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Epilepsy associated with high-titer glutamic acid decarboxylase 65 (GAD65) IgG is often refractory to immunotherapies and antiseizure medication. This study sought to determine the efficacy of vagus nerve stimulation (VNS) and surgical resection in patients with drug-resistant epilepsy associated with GAD65-IgG. We retrospectively identified 15 patients with drug-resistant epilepsy and high serum GAD65 antibody titers (>20 nmol·L-1) who underwent VNS implantation (n = 6), surgical resection (n = 7), or both (n = 2). A responder to VNS was defined as someone with a ≥50% reduction in seizure frequency, and a favorable surgical outcome was defined as Engel I-II. Of the eight patients who underwent VNS implantation, three (37.5%) were initially responders, but this was not sustained in two. Of the nine patients who underwent surgical resection, three (33.3%) had a favorable outcome; however, only one patient was seizure-free at last follow-up. Pathology was available in six patients, and only one had evidence of inflammation; this patient had seizure onset 1 year prior to surgery. Favorable seizure outcome correlated with older age at time of resective surgery, with a trend favoring later age of seizure onset. Taken together, surgical resection and VNS implantation may have limited efficacy in this patient population but can be considered in carefully selected cases.
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The objective of this study was to determine seizure control in women with epilepsy (WWE) undergoing assisted reproductive technology (ART). Through retrospective chart review, WWE undergoing ART were identified. Demographics and details regarding epilepsy type, seizure control, and ART procedures were extracted. Seizure frequency prior to and during ART were compared. We identified 12 WWE, who underwent 29 embryo transfers, resulting in 16 pregnancies and 10 live births. Nine women were seizure-free at least 2 years before fertility treatment, including three with resolved epilepsy. Seven were on antiseizure medications throughout fertility treatment and pregnancy, with only one on polytherapy. Eleven (all with controlled epilepsy or epilepsy in remission) remained seizure-free throughout fertility treatment. One woman with drug-resistant epilepsy continued to have seizures throughout fertility treatment and pregnancy without an exacerbation of seizure frequency. There was no increased seizure frequency associated with fertility treatment and subsequent pregnancy in this small series of WWE. Although this study was statistically underpowered, our results provide some preliminary evidence that ART might not pose a threat to seizure control, but larger, confirmatory studies are necessary.
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OBJECTIVE: Seizures are a common manifestation of paraneoplastic neurologic syndromes. The objective of this study was to describe the seizure characteristics and outcomes in patients with high-risk paraneoplastic autoantibodies (>70% cancer association) and to determine factors associated with ongoing seizures. METHODS: Patients from 2000 to 2020 with seizures and high-risk paraneoplastic autoantibodies were retrospectively identified. Factors associated with ongoing seizures at last follow-up were evaluated. RESULTS: Sixty patients were identified (34 males, median age at presentation = 52 years). ANNA1-IgG (Hu; n = 24, 39%), Ma2-IgG (n = 14, 23%), and CRMP5-IgG (CV2; n = 11, 18%) were the most common underlying antibodies. Seizures were the initial presenting symptom in 26 (43%), and malignancy was present in 38 (63%). Seizures persisted for >1 month in 83%, and 60% had ongoing seizures, with almost all patients (55/60, 92%) still being on antiseizure medications at last follow-up a median of 25 months after seizure onset. Ongoing seizures at last follow-up were associated with Ma2-IgG or ANNA1-IgG compared to other antibodies (p = .04), highest seizure frequency being at least daily (p = .0002), seizures on electroencephalogram (EEG; p = .03), and imaging evidence of limbic encephalitis (LE; p = .03). Death occurred in 48% throughout the course of follow-up, with a higher mortality in patients with LE than in those without LE (p = .04). Of 31 surviving patients at last follow-up, 55% continued to have intermittent seizures. SIGNIFICANCE: Seizures in the setting of high-risk paraneoplastic antibodies are frequently resistant to treatment. Ongoing seizures are associated with ANNA1-IgG and Ma2-IgG, high seizure frequency, and EEG and imaging abnormalities. Although a subset of patients may respond to immunotherapy and achieve seizure freedom, poor outcomes are frequently encountered. Death was more common among patients with LE.
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Encefalitis Límbica , Convulsiones , Masculino , Humanos , Persona de Mediana Edad , Estudios Retrospectivos , Convulsiones/etiología , Autoanticuerpos , Encefalitis Límbica/terapia , Encefalitis Límbica/diagnóstico , Inmunoglobulina GRESUMEN
PURPOSE: This study sought to evaluate the impact of surgical extent on seizure outcome in drug-resistant temporal lobe epilepsy (DR-TLE) with temporal encephaloceles (TE). METHODS: This was a single-institution retrospective study of patients who underwent surgery for DR-TLE with TE between January 2008 and December 2020. The impact of surgical extent on seizure outcome was evaluated. In a subset with dominant DR-TLE, the impact of surgical extent on neuropsychometric outcome was evaluated. RESULTS: Thirty-four patients were identified (female, 56%; median age at surgery, 43 years). TE were frequently overlooked on initial magnetic resonance imaging (MRI), with encephaloceles only detected after repeat or expert re-review of MRI, additional multi-modal imaging, or intra-operatively in 31 (91%). Sixteen (47%) underwent limited resections, including encephalocele resection only (n = 5) and encephalocele resection with more extensive temporal corticectomy sparing the amygdala and hippocampus (n = 11). The remainder (n = 18, 53%) underwent standard anterior temporal lobectomy and amygdalohippocampectomy (ATLAH). Limited resection was performed more frequently on the left (12/17 vs. 4/17, p = 0.015). Twenty-seven patients (79%) had a favourable outcome (Engel I/II), and 17 (50%) were seizure-free at the last follow-up (median seizure-free survival of 27.3 months). There was no statistically significant difference in seizure-free outcomes between limited resection and ATLAH. In dominant DR-TLE, verbal memory decline was more likely after ATLAH than limited resection (3/4 vs. 0/9, p = 0.014). CONCLUSION: Expert re-review of imaging and multi-modal advanced imaging improved TE identification. There was no statistical difference in seizure-free outcomes based on surgical extent. Preservation of verbal memory supports limited resection in dominant temporal cases.
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Epilepsia Refractaria , Epilepsia del Lóbulo Temporal , Humanos , Femenino , Adulto , Epilepsia del Lóbulo Temporal/complicaciones , Epilepsia del Lóbulo Temporal/diagnóstico por imagen , Epilepsia del Lóbulo Temporal/cirugía , Encefalocele/complicaciones , Encefalocele/diagnóstico por imagen , Encefalocele/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Convulsiones/cirugía , Lobectomía Temporal Anterior/métodos , Epilepsia Refractaria/diagnóstico por imagen , Epilepsia Refractaria/cirugía , Hipocampo/diagnóstico por imagen , Hipocampo/cirugía , Imagen por Resonancia MagnéticaRESUMEN
OBJECTIVE: To compare acute treatment responses and long-term outcome in leucine-rich glioma-inactivated 1 (LGI1) antibody encephalitis. METHODS: Retrospective case series of 118 patients with LGI1 antibody encephalitis evaluated at Mayo Clinic across all US sites from 1 May 2008 to 31 March 2019. Patient clinical data were identified and analysed through the neuroimmunology laboratory and electronic medical record. LGI1 antibody detection was by cell-based indirect immunofluorescence assay of serum, cerebrospinal fluid or both. Clinical outcomes were faciobrachial dystonic seizure (FBDS) resolution, modified Rankin Scale (mRS) score, Kokmen Short Test of Mental Status (STMS) score (0-38 point scale) and neuropsychometric testing results. RESULTS: Compared with intravenous immunoglobulin (IVIg) (n=21), patients treated with single-agent acute corticosteroids (intravenous, oral or both) (n=49) were more likely to experience resolution of FBDS (61% vs 7%, p=0.002) and improvements in mRS score (ΔmRS score 2 vs 0, p=0.008) and median Kokmen STMS scores (ΔKokmen STMS score 5 points vs 0 points, p=0.01). In 54 patients with long-term follow-up (≥2 years), the median mRS score was 1 (range 0-6) and the median Kokmen STMS score was 36 (range 24-38) after all combinations of immunotherapy. Neuropsychometric testing in 32 patients with long-term follow-up (≥2 years) demonstrated short-term memory impairments in 37%. CONCLUSIONS: Corticosteroids appeared more effective acutely than IVIg in improving LGI1 antibody encephalitis in this retrospective comparison of immunotherapies. While improvement with immunotherapy is typical and long-term outcome is favourable, short-term memory deficits are noted in approximately a third of the patients.
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Corticoesteroides/uso terapéutico , Autoanticuerpos , Enfermedades Autoinmunes/tratamiento farmacológico , Inmunoglobulinas Intravenosas/uso terapéutico , Péptidos y Proteínas de Señalización Intracelular/inmunología , Encefalitis Límbica/tratamiento farmacológico , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Enfermedades Autoinmunes/inmunología , Femenino , Humanos , Encefalitis Límbica/inmunología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Resultado del Tratamiento , Adulto JovenRESUMEN
The objective of this study was to describe serological association of musicogenic epilepsy and to evaluate clinical features and outcomes of seropositive cases. Through retrospective chart review, musicogenic epilepsy patients were identified. Among 16 musicogenic epilepsy patients, nine underwent autoantibody evaluations and all had high-titer glutamic acid decarboxylase 65-immunoglobulin G (GAD65-IgG; >20 nmol·L-1 , serum, normal ≤ .02 nmol·L-1 , eight women). Median GAD65-IgG serum titer was 294 nmol·L-1 (20.3-3005 nmol·L-1 ), and median cerebrospinal fluid titer (n = 4) was 14.7 nmol·L-1 . All patients had temporal lobe epilepsy, and bitemporal epileptiform abnormalities were common. Right temporal lobe seizures were most frequently captured when seizures were induced by music on electroencephalogram (3/4; 75%). Intravenous (IV) methylprednisolone and/or IV Ig (IVIG) was utilized in four patients, with one having greater than 50% reduction. Rituximab (n = 2) and mycophenolate (n = 1) were ineffective. Two patients underwent right temporal lobe resections but continued to have seizures. Vagus nerve stimulation was effective at reducing seizures in one patient by 50%, and an additional patient was seizure-free by avoiding provoking music. Right temporal lobe epilepsy was more common among patients with musicogenic epilepsy when compared to nonmusicogenic GAD65 epilepsies (n = 71, 89% vs. 47%, p = .03). GAD65-IgG should be tested in patients with musicogenic epilepsy, given implications for management and screening for comorbid autoimmune conditions.
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Autoanticuerpos/inmunología , Enfermedades Autoinmunes/inmunología , Epilepsia Refleja/inmunología , Adulto , Autoantígenos/inmunología , Autoinmunidad/inmunología , Epilepsia Refleja/fisiopatología , Epilepsia del Lóbulo Temporal/inmunología , Epilepsia del Lóbulo Temporal/fisiopatología , Femenino , Glutamato Descarboxilasa/inmunología , Humanos , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
OBJECTIVE: To evaluate the presence of tau deposition and pathologic features of chronic traumatic encephalopathy (CTE) in young adult patients treated with focal cortical resections for drug-resistant epilepsy. METHODS: Sixty consecutive patients who had undergone surgical treatment for drug-resistant focal epilepsy between 18 and 45 years of age were identified (2010-2017). Medical records were reviewed to determine clinical factors, including history of head trauma, age at seizure onset, age at surgical resection, seizure type(s) and frequency, imaging findings, and surgical outcome. All formalin-fixed, paraffin-embedded blocks from the surgical specimens from each subject were sectioned and stained with hematoxylin and eosin and antibodies to tau (Thermo Fisher Scientific Clone AT8), and examined blindly for tau pathology, including lesions characteristic of CTE. RESULTS: The median age at resection was 29.5 years (range = 19-45). A history of head trauma was reported in 19 patients. Although none of the patients had pathological findings characteristic of CTE, 23 patients (38%) demonstrated tau-immunoreactive lesions, including neurites, neurofibrillary pretangles, neurofibrillary tangles, subpial tau, and/or glial tau. In 4 of the 23 patients (7% of the cohort; 17% of those with tau pathology), substantial tau burden was identified. Three of these 4 patients had no significant history of head trauma; 1 patient had multiple sports-related concussions. No specific clinical factors correlated with the presence of tau pathology. SIGNIFICANCE: Tau-immunoreactive lesions were found in 38% of 60 patients who underwent a focal cortical resection for drug-resistant focal epilepsy. Diagnostic features of CTE were not detected in any patient; however, the pathological evaluation for CTE was limited to a surgical specimen. The prominent and excessive tau deposition in 23 patients (38%) is abnormal in this age group and warrants further investigation.
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Encéfalo/patología , Epilepsia Refractaria/patología , Epilepsia Refractaria/cirugía , Epilepsias Parciales/patología , Epilepsias Parciales/cirugía , Proteínas tau/análisis , Adulto , Química Encefálica/fisiología , Humanos , Persona de Mediana Edad , Adulto JovenRESUMEN
OBJECTIVE: We aimed to determine the frequency of probable obstructive sleep apnea (pOSA) in refractory epilepsy monitoring unit inpatients and clinical features associated with pOSA, including risk for sudden unexpected death in epilepsy (SUDEP). METHODS: We prospectively recruited 49 consecutive adult patients admitted to the Mayo Clinic Epilepsy Monitoring Unit with focal, generalized, or unclassified epilepsy syndromes. pOSA was identified using oximetric oxyhemoglobin desaturation index (ODI) and the Sleep Apnea-Sleep Disorders Questionnaire (SA-SDQ) and STOP-BAG screening tools. Revised SUDEP Risk Inventory (rSUDEP-7) scores were calculated, and epilepsy patients with and without pOSA were compared with Wilcoxon signed-rank tests. Correlation and regression analyses were utilized to determine relationships between pOSA and rSUDEP-7 scores. RESULTS: Thirty-five percent of patients had pOSA, with a mean ODI of 11.3 ± 5.1/h (range = 5.1-22.8). Patients with pOSA were older and heavier, and more frequently had a focal epilepsy syndrome and longer epilepsy duration, with higher SA-SDQ and STOP-BAG scores (all P < 0.05). Median rSUDEP-7 score was 3 ± 1.4 (range = 0-6). Higher rSUDEP-7 scores were positively correlated with higher ODI (P = 0.036). rSUDEP-7 score ≥ 5 was associated with pOSA by ODI, SA-SDQ, and STOP-BAG questionnaire criteria (P < 0.05). SIGNIFICANCE: Our pilot study identified a high frequency of pOSA in refractory epilepsy monitoring patients, finding that pOSA patients were older and heavier, with higher screening symptoms for sleep apnea and more frequent focal seizures with a longer epilepsy duration. We also found a possible association between OSA and SUDEP risk. Identification and treatment of OSA in patients with epilepsy could conceivably provide a novel approach toward preventing the risk of SUDEP. Future studies with polysomnography are needed to confirm predictive features for OSA in epilepsy populations, and to determine whether OSA is associated with SUDEP risk.
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Muerte Súbita/epidemiología , Epilepsia , Apnea Obstructiva del Sueño/complicaciones , Adulto , Epilepsia/complicaciones , Epilepsia/epidemiología , Epilepsia/mortalidad , Femenino , Humanos , Masculino , Persona de Mediana Edad , Proyectos Piloto , Estudios Prospectivos , Encuestas y Cuestionarios , Adulto JovenRESUMEN
OBJECTIVE: The prognostic role of electroencephalography during and after targeted temperature management in postcardiac arrest patients, relatively to other predictors, is incompletely known. We assessed performances of electroencephalography during and after targeted temperature management toward good and poor outcomes, along with other recognized predictors. DESIGN: Cohort study (April 2009 to March 2016). SETTING: Two academic hospitals (Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland; Mayo Clinic, Rochester, MN). PATIENTS: Consecutive comatose adults admitted after cardiac arrest, identified through prospective registries. INTERVENTIONS: All patients were managed with targeted temperature management, receiving prespecified standardized clinical, neurophysiologic (particularly, electroencephalography during and after targeted temperature management), and biochemical evaluations. MEASUREMENTS AND MAIN RESULTS: We assessed electroencephalography variables (reactivity, continuity, epileptiform features, and prespecified "benign" or "highly malignant" patterns based on the American Clinical Neurophysiology Society nomenclature) and other clinical, neurophysiologic (somatosensory-evoked potential), and biochemical prognosticators. Good outcome (Cerebral Performance Categories 1 and 2) and mortality predictions at 3 months were calculated. Among 357 patients, early electroencephalography reactivity and continuity and flexor or better motor reaction had greater than 70% positive predictive value for good outcome; reactivity (80.4%; 95% CI, 75.9-84.4%) and motor response (80.1%; 95% CI, 75.6-84.1%) had highest accuracy. Early benign electroencephalography heralded good outcome in 86.2% (95% CI, 79.8-91.1%). False positive rates for mortality were less than 5% for epileptiform or nonreactive early electroencephalography, nonreactive late electroencephalography, absent somatosensory-evoked potential, absent pupillary or corneal reflexes, presence of myoclonus, and neuron-specific enolase greater than 75 µg/L; accuracy was highest for early electroencephalography reactivity (86.6%; 95% CI, 82.6-90.0). Early highly malignant electroencephalography had an false positive rate of 1.5% with accuracy of 85.7% (95% CI, 81.7-89.2%). CONCLUSIONS: This study provides class III evidence that electroencephalography reactivity predicts both poor and good outcomes, and motor reaction good outcome after cardiac arrest. Electroencephalography reactivity seems to be the best discriminator between good and poor outcomes. Standardized electroencephalography interpretation seems to predict both conditions during and after targeted temperature management.
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Coma/etiología , Electroencefalografía , Paro Cardíaco/complicaciones , Paro Cardíaco/fisiopatología , Anciano , Biomarcadores , Femenino , Paro Cardíaco/mortalidad , Paro Cardíaco/terapia , Humanos , Hipotermia Inducida , Masculino , Persona de Mediana Edad , Estudios ProspectivosRESUMEN
OBJECTIVE: To validate predictive models for neural antibody positivity and immunotherapy response in epilepsy. METHODS: We conducted a retrospective study of epilepsy cases at Mayo Clinic (Rochester-MN; Scottsdale-AZ, and Jacksonville-FL) in whom autoimmune encephalopathy/epilepsy/dementia autoantibody testing profiles were requested (06/30/2014-06/30/2016). An Antibody Prevalence in Epilepsy (APE) score, based on clinical characteristics, was assigned to each patient. Among patients who received immunotherapy, a Response to Immunotherapy in Epilepsy (RITE) score was assigned. Favorable seizure outcome was defined as >50% reduction of seizure frequency at the first follow-up. RESULTS: Serum and cerebrospinal fluid (CSF) from 1,736 patients were sent to the Mayo Clinic Neuroimmunology Laboratory for neural autoantibody evaluation. Three hundred eighty-seven of these patients met the diagnostic criteria for epilepsy. Central nervous system (CNS)-specific antibodies were detected in 44 patients. Certain clinical features such as new-onset epilepsy, autonomic dysfunction, viral prodrome, faciobrachial dystonic seizures/oral dyskinesia, inflammatory CSF profile, and mesial temporal magnetic resonance imaging (MRI) abnormalities had a significant association with positive antibody results. A significantly higher proportion of antibody-positive patients had an APE score ≥4 (97.7% vs. 21.6%, p < 0.01). Sensitivity and specificity of an APE score ≥4 to predict presence of specific neural auto-antibody were 97.7% and 77.9%, respectively. In the subset of patients who received immunotherapy (77), autonomic dysfunction, faciobrachial dystonic seizures/oral dyskinesia, early initiation of immunotherapy, and presence of antibodies targeting plasma membrane proteins (cell-surface antigens) were associated with favorable seizure outcome. Sensitivity and specificity of a RITE score ≥7 to predict favorable seizure outcome were 87.5% and 83.8%, respectively. SIGNIFICANCE: APE and RITE scores can aid diagnosis, treatment, and prognostication of autoimmune epilepsy. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
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Autoanticuerpos/líquido cefalorraquídeo , Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/inmunología , Encefalopatías/diagnóstico , Encefalopatías/inmunología , Sistema Nervioso Central/inmunología , Demencia/diagnóstico , Demencia/inmunología , Epilepsia/diagnóstico , Epilepsia/inmunología , Inmunoterapia , Neuronas/inmunología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Enfermedades Autoinmunes/terapia , Encefalopatías/terapia , Niño , Preescolar , Demencia/terapia , Epilepsia/terapia , Femenino , Humanos , Lactante , Masculino , Persona de Mediana Edad , Examen Neurológico , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Resultado del Tratamiento , Adulto JovenRESUMEN
Electroencephalography in the setting of hypothermia and anoxia has been studied in humans since the 1950s. Specific patterns after cardiac arrest have been associated with prognosis since the 1960s, with several prognostic rating scales developed in the second half of the twentieth century. In 2002, two pivotal clinical trials were published, demonstrating improved neurologic outcomes in patients treated with therapeutic hypothermia (TH) after cardiac arrest of shockable rhythms. In the following years, TH became the standard of care in these patients. During the same time period, the use of continuous EEG monitoring in critically ill patients increased, which led to the recognition of subclinical seizures occurring in patients after cardiac arrest. As a result of these changes, greater amounts of EEG data are being collected, and the significance of specific patterns is being re-explored. We review the current role of EEG for the identification of seizures and the estimation of prognosis after cardiac resuscitation.
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Electroencefalografía/métodos , Paro Cardíaco/terapia , Hipotermia Inducida/métodos , Convulsiones/diagnóstico , Sobrevivientes , Paro Cardíaco/complicaciones , Humanos , Convulsiones/etiologíaRESUMEN
Herein we describe an episode of focal varicella-zoster virus (VZV) encephalitis in a healthy young man with neither rash nor radicular pain. The symptoms began with headaches and seizures, after which magnetic resonance imaging detected a single hyperintense lesion in the left temporal lobe. Because of the provisional diagnosis of a brain tumor, the lesion was excised and submitted for pathological examination. No tumor was found. But the tissue immunostained positively for VZV antigens, and wild-type VZV sequences were detected. In short, this case represents VZV reactivation, most likely in the trigeminal ganglion, in the absence of clinical herpes zoster.
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Encefalitis por Varicela Zóster/diagnóstico , Encefalitis por Varicela Zóster/patología , Herpesvirus Humano 3/inmunología , Herpesvirus Humano 3/aislamiento & purificación , Activación Viral , Encéfalo/diagnóstico por imagen , Encéfalo/patología , ADN Viral/genética , ADN Viral/aislamiento & purificación , Encefalitis por Varicela Zóster/fisiopatología , Exantema/patología , Humanos , Inmunohistoquímica , Imagen por Resonancia Magnética , Masculino , Dolor/fisiopatología , Radiografía , Convulsiones/diagnóstico , Convulsiones/etiología , Adulto JovenRESUMEN
With the discovery of an expanding number of neural autoantibodies, autoimmune etiologies of seizures have been increasingly recognized. Clinical phenotypes have been identified in association with specific underlying antibodies, allowing an earlier diagnosis. These phenotypes include faciobrachial dystonic seizures with LGI1 encephalitis, neuropsychiatric presentations associated with movement disorders and seizures in NMDA-receptor encephalitis, and chronic temporal lobe epilepsy in GAD65 neurologic autoimmunity. Prompt recognition of these disorders is important, as some of them are highly responsive to immunotherapy. The response to immunotherapy is highest in patients with encephalitis secondary to antibodies targeting cell surface synaptic antigens. However, the response is less effective in conditions involving antibodies binding intracellular antigens or in Rasmussen syndrome, which are predominantly mediated by cytotoxic T-cell processes that are associated with irreversible cellular destruction. Autoimmune encephalitides also may have a paraneoplastic etiology, further emphasizing the importance of recognizing these disorders. Finally, autoimmune processes and responses to novel immunotherapies have been reported in new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES), warranting their inclusion in any current review of autoimmune-associated seizure disorders.
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Convulsiones , Humanos , Convulsiones/etiología , Convulsiones/inmunología , Convulsiones/fisiopatología , Enfermedades Autoinmunes/inmunología , Enfermedades Autoinmunes/complicaciones , Enfermedades Autoinmunes/fisiopatología , Encefalitis/inmunología , Encefalitis/complicaciones , Encefalitis/fisiopatología , Autoanticuerpos/inmunología , Enfermedades Autoinmunes del Sistema Nervioso/inmunología , Enfermedades Autoinmunes del Sistema Nervioso/fisiopatología , Enfermedades Autoinmunes del Sistema Nervioso/diagnóstico , Enfermedades Autoinmunes del Sistema Nervioso/complicaciones , Inmunoterapia/métodosRESUMEN
Objectives: To study the frequency, causes, and consequences of seizure-related falls and near falls in LGI1-IgG autoimmune encephalitis. Methods: We retrospectively reviewed 136 patients seen at Mayo Clinic with (1) LGI1-IgG seropositivity, (2) clinical phenotypes compatible with LGI1-IgG autoimmune encephalitis, and (3) falls or near falls related to seizures. The clinical documentation, MRI, and EEG data were collected and reviewed. Results: In this cohort of 136 patients, 27% (n = 36) had falls or near falls related to seizures. The median age was 67 years (range 49-86 years) and 23/36 (64%) were male. Facio-brachio-crural dystonic seizures (21/36, 58%) and drop attacks (9/36, 25%) were the most common causes. Seizure-related falls resulted in injuries in 18/30 (60%), ranging from skin lacerations, joint dislocations, bone fractures to life-threatening intracranial hemorrhage. The injuries occurred most with drop attacks 8/9 (89%). Seizure-related falls or near falls resolved with immunotherapy in 24/32 (75%) whereas the responsiveness to anti-seizure medication alone was poor (4/32, 13%). Discussion: Our study demonstrates that seizure-related falls and near falls are common in LGI1-IgG autoimmune encephalitis. Early diagnosis, prompt immunotherapy initiation, and proper counseling are key to improving functional outcomes and preventing secondary injuries.
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BACKGROUND AND OBJECTIVES: Patients with ongoing seizures are usually not allowed to drive. The prognosis for seizure freedom is favorable in patients with autoimmune encephalitis (AIE) with antibodies against NMDA receptor (NMDAR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (CASPR2), and the gamma-aminobutyric-acid B receptor (GABABR). We hypothesized that after a seizure-free period of 3 months, patients with AIE have a seizure recurrence risk of <20% during the subsequent 12 months. This would render them eligible for noncommercial driving according to driving regulations in several countries. METHODS: This retrospective multicenter cohort study analyzed follow-up data from patients aged 15 years or older with seizures resulting from NMDAR-, LGI1-, CASPR2-, or GABABR-AIE, who had been seizure-free for ≥3 months. We used Kaplan-Meier (KM) estimates for the seizure recurrence risk at 12 months for each antibody group and tested for the effects of potential covariates with regression models. RESULTS: We included 383 patients with NMDAR-, 440 with LGI1-, 114 with CASPR2-, and 44 with GABABR-AIE from 14 international centers. After being seizure-free for 3 months after an initial seizure period, we calculated the probability of remaining seizure-free for another 12 months (KM estimate) as 0.89 (95% confidence interval [CI] 0.85-0.92) for NMDAR, 0.84 (CI 0.80-0.88) for LGI1, 0.82 (CI 0.75-0.90) for CASPR2, and 0.76 (CI 0.62-0.93) for GABABR. DISCUSSION: Taking a <20% recurrence risk within 12 months as sufficient, patients with NMDAR-AIE and LGI1-AIE could be considered eligible for noncommercial driving after having been seizure-free for 3 months.
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Autoanticuerpos , Encefalitis , Péptidos y Proteínas de Señalización Intracelular , Proteínas de la Membrana , Proteínas del Tejido Nervioso , Receptores de GABA-B , Recurrencia , Humanos , Femenino , Masculino , Adulto , Péptidos y Proteínas de Señalización Intracelular/inmunología , Autoanticuerpos/sangre , Persona de Mediana Edad , Encefalitis/inmunología , Estudios Retrospectivos , Receptores de GABA-B/inmunología , Proteínas del Tejido Nervioso/inmunología , Adulto Joven , Proteínas de la Membrana/inmunología , Receptores de N-Metil-D-Aspartato/inmunología , Convulsiones/etiología , Convulsiones/inmunología , Enfermedad de Hashimoto/inmunología , Enfermedad de Hashimoto/sangre , Anciano , Adolescente , Estudios de Seguimiento , Proteínas/inmunología , Estudios de CohortesRESUMEN
PURPOSE: The objective of this study was to describe the sEEG-defined seizure onset zone (SOZ), seizure semiology, presurgical evaluations, surgical intervention and outcome in patients with midline onset noninvasive phase I monitoring. METHODS: A single center sEEG database was reviewed to identify patients with seizures onset predominantly involving midline electrodes (FZ, CZ, PZ, OZ) on scalp EEG. Data abstracted included clinical factors, seizure semiology graded into lobar segmentation, imaging and electrographic findings, sEEG plan, interventions, and outcome. RESULTS: Twelve patients were identified (8 males, median age of sEEG 28 years) out of 100 cases of sEEG performed from January 2015-September 2019. "Frontal lobe" seizure semiology was the most common. sEEG-defined SOZ were frontal (5), diffuse (1), multifocal (1), frontal and insular (1), frontal and cingulate (1), insular (1), cingulate (1), and mesial temporal (1). CZ and/or FZ scalp EEG changes were present for all patients with SOZ involving the frontal, cingulate, and insular regions. PZ/OZ scalp involvement was present in one patient with mesial temporal SOZ. Four patients underwent a definitive resective or ablative surgery, and the remaining patients underwent a palliative intervention. Of those with follow-up information available, 8/11 had seizure reduction by ≥ 50%, including 4 with an Engel I outcome. No clinical factors were associated with outcome. CONCLUSIONS: SOZ for midline onset seizures from noninvasive phase I monitoring was most commonly in the frontal, cingulate, and insular regions. A complex cortical network between these regions may explain overlap in semiology and scalp EEG findings. While the number rendered seizure-free was limited, a significant proportion experienced a reasonably favorable outcome justifying use of sEEG to identify surgical options in these patients.
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Epilepsia Refractaria , Cuero Cabelludo , Masculino , Humanos , Adulto , Epilepsia Refractaria/cirugía , Electroencefalografía/métodos , Convulsiones/diagnóstico por imagen , Convulsiones/cirugía , Electrodos Implantados , Imagen por Resonancia MagnéticaRESUMEN
OBJECTIVE: Intracranial hemorrhage (ICH) is a known complication during stereo-electroencephalography (sEEG) however true rates remain unknown. We provide a comprehensive review of ICH during sEEG regardless of clinical symptoms. Secondly, we analyzed sEEG recordings to identify electrographic correlates of ICH. METHODS: This is a retrospective study of patients undergoing sEEG between January 2016 and April 2022 at the Mayo Clinic in Rochester. We reviewed medical records and imaging studies to identify ICH. We analyzed ICH by type, electrode trajectories, timing, sEEG findings and outcomes. RESULTS: There were a total of 201 sEEG implants, of which 23 (11%) cases or 0.9% electrodes implanted had evidence of ICH. The majority of affected patients (82%) were either asymptomatic or had mild clinical neurological manifestations. In 90% of patients who proceeded with surgical treatments, outcomes were favorable. The most common sEEG finding in contacts in proximity of ICH was either focal slowing with interictal discharges or focal electrographic seizures. CONCLUSIONS: ICH associated with sEEG is likely under-reported in literature. We present electroencephalographic correlates of ICH that may aid identification of ICH in the course of performing sEEG monitoring. SIGNIFICANCE: Our data provides clinically relevant information on potential risks and outcomes of ICH. Furthermore, our findings aid identification of ICH during sEEG.
Asunto(s)
Epilepsia Refractaria , Electroencefalografía , Humanos , Estudios Retrospectivos , Electrodos Implantados , Electroencefalografía/métodos , Convulsiones/cirugía , Técnicas Estereotáxicas , Hemorragias Intracraneales/diagnóstico por imagen , Hemorragias Intracraneales/etiología , Epilepsia Refractaria/cirugíaRESUMEN
Cerebral oximetry has not been explored in patients experiencing seizures in the epilepsy monitoring unit (EMU). The purpose of our study was to evaluate the feasibility of periictal measurement of cerebral oxygenation using noninvasive cerebral tissue oximetry and to determine whether there was evidence of cerebral hypoxemia during generalized seizures. Cerebral oxygen saturation findings were subsequently correlated with sudden unexpected death in epilepsy (SUDEP) risk factors. We prospectively evaluated six patients admitted to our EMU with histories of generalized tonic-clonic seizures (GTCS) with prolonged scalp electroencephalography (EEG) and two regional cerebral oxygen saturation (rSO(2)) sensors. Minimum rSO(2) values were recorded in the 5 min preceding seizure onset, during the seizure, and in the 5 min following seizure offset. SUDEP risk was assessed using the SUDEP-7 Inventory. Cerebral oximetry was well tolerated, with a mean duration of rSO(2) monitoring of 81.1 h. Cerebral oxygen saturation data were available from at least one sensor in 9 (90%) of 10 seizures; only 6 (60%) of 10 seizures had useable periictal digital pulse oximetry data. GTCS were associated with significantly lower minimum ictal (p = 0.003) and postictal (p = 0.004) %rSO(2) values than the minimum preictal value. Patients with at least one seizure with a %rSO(2) decrease of ≥20% tended to have higher SUDEP-7 Inventory scores (mean SUDEP-7 Inventory score 7 ± 2.8) versus patients without recorded desaturations (4.3 ± 0.5, p = 0.08). Larger studies are needed to determine the value of cerebral oximetry in the identification of patients at risk of SUDEP.
Asunto(s)
Muerte Súbita/etiología , Epilepsia/complicaciones , Epilepsia/mortalidad , Hipoxia Encefálica/epidemiología , Adulto , Factores de Edad , Estudios de Cohortes , Electrocardiografía , Electroencefalografía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Monitoreo Fisiológico , Oximetría/métodos , Estudios Retrospectivos , Factores Sexuales , Análisis de Supervivencia , Adulto JovenRESUMEN
SUMMARY: Sentinel epileptiform discharges (SEDs) are epileptiform transients preceding the onset of a focal seizure seen on scalp EEG. Despite their potential localizing value, formal study of SED has been limited. The authors report a patient with MRI-negative focal-onset epilepsy whose seizures on scalp and intracranial EEG were always preceded by SED. Although the location and morphology of the SED was invariable, the seizures after the discharge were of two clearly distinct types, each with different semiology and region of spread on intracranial EEG. This suggests that the SED played a role in activating two distinct seizure networks. A right temporal lobectomy with amygdalohippocampectomy was performed. The resection included both the region of the SED as well as the seizure-onset zone of the more common seizure type, achieving seizure freedom at 3 years after surgery. Further research exploring whether the localization of SED is a reliable indicator of the seizure-onset zone could aid surgical planning in patients whose seizures are preceded by SED.