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Seizure Jul 2024Epileptic Encephalopathy / Developmental Epileptic Encephalopathy with spike-and-wave activation during sleep (EE/DEE-SWAS) is a self-limiting childhood epilepsy... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Epileptic Encephalopathy / Developmental Epileptic Encephalopathy with spike-and-wave activation during sleep (EE/DEE-SWAS) is a self-limiting childhood epilepsy syndrome but may cause permanent neurocognitive impairment. Surgical interventions have been controversial in the treatment of EE/DEE-SWAS. This systematic review aims to evaluate the efficacy of various surgical procedures on the outcomes of EE/DEE-SWAS.
METHODS
A systematic review was performed per the PRISMA guidelines. A total of 14 retrospective studies were identified, comprising 131 cases of EE/DEE-SWAS treated with epilepsy surgery. The review analyzed presurgical data, surgical interventions, as well as outcomes related to seizures, EEG, and neuropsychological assessments.
RESULTS
Epilepsy surgery was successfully performed in 131 cases with minor complications. The average age was 2.6 years at seizure onset and 5.0 years at diagnosis of SWAS. Excellent seizure control (Engel I and II) was achieved in 80.6 %, 78.6 %, 77.4 % and 27.2 % of patients receiving hemispherectomies, focal resections, multiple subpial transections (MSTs), and corpus callosotomies (CCTs), respectively. EEG SWAS resolution was seen in 79.7 % of hemispherectomy cases, 78.6 % in focal resections, 63.9 % in MSTs, and 8.3 % in CCTs. Neurocognitive and behavioral improvement was noted in 58.0 %, 71.4 %, 58.3 % and 16.7 % for patients receiving hemispherectomies, focal resections, MSTs, and CCTs, respectively. A correlation between improved seizure control and SWAS resolution was observed with improved neuropsychological outcomes.
CONCLUSION
Epilepsy surgery is a safe and effective treatment for carefully selected children with drug-resistant EE/DEE-SWAS. Patients who underwent epilepsy surgery had reduction of seizure burden, SWAS resolution and improvements in neurocognitive and behavioral function.
Topics: Humans; Electroencephalography; Sleep; Neurosurgical Procedures; Child; Spasms, Infantile
PubMed: 38820673
DOI: 10.1016/j.seizure.2024.05.008 -
Neurosurgical Review May 2024Cerebral cavernous malformations (CCMs) are cerebral vascular lesions that occasionally occur with seizures. We present a retrospective case series from IRCCS Gaslini... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
Cerebral cavernous malformations (CCMs) are cerebral vascular lesions that occasionally occur with seizures. We present a retrospective case series from IRCCS Gaslini Children's Hospital, a systematic review, and meta-analysis of the literature with the goal of elucidating the post-surgery seizure outcome in children with CCMs.
METHODS
a retrospective review of children with cavernous malformation related epilepsy who underwent surgery at Gaslini Children's Hospital from 2005 to 2022 was conducted. We also conducted a comprehensive search on PubMed/MEDLINE and Scopus databases from January 1989 to August 2022. Inclusion criteria were: presence of CCMs-related epilepsy, in under 18 years old subjects with a clear lesion site. Presence of post-surgery seizure outcome and follow-up ≥ 12 months.
RESULTS
we identified 30 manuscripts and 223 patients with CCMs-related epilepsy, including 17 patients reported in our series. We identified 85.7% Engel class I subjects. The risk of expected neurological deficits was 3.7%; that of unexpected neurological deficits 2.8%. We found no statistically significant correlations between Engel class and the following factors: site of lesion, type of seizure, drug resistance, duration of disease, type of surgery, presence of multiple CCMs. However, we found some interesting trends: longer disease duration and drug resistance seem to be more frequent in subjects in Engel class II, III and IV; multiple cavernomas would not seem to influence seizure outcome.
CONCLUSIONS
epilepsy surgery in children with CCMs is a safe and successful treatment option. Further studies are necessary to define the impact of clinical features on seizure prognosis.
Topics: Adolescent; Child; Child, Preschool; Female; Humans; Male; Epilepsy; Hemangioma, Cavernous, Central Nervous System; Neurosurgical Procedures; Retrospective Studies; Seizures; Treatment Outcome; Infant
PubMed: 38819574
DOI: 10.1007/s10143-024-02491-0 -
The Archives of Bone and Joint Surgery 2024To compile the existing literature on bilateral anterior shoulder dislocation (BASD) and analyze patient demographics, mechanisms of injury, injury characteristics,... (Review)
Review
OBJECTIVES
To compile the existing literature on bilateral anterior shoulder dislocation (BASD) and analyze patient demographics, mechanisms of injury, injury characteristics, management, and outcome.
METHODS
This systematic review was conducted in accordance with Preferred Reporting Items for Systematic review and Meta-Analyses (PRISMA) guidelines. Online databases, including Ovid Medline 1946-, Embase.com 1947-, Scopus 1960-, Cochrane Central, and Clinicaltrials.gov were systematically queried. Studies eligible for inclusion were case reports or case-series, documenting BASD. Two reviewers independently screened and applied a set of a priori exclusion criteria to each returned study. Data were extracted, compiled, and synthesized from each reported case of BASD. Contingency tables/Chi-Square Analyses, T-tests, and univariate regression analyses were conducted to assess relationships between different variables.
RESULTS
Eighty-one studies (87 cases of BASD) were included. Patients were 41.1 (SD± 19.5) years old and most were male (n=63; 72.4%). Around a quarter of patients (28.7%) had a history of epilepsy/seizures or were being worked-up for such. Younger males were more likely to have BASD due to a seizure or electrocution (P<0.05). Close to a third of cases (n=27; 31.0%) were delayed in presentation. Those sustaining seizures or electrocutions were more likely to be delayed in presentation (P=0.013). Most events resulted in simple dislocations that were closed reduced successfully. BASD resulting from seizures or electrocutions were more likely to be fracture-dislocations (P=0.018); and in younger patients with fracture-dislocations, closed reduction was more often to fail or not be attempted (P<0.05). Median follow-up was 6 months (IQR: 3 months - 12 months). Seven patients (10.6%) had complications and 4 (2.3%) demonstrated recurrent instability.
CONCLUSION
In young males presenting with BASD without known trauma, suspicion should be high for a convulsant event. In patients with a known seizure disorder who present with chronic bilateral shoulder or arm pain, BASD should be considered and work-up should be expedited to avoid misdiagnosis.
PubMed: 38817413
DOI: 10.22038/ABJS.2024.67743.3211 -
Neurology Jun 2024Parietal lobe epilepsy (PLE) surgery can be an effective treatment for selected patients with intractable epilepsy but can be associated with the risk of serious...
BACKGROUND AND OBJECTIVES
Parietal lobe epilepsy (PLE) surgery can be an effective treatment for selected patients with intractable epilepsy but can be associated with the risk of serious neurologic deficits. We performed a systematic review of the literature to obtain a comprehensive summary of the frequency and types of new postoperative neurologic deficits in patients undergoing PLE resective surgery.
METHODS
We searched MEDLINE, Embase, and Cochrane Central Register of Controlled Trials for articles published between January 1, 1990, and April 28, 2022. We included studies that reported postoperative neurologic outcome following PLE resective surgery confined to the parietal lobe. We required that studies included ≥5 patients. The data collected included demographic information and specific details of postoperative neurologic deficits. When available, individual patient data were collected. We used the Risk of Bias in Nonrandomized Studies of Interventions tool to assess the risk of bias and Grading of Recommendations Assessment, Development, and Evaluation to assess the quality of the evidence.
RESULTS
Of the 3,461 articles screened, 33 studies met the inclusion criteria. A total of 370 patients were included. One hundred patients (27.0%) had a new deficit noted postoperatively. Approximately half of the patients with deficits experienced only transient deficits. Motor deficits were the most commonly identified deficit. The rates of motor deficits noted after PLE surgery were 5.7%, 3.2%, and 2.2% for transient, long-term, and duration not specified, respectively. Sensory and visual field deficits were also commonly reported. Gerstmann syndrome was noted postoperatively in 4.9% of patients and was almost always transient. Individual patient data added information on parietal lobe subregion postoperative neurologic outcome.
DISCUSSION
Our systematic review provides a comprehensive summary of the frequency and types of neurologic deficits associated with PLE surgery. A significant percentage of postoperative deficits are transient. In addition to the expected sensory and visual deficits, PLE surgery is associated with a notable risk of motor deficits. The available literature has important deficiencies. Our study highlights gaps in the literature and provides recommendations for future directions.
TRIAL REGISTRATION INFORMATION
This systematic review was registered on PROSPERO (CRD42022313108, May 26, 2022).
Topics: Humans; Parietal Lobe; Postoperative Complications; Neurosurgical Procedures; Drug Resistant Epilepsy; Treatment Outcome; Epilepsies, Partial
PubMed: 38815235
DOI: 10.1212/WNL.0000000000209322 -
Journal of Neurological Surgery Reports Apr 2024Diastematomyelia is a rare congenital disorder characterized by the separation of the spinal cord by an osseocartilaginous or fibrous septum. While diastematomyelia has...
Diastematomyelia is a rare congenital disorder characterized by the separation of the spinal cord by an osseocartilaginous or fibrous septum. While diastematomyelia has been reported to be more common in the thoracic and lumbar regions, the true incidence of cervical diastematomyelia is currently unknown. In this study, we conducted the most comprehensive systematic review to date of all other case reports of diastematomyelia to better characterize the incidence of cervical diastematomyelia and provide comprehensive statistics on the clinical characteristics of diastematomyelia generally. Ninety-one articles were included in our study, which comprised 252 males (27.9%) and 651 females (72.0%) (and one patient with unspecified gender). In 507 cases, the vertebral level of the diastematomyelia was described, and we recorded those levels as either cervical ( = 8, 1.6%), thoracic ( = 220, 43.4%), lumbar ( = 277, 54.6%), or sacral ( = 2, 0.4%). In 719 cases, the type of diastematomyelia was specified as either Type I ( = 482, 67.0%) or Type II ( = 237, 33.0%). Our study found that diastematomyelia has been reported in the cervical region in only 1.6% of cases, and we provide comprehensive data that this disorder occurs in female-to-male ratio of approximately 2.6:1 and Type I versus Type II diastematomyelia in an estimated ratio of 2:1.
PubMed: 38798790
DOI: 10.1055/a-2319-3444 -
Epilepsia Open May 2024To evaluate the prevalence of and risk factors for attention-deficit/hyperactivity disorder (ADHD) in children with epilepsy (CWE). (Review)
Review
OBJECTIVE
To evaluate the prevalence of and risk factors for attention-deficit/hyperactivity disorder (ADHD) in children with epilepsy (CWE).
METHODS
We conducted a systematic search in PubMed and Embase for the meta-analysis. The pooled prevalence of ADHD was calculated using a random-effects model; subgroup analyses were performed to explore heterogeneity. We collected raw data from articles reporting potential risk factors, which were included in the subsequent risk factor analysis.
RESULTS
Forty-six articles met the inclusion criteria for the meta-analysis, which showed a pooled ADHD prevalence of 30.7% in CWE, with a predominance of the inattentive subtype of ADHD; the heterogeneity of prevalence was related to population source/study setting (clinic based, community based, or database based) and method of ADHD diagnosis (with or without clinical review). Risk factors for ADHD in epilepsy included younger age, intellectual/developmental disabilities, a family history of epilepsy, earlier epilepsy onset, absence epilepsy, more frequent seizures, and polytherapy; In contrast, risk factors such as sex, generalized epilepsy or seizures, epilepsy etiology, and electroencephalogram abnormalities were not significantly associated with the occurrence of ADHD.
SIGNIFICANCE
The prevalence of ADHD in CWE is high and several potential risk factors are associated with it. This study contributes to a better understanding of ADHD in epilepsy for screening and treatment.
PLAIN LANGUAGE SUMMARY
This systematic review summarizes the prevalence of attention-deficit/hyperactivity disorder (ADHD) occurring in children with epilepsy and analyses the risk factors for comorbid ADHD in epilepsy. By reviewing 46 articles, we concluded that the overall prevalence of ADHD in children with epilepsy was 30.7% and that intellectual/developmental disabilities were the most significant risk factor for combined ADHD in children with epilepsy. This study provides a wealth of information on comorbid ADHD in epilepsy, which will help clinicians identify and treat potential ADHD in children with epilepsy in a timely manner.
PubMed: 38798030
DOI: 10.1002/epi4.12939 -
Seizure Jul 2024Traumatic brain injury (TBI) is often followed by post-traumatic epilepsy (PTE), a condition often difficult to treat and leading to a substantial decline in quality of... (Review)
Review
Traumatic brain injury (TBI) is often followed by post-traumatic epilepsy (PTE), a condition often difficult to treat and leading to a substantial decline in quality of life as well as increased long-term mortality. The latent period between TBI and the emergence of spontaneous recurrent seizures provides an opportunity for pharmacological intervention to prevent epileptogenesis. Biomarkers capable of predicting PTE development are urgently needed to facilitate clinical trials of putative anti-epileptogenic drugs. EEG is a widely available and flexible diagnostic modality that plays a fundamental role in epileptology. We systematically review the advances in the field of the discovery of EEG biomarkers for the prediction of PTE in humans. Despite recent progress, the field faces several challenges including short observation periods, a focus on early post-injury monitoring, difficulties in translating findings from animal models to scalp EEG, and emerging evidence indicating the importance of assessing altered background scalp EEG activity alongside epileptiform activity using quantitative EEG methods while also considering sleep abnormalities in future studies.
Topics: Humans; Epilepsy, Post-Traumatic; Electroencephalography; Biomarkers; Brain Injuries, Traumatic; Animals
PubMed: 38796954
DOI: 10.1016/j.seizure.2024.05.006 -
International Journal of Molecular... May 2024The gene encodes an orphan transcription factor of the steroid-thyroid hormone-retinoid receptor superfamily. This review focuses on the clinical findings associated... (Review)
Review
The gene encodes an orphan transcription factor of the steroid-thyroid hormone-retinoid receptor superfamily. This review focuses on the clinical findings associated with the pathogenic variants so far reported, including three unreported cases. Also, its role in neurodegenerative diseases, such as Parkinson's or Alzheimer's disease, is examined, as well as a brief exploration on recent proposals to develop novel therapies for these neurological diseases based on small molecules that could modulate transcriptional activity. The main characteristic shared by all patients is mild to severe developmental delay/intellectual disability. Moderate to severe disorder of the expressive and receptive language is present in at least 42%, while neuro-psychiatric issues were reported in 53% of patients. Movement disorders, including dystonia, chorea or ataxia, are described in 37% patients, although probably underestimated because of its frequent onset in late adolescence-young adulthood. Finally, epilepsy was surprisingly present in 42% of patients, being drug-resistant in three of them. The age at onset varied widely, from five months to twenty-six years, as did the classification of epilepsy, which ranged from focal epilepsy to infantile spasms or Lennox-Gastaut syndrome. Accordingly, we propose that should be considered as a first-tier target gene for the genetic diagnosis of developmental and epileptic encephalopathy.
Topics: Humans; Epilepsy; Nuclear Receptor Subfamily 4, Group A, Member 2; Developmental Disabilities; Intellectual Disability
PubMed: 38791237
DOI: 10.3390/ijms25105198 -
Journal of Neurology May 2024Ketamine, as an anesthetic, has been considered for terminating status epilepticus (SE); however, due to the urgency and severity of the condition, there are currently... (Review)
Review
BACKGROUND
Ketamine, as an anesthetic, has been considered for terminating status epilepticus (SE); however, due to the urgency and severity of the condition, there are currently no randomized controlled trials internationally assessing the efficacy of ketamine for treating super-refractory status epilepticus. Similarly, there appears to be a lack of systematic reviews addressing this topic in the literature. Therefore, this systematic review aims to explore the effectiveness and safety of ketamine for terminating super-refractory status epilepticus.
METHODS
We conducted a systematic search on PubMed, EMBASE, and Web of Science databases. Manuscripts unrelated to the research on super-refractory status epilepticus were excluded, as were manuscripts published in non-English languages. The quality assessment and risk of bias were evaluated using the MINORS criteria. Data extraction was limited to qualitative synthesis due to the unsuitability of the data for meta-analysis.
RESULTS
Out of 782 studies retrieved from electronic databases, 11 met the inclusion criteria. Among them, 10 studies were retrospective, and 1 study was prospective. Patient data for inclusion were sourced from the case registries of the researchers' respective hospitals. Across all included studies, the administration of ketamine significantly reduced the duration of status epilepticus and demonstrated higher safety compared to patients not receiving ketamine treatment for super-refractory status epilepticus. Additionally, early administration of ketamine correlated with improved treatment outcomes. The risk of bias across all studies was deemed low.
CONCLUSION
This systematic review suggests that ketamine may be a feasible treatment option for super-refractory status epilepticus. However, given the critical nature of super-refractory status epilepticus, clinicians should prioritize its termination over evaluating the efficacy of specific medications, ensuring patient safety remains paramount. If feasible in real-world medical settings, future research should focus on designing randomized controlled trials to observe the specific efficacy and mechanisms of ketamine. Careful validation is necessary before considering ketamine as a first-line treatment for super-refractory status epilepticus.
PubMed: 38782798
DOI: 10.1007/s00415-024-12453-7 -
Epilepsy & Behavior Reports 2024Exercise interventions in epilepsy have been shown to improve seizure frequency, physical capacity, quality of life, mood, and cognitive functioning. However, the... (Review)
Review
Exercise interventions in epilepsy have been shown to improve seizure frequency, physical capacity, quality of life, mood, and cognitive functioning. However, the effectiveness of exercise in improving sleep in epilepsy is less clear. The purpose of this report is to identify the published literature regarding exercise interventions in people with epilepsy to determine 1) what proportion of published clinical trials assess sleep as an outcome, and 2) what benefits of exercise interventions on sleep have been observed. We searched the PubMed, PsycINFO, and SCOPUS electronic databases using the search terms "epilepsy AND [exercise OR physical activity]" and identified 23 articles reporting on 18 unique clinical trials. Nine studies were conducted in adults, five in children, and four in adults and children with active seizures, controlled seizures, or both. Exercise modalities included aerobic exercise, strength training, walking, and yoga, among others, and some also included educational and motivational components. Exercise effects on sleep were tested in four studies, two of which only included indirect measures of sleep- and rest-related fatigue, with mixed results. Of the two reports assessing sleep directly, one reported marginal non-significant improvements in subjective sleep quality and no improvements in objective sleep quality in children after twelve weeks of walking, and the other reported no benefits in subjective sleep quality after twelve weeks of combined aerobic, strength, and flexibility training in adults. Given the health benefits of sleep and detrimental effects of sleep deprivation in epilepsy, epilepsy researchers need to assess the effects of exercise interventions on sleep.
PubMed: 38779424
DOI: 10.1016/j.ebr.2024.100675