoperative seizure was evaluated using survival analysis and univariate analysis at annual intervals. Results. Among 70 patients, 54 (77%) had temporal and 16 (23%) had extratemporal resections. At last follow-up (mean 48 months; range 24-87 months), the outcome was Engel class I in 84% (n = 59) of patients. Seizure recurrence followed two patterns: recurrence was "early" (within 2 years) in 82% of patients, of whom 83% continued to have seizures despite optimum medical therapy; recurrence was "late" (after 2 years) in 18%, of whom 25% continued to have seizures subsequently. Among the variables of interest, only resection site and ictal EEG remained as independent predictors of seizure recurrence over the long term (p < 0.05). Extratemporal resection and discordance between ictal EEG and resection area were associated with 4.2-fold and 5.6-fold higher risk of seizure recurrence, respectively. Conclusions. Extratemporal epilepsy and uncertainty in ictal EEG localization are independent predictors of unfavorable outcome. Seizure recurrence within two years of surgery indicates poor long-term outcome.

17.
J Stroke Cerebrovasc Dis ; 23(3): e207-14, 2014 Mar.
Article in English | MEDLINE | ID: mdl-24321775

ABSTRACT

BACKGROUND: Symptomatic intracranial hemorrhage (sICH) occurs uncommonly after ischemic stroke therapy with tissue plasminogen activator (tPA). Clotting factor administration may be a treatment option. OBJECTIVE: To determine if treatment with clotting factors (fresh frozen plasma [FFP] or cryoprecipitate) was associated with improved outcomes in sICH. METHODS: We conducted a retrospective cohort study within University of Texas at Houston Stroke registry involving consecutive patients from February 1, 2007, to June 30, 2011, with tPA-related sICH, including cases with subsequent intra-arterial therapy. Outcomes were Modified Rankin Scale (mRS) score at discharge, death, and hematoma expansion. RESULTS: Of 921 patients treated with tPA, 48 (5.2%) had sICH and 45 met criteria for the study. Nineteen patients received clotting factors (42.2%; 18 received FFP and 7 received cryoprecipitate), whereas 26 (57.8%) patients received conservative management without clotting factors. None of the patients treated with clotting factors and only 2 of those who did not receive clotting factors had a good outcome, mRS score of 2 or less. All the patients treated with clotting factors and most of those not treated were left bedridden or dead (mRS score 4-6), 19 (100%) versus 22 (85%). Mortality was 9 (47.4%) versus 9 (34.6%), respectively. There was no difference in hematoma expansion between the 2 groups. CONCLUSIONS: We found no evidence that treatment for sICH with clotting factors has a favorable effect on clinical or radiological outcomes. However, the sample was small because of the low frequency of sICH. New treatments are urgently needed for this uncommon yet serious condition.


Subject(s)
Blood Coagulation/drug effects , Brain Ischemia/drug therapy , Coagulants/therapeutic use , Fibrinolytic Agents/adverse effects , Intracranial Hemorrhages/drug therapy , Stroke/drug therapy , Thrombolytic Therapy/adverse effects , Tissue Plasminogen Activator/adverse effects , Aged , Aged, 80 and over , Brain Ischemia/blood , Brain Ischemia/diagnosis , Brain Ischemia/mortality , Coagulants/adverse effects , Female , Hospitals, University , Humans , Intracranial Hemorrhages/chemically induced , Intracranial Hemorrhages/diagnosis , Intracranial Hemorrhages/mortality , Male , Middle Aged , Registries , Retrospective Studies , Risk Factors , Stroke/blood , Stroke/diagnosis , Stroke/mortality , Texas , Thrombolytic Therapy/mortality , Time Factors , Treatment Outcome
SELECTION OF CITATIONS
SEARCH DETAIL
...