RESUMO
Posterior reversible encephalopathy syndrome (PRES) is an increasingly recognized clinical entity associated with a variety of medical conditions. It is commonly considered in the presentation of uncontrolled, severe hypertension. However, more recently, it has been described in the setting of blood transfusion, particularly in those with chronic anemia, even in the absence of severe hypertension. We describe a patient who presented to the emergency department 12 days after large blood transfusion for severe, chronic anemia with headache, vision loss, expressive aphasia and a change in mental status, with only mild blood pressure elevation, who was ultimately diagnosed with PRES and refractory non-convulsive status epilepticus. Emergency physicians are often the first to initiate blood transfusion for those with a low hemoglobin. Therefore, it is prudent to proceed with caution in transfusing those with chronic anemia. It is also important for the emergency physician to keep PRES on the differential for those presenting with a neurologic complaint after correction of their chronic anemia, even in the absence of severe hypertension.
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Síndrome da Leucoencefalopatia Posterior , Humanos , Síndrome da Leucoencefalopatia Posterior/etiologia , Síndrome da Leucoencefalopatia Posterior/diagnóstico , Reação Transfusional/diagnóstico , Reação Transfusional/complicações , Feminino , Estado Epiléptico/etiologia , Anemia/etiologia , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , MasculinoRESUMO
BACKGROUND: Approximately 10% to 20% of children with epilepsy experience status epilepticus (SE), and children with seizure clustering are at higher risk. Ketamine is growing in use for SE. This study examines the efficacy and safety of enteral ketamine in the treatment of convulsive status epilepticus (CSE) characterized by refractory seizure clusters and nonconvulsive status epilepticus (NCSE) in children with epilepsy. METHODS: Patient charts were reviewed retrospectively. Children with epilepsy aged one to 21 years presenting in SE and treated with enteral ketamine between September 1, 2021 and September 1, 2022 at a pediatric tertiary care center were identified. Resolution or reduction in seizure frequency within 48 hours, clinical presentation, endotracheal intubation, hospitalization duration, side effects, and readmission were assessed. RESULTS: Nine patients aged two to 21 years were identified. Six patients presented in CSE characterized by recurrent seizures, and three patients presented in NCSE. Five patients had genetic epilepsies, including PCDH19- and MECP2-related epilepsy. Seven patients had resolution or reduction in seizures within 48 hours of ketamine initiation. Two patients were intubated. Hospitalization duration ranged from one to 34 days. Three patients reported side effects. Three patient readmissions with early ketamine treatment had equal or shorter hospitalizations. CONCLUSIONS: Enteral ketamine may prove an effective, well-tolerated option for treatment of convulsive and nonconvulsive SE in children with epilepsy, including genetic epilepsies, and may prevent intubation and shorten hospitalization time.
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Ketamina , Estado Epiléptico , Humanos , Ketamina/administração & dosagem , Estado Epiléptico/tratamento farmacológico , Estado Epiléptico/etiologia , Criança , Adolescente , Feminino , Masculino , Pré-Escolar , Estudos Retrospectivos , Adulto Jovem , Epilepsia/tratamento farmacológico , Lactente , AdultoRESUMO
In the acute care setting, the two most common causes of giant upright T waves include hyperkalemia and the very early phase of acute myocardial infarction (MI). The former is characterized by narrow based and peaked T waves. The giant T waves of early MI, also called "hyperacute T waves," are usually more broad-based. The general recommendation is to consider hyperacute T waves a form of occlusion MI, and to proceed with emergent cardiac catheterization and revascularization. In this report, we present the case of a young man with cocaine toxicity and status epilepticus where the initial electrocardiogram (ECG) demonstrated giant T waves. Both hyperkalemia and coronary occlusion were ruled out. Within a few hours, the ECG spontaneously normalized. Review of the literature revealed that although uncommon, acute cerebral events including seizures can cause transient giant T waves. When giant T waves are noted in association with a cerebral event, emergent cardiac catheterization may not be warranted.
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Eletrocardiografia , Humanos , Masculino , Adulto , Transtornos Relacionados ao Uso de Cocaína/complicações , Estado Epiléptico/etiologia , Infarto do Miocárdio/diagnóstico , Infarto do Miocárdio/etiologiaRESUMO
We present a case of a newborn with a prenatally discovered cardiac rhabdomyoma leading to early genetic diagnosis of tuberous sclerosis complex (TSC). This early diagnosis prompted a presymptomatic electroencephalography (EEG) that revealed subclinical seizures meeting the definition for status epilepticus on day 1 of life. Antiseizure medications (ASMs), including vigabatrin, were started. The EPISTOP and PREVeNT trials demonstrated that early life initiation of vigabatrin may reduce the degree of refractory epilepsy and epileptic spasms (ES) in this population (TSC). Although neonatal seizures are a known entity in TSC, continuous neonatal EEG monitoring is not standard at birth. This case supports early consideration for neonatal EEG monitoring to identify and treat neonatal seizures, reduce risk for infantile spasms, and potentially improve neurodevelopmental outcomes.
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Eletroencefalografia , Estado Epiléptico , Esclerose Tuberosa , Humanos , Esclerose Tuberosa/complicações , Esclerose Tuberosa/diagnóstico , Estado Epiléptico/tratamento farmacológico , Estado Epiléptico/etiologia , Estado Epiléptico/diagnóstico , Recém-Nascido , Deficiências do Desenvolvimento/etiologia , Anticonvulsivantes , Feminino , Masculino , Vigabatrina/uso terapêutico , LactenteRESUMO
We aimed to provide a detailed phenotypic description of status epilepticus (SE) in a large cohort of patients with POLG disease and identify prognostic biomarkers to improve the management of this life-threatening condition. In a multinational, retrospective study with data on patients with POLG disease from seven European countries, we identified those who had SE. The age of SE onset, accompanying clinical, laboratory, imaging and genetic findings were analysed. One hundred and ninety-five patients with genetically confirmed POLG disease were recruited, of whom 67% (130/194) had epilepsy. SE was identified in 77% (97/126), with a median age of SE onset of 7 years. SE was the presenting symptom of the disease in 43% (40/93) of those with SE, while 57% (53/93) developed SE during the disease course. Convulsive SE was reported in 97% (91/94) followed by epilepsia partialis continua in 67% (56/84). Liver impairment 78% (74/95), ataxia 69% (60/87), stroke-like episodes 57% (50/88), were the major comorbidities. In the majority (66%; 57/86) with SE this became refractory or super-refractory. The presence of seizures was associated with significantly higher mortality compared to those without (P ≤ 0.001). The median time from SE debut to death was 5 months. SE is a major clinical feature of POLG disease in early and juvenile to adult-onset disease and can be the presenting feature or arise as part of a multisystem disease. It is associated with high morbidity and mortality, with the majority of patients with SE going on to develop refractory or super-refractory SE.
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DNA Polimerase gama , Estado Epiléptico , Humanos , Estado Epiléptico/etiologia , Estado Epiléptico/genética , Masculino , Feminino , Adulto , Adolescente , Adulto Jovem , Estudos Retrospectivos , Criança , Europa (Continente)/epidemiologia , DNA Polimerase gama/genética , Pré-Escolar , Pessoa de Meia-Idade , Lactente , Doenças Mitocondriais/genética , Doenças Mitocondriais/complicações , Doenças Mitocondriais/epidemiologia , Idade de InícioRESUMO
PURPOSE: New-onset refractory status epilepticus (NORSE) is defined as a state of prolonged seizure activity that does not improve despite the appropriate administration of medications, with underlying causes unknown after the initial diagnosis of status epilepticus. Because episodes of NORSE are accompanied by severe complications and a high risk of mortality, the prompt identification of the underlying cause is crucial for effective treatment and outcome prediction. This study assessed the relationship of NORSE etiologies with baseline clinical features in pediatric population. METHODS: Seventy-one pediatric patients, under 18 years of age at the initial diagnosis (4.50 ± 4.04, mean ± standard deviation), who experienced at least one episode of NORSE and underwent a comprehensive diagnostic evaluation between January 2005 and June 2020 at our center, were retrospectively selected. We reviewed clinical features at disease onset and long-term follow-up data. Uniform manifold approximation and projection (UMAP) was used to distinguish etiological clusters according to baseline clinical characteristics, and further analysis was performed based on underlying etiologies. RESULTS: Two distinct etiological groups-genetic and non-genetic-were identified based on the UMAP of clinical characteristics. Dravet syndrome (12/15, 80%) was more predominant in patients with a genetic diagnosis, whereas cryptogenic NORSE and encephalitis were prevalent in patients without a genetic diagnosis. The analysis of etiological categories revealed that age at the onset of status epilepticus (P=0.021) and progression to super refractory status epilepticus (SRSE) (P=0.038) were independently associated with differences in etiologies. CONCLUSION: Several clinical features in patients with NORSE, including the age of onset and the development of SRSE, can help identify underlying causes, which necessitate prompt and adequate treatment.
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Epilepsia Resistente a Medicamentos , Estado Epiléptico , Humanos , Estado Epiléptico/diagnóstico , Estado Epiléptico/etiologia , Masculino , Estudos Retrospectivos , Feminino , Pré-Escolar , Criança , Lactente , Epilepsia Resistente a Medicamentos/diagnóstico , Adolescente , Encefalite/diagnóstico , Encefalite/complicações , Epilepsias Mioclônicas/diagnóstico , Epilepsias Mioclônicas/fisiopatologiaRESUMO
PURPOSE: Status epilepticus (SE) represents a neurological emergency with significant morbidity and mortality. SE in patients with primary brain tumors received only limited attention to date; detailed analysis of treatment flow is lacking, especially as compared to other SE causes. This study aims to describe the frequency and treatment flow of tumor-related SE and compare it to other SE etiologies. METHODS: Retrospective cohort study based on an institutional SE registry (SERCH) comprising adult SE (excluding post-anoxic causes), treated between January 2013 and December 2022, comparing SE management, frequency of refractory SE, and clinical outcome, among four patients' groups stratified by SE etiology: Non-neoplastic, Gliomas, Brain metastases, Other brain tumors. RESULTS: We analyzed 961 episodes in 831 patients (Non-neoplastic: 649, Gliomas: 85, Metastases: 77, Other brain tumors: 20). Although tumor-patients presented more often with focal episodes and less consciousness impairment than non-neoplastic patients, administration of benzodiazepines as first-line treatment (>75% across all groups), and utilization of second-line ASM were similar across groups. Treatment adequacy was marginally higher in glioma patients compared to the non-neoplastic population (p: 0.049), while refractory SE was comparable in all groups (p: 0.269). No significant differences in clinical outcomes were observed (mortality: non-neoplastic (89/649, 13.7%), glioma (8/85, 9.4%), metastases (14/77, 18.2%), other tumors (5/20, 25.0%), p: 0.198; non-neoplastic vs. glioma, p: 0.271) CONCLUSION: Tumor-associated SE represents 1/5 of all SE episodes, and is managed similarly to other SE causes. Treatment responsiveness and short-term clinical outcomes also exhibit comparable results.
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Anticonvulsivantes , Neoplasias Encefálicas , Estado Epiléptico , Humanos , Estado Epiléptico/etiologia , Estado Epiléptico/tratamento farmacológico , Estado Epiléptico/terapia , Masculino , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/secundário , Neoplasias Encefálicas/terapia , Feminino , Pessoa de Meia-Idade , Estudos Retrospectivos , Idoso , Anticonvulsivantes/uso terapêutico , Adulto , Estudos de Coortes , Sistema de Registros/estatística & dados numéricos , Glioma/complicações , Glioma/terapiaRESUMO
PURPOSE: We aimed to describe risks of status epilepticus (SE) after different brain disorders in adults using population-wide register data. Our hypothesis was that SE would be more common in disorders with widespread pathology and that the risk would increase with disorder severity. METHODS: We analyzed five large datasets created from the Swedish National Patient Register, the Cause of Death Register, and national quality registers with adults in Sweden with brain infections, dementia, multiple sclerosis (MS), stroke, and traumatic brain injury (TBI). Risk factors were assessed using Cox regression. RESULTS: In adults with TBI, stroke, dementia, MS, or brain infections, the incidence rate of SE was highest in survivors of brain infections (64/100,000 person years) and stroke (64/100,000), followed by TBI (37/100,000), dementia (36/100,000), and MS (26/100,000). SE was considerably more common in patients with epilepsy after their brain disorder. Across all datasets severe disorder increased SE-risk. Herpes simplex encephalitis (HR 5.5 95 % CI: 2.6-12), progressive MS (HR 2.3, 95 % CI: 1.1-4.7), structural TBI (2.0, 95 % CI: 1.6-2.6), and intracerebral hemorrhage (HR 1.5, 95 % CI: 1.2-2.0) were the subtypes of brain disorders with the highest relative risk of SE. Having another CNS disorder increased SE-risk in TBI (HR 2.9, 95 % CI: 2.3-3.7), brain infections (HR 2.8, 95 % CI: 1.7-4.5), and dementia (HR 2.5, 95 % CI: 1.5-4.2). CONCLUSION: SE-risk increases with disorder severity and number of CNS comorbidities. These findings can guide treatment strategy by allowing identification of high-risk patients. Pathophysiological studies are needed to better understand remote symptomatic SE.
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Sistema de Registros , Estado Epiléptico , Humanos , Feminino , Masculino , Estado Epiléptico/epidemiologia , Estado Epiléptico/etiologia , Fatores de Risco , Pessoa de Meia-Idade , Adulto , Suécia/epidemiologia , Idoso , Estudos de Coortes , Encefalopatias/epidemiologia , Idoso de 80 Anos ou mais , Incidência , Adulto JovemRESUMO
FIRES and NORSE are clinical presentations of disease processes that, to date, remain unexplained without an established etiology in many cases. Neuroinflammation is thought to have paramount importance in the genesis of these conditions. We hereby report the clinical, EEG, brain MRI, and genetic findings of a nuclear family with recurrent febrile-related encephalopathy with refractory de novo Status Epilepticus. Whole-exome sequencing (WES) revealed a homozygous p.C105W pathogenic variant of FADD gene (FAS-associated protein with death domain, FADD), known to cause ultrarare forms of autosomal recessive immunodeficiency that could be associated with variable degrees of lymphoproliferation, cerebral atrophy, and cardiac abnormalities. The FADD-related conditions disrupt FAS-mediated apoptosis and can cause a clinical picture with the characteristics of FIRES. This observation is important because, on one hand, it increases the number of reported patients with FADD deficiency, showing that this disorder may present variable expressivity, and on the other hand, it demonstrates a genetic cause of FIRES involving a cell-mediated inflammation regulatory pathway. This finding supports early treatment with immunomodulatory therapy and could represent a new avenue of research in the field of new onset refractory status epilepticus and related conditions.
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Proteína de Domínio de Morte Associada a Fas , Humanos , Proteína de Domínio de Morte Associada a Fas/genética , Feminino , Masculino , Convulsões Febris/genética , Estado Epiléptico/genética , Estado Epiléptico/etiologia , Linhagem , Sequenciamento do Exoma , Febre/genética , Febre/complicações , Síndromes Epilépticas/genética , EletroencefalografiaRESUMO
INTRODUCTION: Maternal epilepsy is a critical condition that can significantly affect mothers and fetuses. Notably, the admission of a laboring mother with uncontrolled refractory status epilepticus (RSE) to the operating room presents a challenging scenario for anesthesiologists. THE MAIN SYMPTOMS OF THE PATIENT AND THE IMPORTANT CLINICAL FINDINGS: A 30-year-old primigravida was transferred to the operating room for an emergency cesarean section. Cesarean section was performed after a provisional diagnosis of preeclampsia was made. THE MAIN DIAGNOSES, THERAPEUTIC INTERVENTIONS, AND OUTCOMES: Cesarean section was performed under general anesthesia. During the postoperative period, the patient exhibited no seizure activity in the brain; however, she experienced mild cognitive dysfunction for up to 6 months postdelivery. The neonate were discharged without any complications. CONCLUSION: Inducing anesthesia in pregnant women with ongoing seizure activity are challenging; however, anesthesiologists provide judgment based on the balance between the safety of the mother and fetus and the balance between patient monitoring and the progression of anesthesia. This challenge can be addressed through multidisciplinary collaboration.
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Anestesia Geral , Cesárea , Estado Epiléptico , Humanos , Feminino , Cesárea/efeitos adversos , Adulto , Estado Epiléptico/etiologia , Gravidez , Anestesia Geral/métodos , Anestesia Geral/efeitos adversos , Complicações na Gravidez/cirurgia , Anestesia Obstétrica/métodosRESUMO
BACKGROUND: There are only six past reports of super-refractory status epilepticus induced by spinal anesthesia. None of those patients have died. Only < 15 mg of bupivacaine was administered to all six of them and to our case. Pathophysiology ensuing such cases remains unclear. CASE PRESENTATION: A 27 year old gravida 2, para 1, mother at 37 weeks of gestation came to the operating theater for an elective cesarean section. She had no significant medical history other than controlled hypothyroidism and one episode of food allergy. Her current pregnancy was uneventful. Her American Society of Anesthesiologists (ASA) grade was 2. She underwent spinal anesthesia and adequate anesthesia was achieved. After 5-7 min she developed a progressive myoclonus. After delivery of a healthy baby, she developed generalized tonic clonic seizures that continued despite the induction of general anesthesia. She had rhabdomyolysis, one brief cardiac arrest and resuscitation, followed by stress cardiomyopathy and central hyperthermia. She died on day four. There were no significant macroscopic or histopathological changes in her brain that explain her super refractory status epilepticus. Heavy bupivacaine samples of the same batch used for this patient were analyzed by two specialized laboratories. National Medicines Quality Assurance Laboratory of Sri Lanka reported that samples failed to confirm United States Pharmacopeia (USP) dextrose specifications and passed other tests. Subsequently, Therapeutic Goods Administration of Australia reported that the drug passed all standard USP quality tests applied to it. Nonetheless, they have detected an unidentified impurity in the medicine. CONCLUSIONS: After reviewing relevant literature, we believe that direct neurotoxicity by bupivacaine is the most probable cause of super-refractory status epilepticus. Super-refractory status epilepticus would have led to her other complications and death. We discuss probable patient factors that would have made her susceptible to neurotoxicity. The impurity in the drug detected by one laboratory also would have contributed to her status epilepticus. We propose several possible mechanisms that would have led to status epilepticus and her death. We discuss the factors that shall guide investigators on future such cases. We suggest ways to minimize similar future incidents. This is an idiosyncratic reaction as well.
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Raquianestesia , Cardiomiopatias , Hipertermia Induzida , Rabdomiólise , Estado Epiléptico , Humanos , Gravidez , Feminino , Adulto , Raquianestesia/efeitos adversos , Cesárea , Estado Epiléptico/etiologia , Estado Epiléptico/terapia , Bupivacaína/efeitos adversos , Cardiomiopatias/terapia , Rabdomiólise/terapiaRESUMO
A 69-year-old woman was admitted after a cardiac arrest. She developed status epilepticus and was later found to have variable morphologies of a "spiked helmet sign" (SHS) on ECGs in the setting of prolonged QT interval, raising the question of whether this sign is a manifestation of QT prolongation.
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Eletrocardiografia , Síndrome do QT Longo , Humanos , Feminino , Idoso , Síndrome do QT Longo/diagnóstico , Síndrome do QT Longo/fisiopatologia , Diagnóstico Diferencial , Estado Epiléptico/etiologia , Estado Epiléptico/diagnóstico , Parada Cardíaca/etiologiaRESUMO
OBJECTIVE: This study was undertaken to determine whether hippocampal T2 hyperintensity predicts sequelae of febrile status epilepticus, including hippocampal atrophy, sclerosis, and mesial temporal lobe epilepsy. METHODS: Acute magnetic resonance imaging (MRI) was obtained within a mean of 4.4 (SD = 5.5, median = 2.0) days after febrile status on >200 infants with follow-up MRI at approximately 1, 5, and 10 years. Hippocampal size, morphology, and T2 signal intensity were scored visually by neuroradiologists blinded to clinical details. Hippocampal volumetry provided quantitative measurement. Upon the occurrence of two or more unprovoked seizures, subjects were reassessed for epilepsy. Hippocampal volumes were normalized using total brain volumes. RESULTS: Fourteen of 22 subjects with acute hippocampal T2 hyperintensity returned for follow-up MRI, and 10 developed definite hippocampal sclerosis, which persisted through the 10-year follow-up. Hippocampi appearing normal initially remained normal on visual inspection. However, in subjects with normal-appearing hippocampi, volumetrics indicated that male, but not female, hippocampi were smaller than controls, but increasing hippocampal asymmetry was not seen following febrile status. Forty-four subjects developed epilepsy; six developed mesial temporal lobe epilepsy and, of the six, two had definite, two had equivocal, and two had no hippocampal sclerosis. Only one subject developed mesial temporal epilepsy without initial hyperintensity, and that subject had hippocampal malrotation. Ten-year cumulative incidence of all types of epilepsy, including mesial temporal epilepsy, was highest in subjects with initial T2 hyperintensity and lowest in those with normal signal and no other brain abnormalities. SIGNIFICANCE: Hippocampal T2 hyperintensity following febrile status epilepticus predicted hippocampal sclerosis and significant likelihood of mesial temporal lobe epilepsy. Normal hippocampal appearance in the acute postictal MRI was followed by maintained normal appearance, symmetric growth, and lower risk of epilepsy. Volumetric measurement detected mildly decreased hippocampal volume in males with febrile status.
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Epilepsia do Lobo Temporal , Hipocampo , Imageamento por Ressonância Magnética , Esclerose , Convulsões Febris , Estado Epiléptico , Humanos , Hipocampo/patologia , Hipocampo/diagnóstico por imagem , Epilepsia do Lobo Temporal/diagnóstico por imagem , Epilepsia do Lobo Temporal/patologia , Masculino , Feminino , Esclerose/patologia , Estado Epiléptico/diagnóstico por imagem , Estado Epiléptico/patologia , Estado Epiléptico/etiologia , Convulsões Febris/patologia , Convulsões Febris/diagnóstico por imagem , Lactente , Pré-Escolar , Criança , Seguimentos , Atrofia/patologia , Esclerose HipocampalRESUMO
Febrile infection-related epilepsy syndrome (FIRES) is a subset of new onset refractory status epilepticus (NORSE) that involves a febrile infection prior to the onset of the refractory status epilepticus. It is unclear whether FIRES and non-FIRES NORSE are distinct conditions. Here, we compare 34 patients with FIRES to 30 patients with non-FIRES NORSE for demographics, clinical features, neuroimaging, and outcomes. Because patients with FIRES were younger than patients with non-FIRES NORSE (median = 28 vs. 48 years old, p = .048) and more likely cryptogenic (odds ratio = 6.89), we next ran a regression analysis using age or etiology as a covariate. Respiratory and gastrointestinal prodromes occurred more frequently in FIRES patients, but no difference was found for non-infection-related prodromes. Status epilepticus subtype, cerebrospinal fluid (CSF) and magnetic resonance imaging findings, and outcomes were similar. However, FIRES cases were more frequently cryptogenic; had higher CSF interleukin 6, CSF macrophage inflammatory protein-1 alpha (MIP-1a), and serum chemokine ligand 2 (CCL2) levels; and received more antiseizure medications and immunotherapy. After controlling for age or etiology, no differences were observed in presenting symptoms and signs or inflammatory biomarkers, suggesting that FIRES and non-FIRES NORSE are very similar conditions.
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Febre , Estado Epiléptico , Humanos , Estado Epiléptico/etiologia , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Febre/etiologia , Febre/complicações , Adulto Jovem , Adolescente , Epilepsia Resistente a Medicamentos/etiologia , Criança , Convulsões Febris/etiologia , Eletroencefalografia , Idoso , Imageamento por Ressonância Magnética , Síndromes Epilépticas , Pré-EscolarRESUMO
Psychosis of epileptic origin can present a wide range of cognitive and affective symptoms and is often underrecognized. Usually occurring in the inter- and postictal phase, epileptic psychosis is mostly related to temporal lobe epilepsy. Here, we describe the clinical presentation and diagnostic workup including routine EEG recording and brain MRI of a 63-year-old woman expressing isolated nihilistic delusions comprising belief of being dead and denial of self-existence. EEG showed an ictal pattern fulfilling the Salzburg criteria of nonconvulsive status epilepticus and brain MRI revealed extensive peri-ictal hyperperfusion. Delusional symptoms and EEG abnormalities subsided after acute antiseizure treatment. Our case illustrates how nihilistic delusions can occur as a direct clinical correlate of seizure activity, thereby expanding the spectrum of ictal neuropsychiatric phenomena in temporal lobe epilepsy and highlighting the need to consider an epileptic origin in patients presenting with psychotic symptoms.
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Delusões , Eletroencefalografia , Estado Epiléptico , Humanos , Estado Epiléptico/fisiopatologia , Estado Epiléptico/etiologia , Feminino , Delusões/etiologia , Delusões/fisiopatologia , Pessoa de Meia-Idade , Imageamento por Ressonância Magnética , Transtornos Psicóticos/fisiopatologia , Transtornos Psicóticos/etiologia , Anticonvulsivantes/uso terapêuticoRESUMO
BACKGROUND Acute necrotizing encephalopathy of childhood (ANEC) is a rare form of acute encephalopathy in children that is characterized by rapid and progressive deterioration in the patient's clinical condition. Our aim in reporting the case is to highlight the importance of early diagnoses and aggressive early management of ANEC to achieve a better outcome. We report the case of a 5-year-old girl who presented with acute progressive encephalopathy with bilateral symmetrical thalamic involvement and brainstem lesion proceeded by a flu-like illness. CASE REPORT We report a 5-year-old girl who was previously healthy. She presented with rapid progressive encephalopathy and status epilepticus following a flu-like illness. Her magnetic resonance imaging (MRI) showed significance symmetrical bilateral thalamic enhancement with brainstem lesions, which is consistent with the diagnosis of ANEC of childhood, and her initial electroencephalogram (EEG) showed severe encephalopathy. Despite extensive management of ANEC, the patient showed a poor prognosis. CONCLUSIONS ANEC is a rare, specific, devastating condition in the pediatric population. Early diagnosis and aggressive management should be initiated immediately to avoid the high mortality associated with the condition. Furthermore, to highlight the importance of the clear history of the patient who presented with rapid and progressive deterioration in the level of consciousness and status epilepticus proceeded by viral illness, a rapid neuroradiological images such as brain MRI should be obtained to confirm the diagnosis of this rare condition. We also seek to increase awareness of this disorder among healthcare workers and general practitioners.
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Encefalopatias , Encefalite , Estado Epiléptico , Pré-Escolar , Feminino , Humanos , Doença Aguda , Encéfalo/patologia , Encefalopatias/diagnóstico por imagem , Encefalopatias/etiologia , Imageamento por Ressonância Magnética , Estado Epiléptico/etiologiaRESUMO
BACKGROUND: Malaria is an infectious malady caused by Plasmodium parasites, cerebral malaria standing out as one of its most severe complications. Clinical manifestation include elevated body temperature, loss of consciousness, and seizures. However, reports of cerebral malaria presenting as nonconvulsive status epilepticus are extremely rare. The case presented involves psychiatric symptoms, with the electroencephalogram indicated nonconvulsive status epilepticus associated with cerebral malaria. CASE PRESENTATION: A 53-year-old male, was urgently admitted, due to confusion and abnormal behaviour for 10 h. The patient returned to China after developing a fever while working in Tanzania two months ago. The blood smear revealed Plasmodium vivax and Plasmodium falciparum, and he was diagnosed with malaria. He recovered following anti-malarial treatment. After admission, the patient was confused, unable to communicate normally, and unwilling to cooperate with the physical examination. Plasmodium was not found in the blood smear, but the DNA sequence of P. falciparum was discovered using metagenomic next-generation sequencing of cerebrospinal fluid. Brain MRI revealed no significant abnormalities. Continuous electroencephalogram monitoring revealed that the patient had non-convulsive status epilepticus, which was treated with diazepam and levetiracetam. The patient had normal consciousness and behaviour. He received anti-malarial treatment for two weeks and fully recovered. CONCLUSIONS: This case demonstrates that nonconvulsive status epilepticus can be a manifestation of cerebral malaria. It is imperative for attending physicians to heighten vigilance when encountering patients with a history of travel to malaria-endemic regions or a prior malaria infection, especially in the presence of unusual clinical presentations.
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Antimaláricos , Malária Cerebral , Malária Falciparum , Plasmodium , Estado Epiléptico , Masculino , Humanos , Pessoa de Meia-Idade , Malária Cerebral/complicações , Malária Cerebral/diagnóstico , Malária Cerebral/tratamento farmacológico , Antimaláricos/uso terapêutico , Malária Falciparum/complicações , Malária Falciparum/diagnóstico , Malária Falciparum/tratamento farmacológico , Estado Epiléptico/diagnóstico , Estado Epiléptico/tratamento farmacológico , Estado Epiléptico/etiologiaRESUMO
To evaluate EEG monitoring during neonatal ECMO and to identify any correlations between seizure detection to abnormal neuroimaging. Eight-year, service evaluation of neonates who received at least one continuous EEG (cEEG) whilst on ECMO at Great Ormond Street Hospital. Pearson's chi-square test and multivariate logistic regression analysis were used to assess clinical and EEG variables association with seizures and neuroimaging findings. Fifty-seven neonates were studied; 57 cEEG recordings were reviewed. The incidence of seizures was 33% (19/57); of these 74% (14/19) were electrographic-only. The incidence of status epilepticus was 42%, (8/19 with 6 neonates having electrographic-only status and 2 electro-clinical status. Seizures were detected within an hour of recording in 84%, (16/19). The overall mortality rate was 39% (22/57). Seizure detection was strongly associated with female gender (OR 4.8, 95% CI: 1.1-20.4, p = 0.03), abnormal EEG background activity (OR 2.8, 95% CI: 1.1-7.4, p = 0.03) and abnormal EEG focal features (OR 23.6, 95% CI: 5.4-103.9, p = 0.001). There was a strong association between the presence of seizures and abnormal neuroimaging findings (OR 10.9, 95% CI: 2.8-41.9, p = 0.001). Neonates were highly likely to have abnormal neuroimaging findings in the presence of severely abnormal background EEG (OR 9.5, 95% CI 1.7-52.02, p = 0.01) and focal EEG abnormalities (OR 6.35, 95% CI 1.97-20.5, p = 0.002)Conclusion: The study highlights the importance of cEEG in neonates undergoing ECMO. An association between seizure detection and abnormal neuroimaging findings was described. What is Known: ⢠Patients on ECMO are at a higher risk of seiures. ⢠Continuous EEG monitoring is recommended by the ACNS for high risk and ECMO patients. What is New: ⢠In this cohort, neonates with sezirues were 11 times more likely of having abnromal neuroimaging findings. ⢠Neonates with burst suppressed or suppressed EEG background were 9.5 times more likely to have abnormal neuroimaging findings. What does this study add? ⢠This study reports a 33% incidence of neonatal seizures during ECMO. ⢠Neonates with seizures were 11 times more likely to have an abnormal brain scan. ⢠The study captures the real-time approach of EEG monitoring. ⢠Recommended cEEG monitoring should last at least 24 h for ECMO patients. ⢠This is the first study to assess this in neonates only.
Assuntos
Eletroencefalografia , Oxigenação por Membrana Extracorpórea , Convulsões , Humanos , Masculino , Recém-Nascido , Feminino , Eletroencefalografia/métodos , Convulsões/etiologia , Convulsões/diagnóstico , Estudos Retrospectivos , Incidência , Estado Epiléptico/etiologia , Estado Epiléptico/diagnóstico , Neuroimagem/métodos , Modelos LogísticosRESUMO
BACKGROUND: The occurrence of seizures following a stroke is a well-recognized complication associated with a significant increase in morbidity and mortality. Despite the numerous studies examining outcomes and risk factors related to post-stroke seizures (PSS), there remains a lack of clarity regarding the clinical characteristics, treatment, and PSS recurrence (PSSR) rates in patients experiencing their initial episode of PSS. PURPOSE: This study aimed to determine the risk factors for developing recurrent seizures after first PSS and their effects on functional outcomes and mortality. METHODS: All patients underwent an electroencephalography (EEG) and were monitored for a minimum of 24 months following the first PSS. The primary endpoint was the recurrence of seizures. Predictive factors for PSSR were determined by using the Cox-proportional hazards model, and the cumulative latency of recurrence at 90, 180, 360, and 720 days was estimated using Kaplan-Meier analysis. RESULTS: Seizure recurred in 36.8% (39/106). Significant association of PSSR was noted with female gender, use of older anti-seizure medications (ASMs) (p<0.001), EEG findings as focal slow wave activity (p<0.001), Ictal epileptiform abnormalities (p=0.015), status epilepticus (p=0.015), and with severe disability (p=0.008). However, multivariate cox-proportional hazards model showed significant association of female gender (HR=3.28; 95% CI: 1.42-7.58; p=0.006). Hazard ratio (HR) was increased with older ASMs use, focal aware seizure types, Ictal EAs, and periodic discharges on EEG; though, statistically significant. CONCLUSION: Factors such as the type of ASMs, EEG findings, and seizure type were significantly linked to PSSR. Female gender was the only independent predictor established. Additionally, significant functional decline was reported with recurrence.