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BACKGROUND: The analysis of EEG demands expertise and keen observation to distinguish epileptiform discharges from benign epileptiform variants (BEVs), a frequent source of erroneous interpretation. The prevalence of BEVs varies based on geographical, racial, and ethnic characteristics. However, most data on BEVs originates from Western populations, and additional studies on different cohorts would enrich the existing literature. METHODS: We reviewed EEGs from our institutional database to study the prevalence of benign epileptiform variants and analyzed their frequency, topography, and other characteristics. Additionally, we investigated the co-existence of epileptiform discharges with BEVs. RESULTS: We identified 296 patients with BEVs after reviewing 3000 EEGs (9.9%). The most common BEV was small sharp spikes (SSS), observed in 114 patients (3.8%). Wicket waves, 6 Hz spike and slow wave, 14 and 6 Hz positive bursts, and Rhythmic Temporal Theta of Drowsiness (RTTD) were identified in 67 (2.2%), 40 (1.3%), 39 (1.3%), and 35 (1.16%) patients, respectively and one patient with Subclinical Rhythmic EEG Discharges in Adults (SREDA). Additionally, we observed the co-existence of epileptiform discharges with BEVs, most commonly with SSS (27.8%). CONCLUSIONS: The present study is a large study with 3000 EEGs to describe the BEV characteristics. BEVs were seen in 9.9% of patients, BSSS being the most common. There were minor differences in frequency, gender or age distribution compared to existing literature. We demonstrated the co-existence of epileptiform discharges. Morphological characteristics remain the cornerstone in recognising BEVs. EEG readers need to be aware of features of BEVs to avoid wrongly interpretation.
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Electroencefalografía , Epilepsia , Humanos , Electroencefalografía/métodos , Femenino , Masculino , Adulto , Adulto Joven , Adolescente , Niño , Epilepsia/fisiopatología , Epilepsia/diagnóstico , Persona de Mediana Edad , Preescolar , Lactante , Estudios Retrospectivos , Anciano , Encéfalo/fisiopatología , Ondas Encefálicas/fisiologíaRESUMEN
BACKGROUND: Psychotherapy for people with acquired brain injury (ABI) is considered to be an important component of a holistic neuropsychological rehabilitation approach. This helps in making sense of the loss of the sense of self they experience. Gender, premorbid personality, and socio-cultural discourses guide this process of understanding. Narrative formulation takes these considerations into account and, thus, can be used for formulating therapeutic plans. AIM: To present a case report which highlights the use of narrative case formulation to understand the psychological, social, and cultural factors forming the dominant discourse of a woman with ABI. METHODS: Ms. VA, a 43-year-old female, presented herself with a diagnosis of hypoxic ischemic encephalopathy with small chronic infarcts with gliosis in the bilateral cerebellar hemisphere, myoclonic seizures, mild cognitive impairment, depression, generalized dystonia, and bronchial asthma. Along with neuropsychological rehabilitation and cognitive retraining, 25 sessions of psychotherapy using narrative formulation were performed. RESULTS: Following the therapy, microgains such as a developing strong therapeutic relationship, accommodating vulnerability in her narrative, and finding moments of independence and assertion within the constraints of ABI were observed. Acceptance of her current predicament vis-à-vis her lost self and finding meaning in her new self were facilitated. CONCLUSION: There is paucity of research detailing psychotherapeutic management of ABI, especially in India. Psychotherapy, particularly using narrative formulation, can be helpful in understanding the intersections of gender role and expectations, premorbid personality and ABI, and aiding the post-ABI rehabilitation and adjustment. Future work in this area can explore the socio-cultural aspects that play an important role in the therapy process.
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PURPOSE: Subacute sclerosing panencephalitis (SSPE) is a fatal neurological disorder resulting from persistent measles virus infection within the brain. Although neurological manifestations have been well-documented, the impact of SSPE on cardiac autonomic function, assessed through heart rate variability (HRV), remains understudied. METHODS: In this prospective single-center study conducted from January 2022 to March 2023 in Southern India, 30 consecutive SSPE patients and age- and sex-matched controls underwent electrocardiogram recordings for HRV analysis. Various HRV parameters were assessed, including time-domain metrics (SD of normal-to-normal intervals, root mean square of successive differences between normal heartbeats, percentage of successive normal interbeat intervals greater than 50 msec), SD1 and SD2 for Poincaré plot analysis, and frequency-domain metrics (low frequency %, high frequency %, low frequency:high frequency ratio). RESULTS: In the study, SSPE patients exhibited markedly reduced HRV. Specifically, SD of normal-to-normal intervals (P = 0.003), percentage of successive normal interbeat intervals greater than 50 msec (P = 0.03), and SD2 (P = 0.0016) were significantly lower compared with controls. Frequency-domain analysis did not reveal significant distinctions. Correlation analysis demonstrated a negative relationship between percentage of successive normal interbeat intervals greater than 50 msec and SSPE severity (r = -0.37, P = 0.042). Heart rate variability did not significantly differ between SSPE stages or with clinical variables. The interbeat interval range showed a narrower distribution in SSPE subjects. CONCLUSIONS: Our study highlights the clinical relevance of HRV analysis in SSPE and autonomic dysfunction throughout the disease course underscoring its importance in SSPE. This investigation provides valuable insights into cardiac autonomic dysfunction probably because of affliction of the central autonomic networks caused by the disease process and may be a contributing factor to mortality in SSPE.
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OBJECTIVE: To assess presentation of neurosyphilis with a focus on the psychiatric aspects. METHOD: File review of the cases with a positive cerebrospinal fluid venereal disease research laboratory test between 1999 to 2020. RESULTS: Medical records of 143 neurosyphilis patients were analysed. Hallucinations, delusions, and catatonia were the commonest psychiatric symptoms. Brain atrophy was the commonest neuroimaging finding. The number of neurosyphilis patients and the proportion with delirium or catatonia declined during the second decade (2010-2020). CONCLUSION: Atypical presentation of psychiatric symptoms around the fifth decade, with associated neurological symptoms or brain imaging changes, should prompt evaluation for neurosyphilis.
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Catatonia , Neurosífilis , Humanos , Catatonia/complicaciones , Atención Terciaria de Salud , Neurosífilis/complicaciones , Neurosífilis/diagnóstico , India/epidemiología , HospitalesRESUMEN
Objectives: Nearly 40% of pediatric epilepsies have a genetic basis. There is significant phenotypic and genotypic heterogeneity, especially in epilepsy syndromes caused by sodium channelopathies. Sodium channel subunit 1A (SCN1A)-related epilepsy represents the archetypical channel-associated gene that has been linked to a wide spectrum of epilepsies of varying severity. Subsequently, other sodium channels have also been implicated in epilepsy and other neurodevelopmental disorders. This study aims to describe the phenotypes in children with sodium channelopathies from a center in Southern India. Materials and Methods: This is a retrospective, descriptive, and single-center study. Out of 112 children presenting with epilepsy who underwent genetic testing between 2017 and 2021, 23 probands (M: F = 12:11) were identified to have clinically significant sodium channel mutations. Clinical presentation, electroencephalography, and imaging features of these patients were recorded. The utility of genetic test results (e.g., in planning another child, withdrawal of medications, or change in treatment) was also recorded. Results: Age at onset of seizures ranged from day 4 of life to 3.5 years. Clinical epilepsy syndromes included generalized epilepsy with febrile seizures plus (n = 3), Dravet syndrome (n = 5), early infantile epileptic encephalopathy (n = 7), drug-resistant epilepsy (n = 5), and epilepsy with associated movement disorders (n = 3). The most common type of seizure was focal with impaired awareness (n = 18, 78.2%), followed by myoclonic jerks (n = 8, 34.78%), epileptic spasms (n = 7, 30.4%), bilateral tonic-clonic seizures/generalized tonic-clonic seizures (n = 3, 13%), and atonic seizures (n = 5, 23.8%). In addition to epilepsy, other phenotypic features that were discerned were microcephaly (n = 1), cerebellar ataxia (n = 2), and chorea and dystonia (n = 1). Conclusion: Sodium channelopathies may present with seizure phenotypes that vary in severity. In addition to epilepsy, patients may also have other clinical features such as movement disorders. Early clinical diagnosis may aid in tailoring treatment for the given patient.
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Background: SH3TC2 variations lead to demyelinating recessive Charcot-Marie-Tooth (CMT) disease, which is commonly associated with early-onset scoliosis and cranial neuropathy. Data from Indian ethnicity is limited. Objective: We aim to report the characteristics of patients with SH3TC2-associated neuropathy from an Indian cohort. Patients and Methods: Data of five unrelated subjects with SH3TC2 variations were analyzed. Results: Clinical features included female predominance (n = 4), early-onset neuropathy (n = 2), pes cavus and hammer toes (n = 4), kyphoscoliosis (n = 1), impaired vision and hearing (n = 1), facial muscle weakness (n = 1), impaired kinaesthetic sense (n = 3), tremor (n = 2), and ataxia (n = 1). Four patients had the "CMT" phenotype, while one patient had Roussy-Levy syndrome. All had demyelinating electrophysiology with conduction velocities being "very slow" in one, "slow" in one, "mildly slow" in two, and "intermediate" in one patient. Brain stem auditory evoked potentials were universally abnormal though only one patient had symptomatic deafness. Seven variants were identified in SH3TC2 [homozygous = 3 (c.1412del, c.69del, c.3152G>A), heterozygous = 4 (c.1105C>T, c.3511C>T, c.2028G>C, c.254A>T)]. Except for c.3511C>T variant, the rest were novel. Three patients had additional variations in genes having pathobiological relevance in other CMTs or amyotrophic lateral sclerosis. Conclusion: We provide data on a cohort of patients of Indian origin with SH3TC2 variations and highlight differences from other cohorts. Though the majority were not symptomatic for hearing impairment, evoked potentials disclosed abnormalities in all. Further studies are required to establish the functional consequences of novel variants and their interacting molecular partners identified in the present study to strengthen their association with the phenotype.
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Enfermedad de Charcot-Marie-Tooth , Péptidos y Proteínas de Señalización Intracelular , Humanos , Femenino , Masculino , Péptidos y Proteínas de Señalización Intracelular/genética , Mutación , Fenotipo , Enfermedad de Charcot-Marie-Tooth/genética , Fenómenos ElectrofisiológicosRESUMEN
PURPOSE: Study assessed the role of MSI in predicting the post-operative seizure outcome. METHODS: This retrospective study included patients who underwent MEG and epilepsy surgery and had a minimum 6 months of postoperative follow-up. Concordance of MEG cluster with post-surgical resection cavity was classified as follows Class I) Concordant and region-specific, Class II) Concordant and region non-specific, Class III) Concordant lateralization only and Class IV) Discordant lateralization. The relationship between MSI concordance and post-operative seizure outcome was assessed. RESULTS: A total of 183 patients (M: F = 109:74) were included. The mean age at onset of seizures: 8.0 ± 6.4 years. The dipoles were frequent in 123(67.2 %). The primary cluster orientation was regular in 59 (32.2 %) and mixed in 124 (67.8 %) patients. Concordance between MEG and resection cavity: Class I - 124 (67.8 %), class II- 30 (16.4 %), class III- 23 (12.6 %), and class IV- 6 (3.3 %). The post-surgically mean duration of follow-up was 19.52 ± 11.27 months. At 6-month follow-up period, 144 (78.7 %) patients had complete seizure freedom out of which 106 (73.6 %) had class I concordance. Concordance of MEG with resection cavity was associated with a good outcome at 6 months (p = 0.001), 1 year (p = 0.001), 2 years (p = 0.0005) and 5 years (p = 0.04). MEG cluster characteristics had no association with seizure outcome except the strength of the cluster and outcome at 3 years (p = 0.02) follow-up. CONCLUSION: The study supports that the complete resection of the MEG cluster had high chance of seizure-freedom and can be used as a complementary noninvasive presurgical evaluation tool.
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Electroencefalografía , Magnetoencefalografía , Humanos , Lactante , Preescolar , Niño , Adolescente , Estudios Retrospectivos , Resultado del Tratamiento , Convulsiones/diagnóstico , Convulsiones/cirugía , Imagen por Resonancia MagnéticaRESUMEN
OBJECTIVES: This study aimed to localise the eloquent cortex and measure evoked field (EF) parameters using magnetoencephalography in patients with epilepsy and tumours near the eloquent cortex. METHODS: A total of 41 patients (26 with drug-refractory epilepsy and 15 with tumours), with a mean age of 33 years, were recruited. Visual evoked field (VEF), auditory evoked field (AEF), sensory evoked field (SSEF), and motor-evoked field (MEF) latencies, amplitudes, and localisation were compared with those of a control population. Subgroup analyses were performed based on lobar involvement. Evoked Field parameters on the affected side were compared with those on the opposite side. The effect of distance from the lesion on nearby and distant evoked fields was evaluated. RESULTS: AEF and VEF amplitudes and latencies were reduced bilaterally (p < 0.05). Amplitude in the ipsilateral SSEF was reduced by 29.27% and 2.16% in the AEF group compared to the contralateral side (p = 0.02). In patients with temporal lobe lesions, the SSEF amplitude was reduced bilaterally (p < 0.02), and latency was prolonged compared with controls. The MEF amplitude was reduced and latency was prolonged in patients with frontal lobe lesions (p = 0.01). EF displacement was 32%, 57%, 21%, and 16% for AEF, MEF, VEF, and SSEF respectively. Patients in the epilepsy group had distant EF abnormalities. CONCLUSIONS: EF amplitude was reduced and latency was prolonged in the involved hemisphere. Distant EF amplitudes were more affected than latencies in epilepsy. Amplitude and distance from the lesion had negative correlation for all EF. EF changes indicated eloquent cortical displacement which may not be apparent on MRI.
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Anterior temporal lobectomy with amygdalohippocampectomy is the most common epilepsy surgery, which, in cases of mesial temporal lobe epilepsy caused by mesial temporal sclerosis, usually leads to improvements in seizure control, cognitive function, and quality of life. Nevertheless, while the primary goal of intervention is achieved in a large majority of patients, a small number of them, unfortunately, encounter complications. Some morbidity is nonspecific and may be noted after any craniotomy (e.g., surgical site infections, meningitis, bone flap osteomyelitis, and operative site or craniotomy-related hematomas). On the other hand, certain complications are specifically associated with surgery for temporal lobe epilepsy and can be discussed from the etiological standpoint: mechanical injuries of the brain; injury of eloquent neuronal structures; arterial and venous injuries; cerebral venous thrombosis; remote cerebellar hemorrhage; and postoperative hydrocephalus, seizures, and psychiatric disorders. In many cases, these complications are manifested in the early postoperative period by alterations of consciousness and a focal neurological deficit, and it may require immediate decisions on their appropriate management.
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Epilepsia del Lóbulo Temporal , Humanos , Epilepsia del Lóbulo Temporal/cirugía , Epilepsia del Lóbulo Temporal/complicaciones , Calidad de Vida , Resultado del Tratamiento , Convulsiones/complicaciones , Convulsiones/cirugía , Lobectomía Temporal Anterior/efectos adversos , Hipocampo/cirugía , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiologíaAsunto(s)
Síndrome de Inmunodeficiencia Adquirida , Infecciones Protozoarias del Sistema Nervioso Central , Meningoencefalitis , Neurosífilis , Humanos , Síndrome de Inmunodeficiencia Adquirida/complicaciones , Meningoencefalitis/complicaciones , Granuloma , Daño Encefálico Crónico , Neurosífilis/complicaciones , Huésped InmunocomprometidoRESUMEN
Objective The study explores whether the epileptic networks associate with predetermined seizure onset zone (SOZ) identified from other modalities such as electroencephalogram/video electroencephalogram/structural MRI (EEG/VEEG/sMRI) and with the degree of resting-state functional MRI/positron emission tomography (RS-fMRI/PET) coupling. Here, we have analyzed the subgroup of patients who reported having a seizure on the day of scan as postictal cases and compared the findings with interictal cases (seizure-free interval). Methods We performed independent component analysis (ICA) on RS-fMRI and 20 ICA were hand-labeled as large scale, noise, downstream, and epilepsy networks (Epinets) based on their profile in spatial, time series, and power spectrum domains. We had a total of 43 cases, with 4 cases in the postictal group (100%). Of 39 cases, 14 cases did not yield any Epinet and 25 cases (61%) were analyzed for the final study. The analysis was done patient-wise and correlated with predetermined SOZ. Results The yield of finding Epinets on RS-fMRI is more during the postictal period than in the interictal period, although PET and RS-fMRI spatial, time series, and power spectral patterns were similar in both these subgroups. Overlaps between large-scale and downstream networks were noted, indicating that epilepsy propagation can involve large-scale cognition networks. Lateralization to SOZ was noted as blood oxygen level-dependent activation and correlated with sMRI/PET findings. Postoperative surgical failure cases showed residual Epinet profile. Conclusion RS-fMRI may be a viable option for trimodality imaging to obtain simultaneous physiological information at the functional network and metabolic level.
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Neurocysticercosis (NCC)-a parasitic CNS infection endemic to developing nations-has been called the leading global cause of acquired epilepsy yet remains understudied. It is currently unknown why a large proportion of patients develop recurrent seizures, often following the presentation of acute seizures. Furthermore, the presentation of NCC is heterogenous and the features that predispose to the development of an epileptogenic state remain uncertain. Perilesional factors (such as oedema and gliosis) have been implicated in NCC-related ictogenesis, but the effects of cystic factors, including lesion load and location, seem not to play a role in the development of habitual epilepsy. In addition, the cytotoxic consequences of the cyst's degenerative stages are varied and the majority of research, relying on retrospective data, lacks the necessary specificity to distinguish between acute symptomatic and unprovoked seizures. Previous research has established that epileptogenesis can be the consequence of abnormal network connectivity, and some imaging studies have suggested that a causative link may exist between NCC and aberrant network organisation. In wider epilepsy research, network approaches have been widely adopted; studies benefiting predominantly from the rich, multimodal data provided by advanced MRI methods are at the forefront of the field. Quantitative MRI approaches have the potential to elucidate the lesser-understood epileptogenic mechanisms of NCC. This review will summarise the current understanding of the relationship between NCC and epilepsy, with a focus on MRI methodologies. In addition, network neuroscience approaches with putative value will be highlighted, drawing from current imaging trends in epilepsy research.
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Epilepsia , Neurocisticercosis , Humanos , Neurocisticercosis/complicaciones , Neurocisticercosis/diagnóstico por imagen , Neurocisticercosis/epidemiología , Estudios Retrospectivos , Convulsiones/etiología , Convulsiones/complicaciones , Epilepsia/diagnóstico por imagen , Epilepsia/etiología , Epilepsia/epidemiología , BiomarcadoresRESUMEN
BACKGROUND: Corpus callosotomy (CC) is a surgical palliative procedure done for a selected group of patients with drug resistant epilepsy (DRE) to stop drop attacks and prevent falls. METHODS: We performed a retrospective chart review of consecutive patients who underwent CC for DRE with drop attacks at our center between 2015 and 2019. Clinical, imaging details and surgical findings were noted. Clinical outcomes and functional status were evaluated. RESULTS: During the study period, 17 patients underwent corpus callosotomy (Male: Female 14:3). The mean age at surgery was 10.3 years (standard deviation - 5.85, interquartile range [IQR] = 6.5). The mean age at onset of seizure was 2.23 years (standard deviation - 3.42, IQR = 1.5). Preoperative seizure frequency ranged from 2 to 60 attacks per day (median: 20, IQR= 36). All patients had atonic seizures/drop attacks. One patient underwent anterior CC and 16 underwent complete CC. Three patients had complications in the postoperative period. The median follow-up was 26 months. All patients had cessation of drop attacks immediately following surgery. One patient with anterior CC had a recurrence of drop attacks for which she underwent completion CC. Another patient had recurrent drop attacks 3 years later and was found to have a residual callosal connection. Three patients had complete seizure freedom and 4 patients had a <50% reduction in seizure frequency. CONCLUSIONS: Our study lends additional support to the efficacy of CC in patients with DRE, with the cessation of drop attacks. It also provided a reasonable reduction in seizure frequency. Complete CC led to better control of drop attacks.
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Epilepsia Refractaria , Psicocirugía , Humanos , Masculino , Femenino , Niño , Epilepsia Refractaria/cirugía , Estudios Retrospectivos , Convulsiones/cirugía , Psicocirugía/métodos , Síncope/cirugía , Cuerpo Calloso/cirugía , Resultado del TratamientoRESUMEN
INTRODUCTION: Video Electroencephalography (VEEG) is crucial for presurgical evaluation of Drug Refractory Epilepsy (DRE). The yield of VEEG in large volume centers, particularly those situated in Low-and Middle-Income countries (LMIC) is not well studied. METHODOLOGY: We studied 1200 adults with drug resistant focal epilepsy whose seizures were recorded during VEEG in the epilepsy monitoring unit. VEEG review and analysis was done independently by trained epileptologists. Video EEG and MRI data were examined for concordance in order to generate a hypothesis for the presumed epileptogenic zone. RESULTS: Analysis of seizure semiology provided information on the symptomatogenic zone in most cases except for 33 (2.75%) patients. A total of 1050 (87.5%) patients showed interictal epileptiform discharges (IEDs) with most (58.3%) showing unilateral IEDs. Most patients (n = 1162, 96.83%) showed ictal EEG discharges of which 951(81.8%) had unilateral ictal onset. Abnormal MRI was seen in 978 (81.5%) patients. Concordance of electroclinical data obtained by analysis of VEEG with MRI abnormality could be established in most patients (63%). Concordance was higher for patients with ictal onset from temporal regions (83.71%) as compared to posterior cortex (55.4%) and frontal regions (43.5%.) CONCLUSION: This study highlights the high yield of VEEG in phase 1 presurgical evaluation in DRE. Systematic evaluation of data from VEEG provided lateralizing and localizing information in most cases. Concordance between VEEG and MRI findings was noted in most patients. These findings support steps to increase referral for pre-surgical evaluation in DRE.
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Epilepsia Refractaria , Humanos , Adulto , Epilepsia Refractaria/cirugía , Estudios Retrospectivos , Países en Desarrollo , Convulsiones , Electroencefalografía , Imagen por Resonancia MagnéticaRESUMEN
Background: A third of people with juvenile myoclonic epilepsy (JME) are drug-resistant. Three-quarters have a seizure relapse when attempting to withdraw anti-seizure medication (ASM) after achieving seizure-freedom. It is currently impossible to predict who is likely to become drug-resistant and safely withdraw treatment. We aimed to identify predictors of drug resistance and seizure recurrence to allow for individualised prediction of treatment outcomes in people with JME. Methods: We performed an individual participant data (IPD) meta-analysis based on a systematic search in EMBASE and PubMed - last updated on March 11, 2021 - including prospective and retrospective observational studies reporting on treatment outcomes of people diagnosed with JME and available seizure outcome data after a minimum one-year follow-up. We invited authors to share standardised IPD to identify predictors of drug resistance using multivariable logistic regression. We excluded pseudo-resistant individuals. A subset who attempted to withdraw ASM was included in a multivariable proportional hazards analysis on seizure recurrence after ASM withdrawal. The study was registered at the Open Science Framework (OSF; https://osf.io/b9zjc/). Findings: Our search yielded 1641 articles; 53 were eligible, of which the authors of 24 studies agreed to collaborate by sharing IPD. Using data from 2518 people with JME, we found nine independent predictors of drug resistance: three seizure types, psychiatric comorbidities, catamenial epilepsy, epileptiform focality, ethnicity, history of CAE, family history of epilepsy, status epilepticus, and febrile seizures. Internal-external cross-validation of our multivariable model showed an area under the receiver operating characteristic curve of 0·70 (95%CI 0·68-0·72). Recurrence of seizures after ASM withdrawal (n = 368) was predicted by an earlier age at the start of withdrawal, shorter seizure-free interval and more currently used ASMs, resulting in an average internal-external cross-validation concordance-statistic of 0·70 (95%CI 0·68-0·73). Interpretation: We were able to predict and validate clinically relevant personalised treatment outcomes for people with JME. Individualised predictions are accessible as nomograms and web-based tools. Funding: MING fonds.
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Background: Sensory nerve conduction parameters in Guillain Barre Syndrome (GBS) are underemphasized. Objective: To describe abnormalities on sensory conduction studies in a large cohort of prospectively evaluated patients of GBS and to correlate with clinico-electrophysiological features. Methods and Materials: Sensory conduction parameters of three nerves (median, ulnar, and sural) were analyzed using standard protocols in 238 patients (M: F 163:75, mean age: 35.76 ± 15.9 years). Electrophysiological subtyping was based on criteria of Hadden et al., and Rajabally et al. Results: Among patients with "typical" GBS who underwent electrophysiological testing within 30 days of symptom-onset (n = 219), 183 (83.5%) had abnormal sensory potentials (one nerve = 52, two nerves = 77, all three nerves = 54). Frequency of abnormalities in sensory potentials increased with duration of illness. Commonest abnormality was reduced amplitude or in-excitable nerves. Mean amplitude and velocity of median and ulnar nerve sensory potentials were significantly lower among those with demyelinating electrophysiology (P < 0.05). Proportion of subjects with reduced amplitude and velocity of median and ulnar nerve sensory potentials was higher among those who required mechanical ventilation (P < 0.05). Frequency of "sural sparing" ranged from 10.5% to 84.5% depending on the criteria used and almost always was significantly associated with demyelinating neuropathy. Conclusion: This prospective study provides comprehensive data on sensory conduction parameters in GBS. Abnormalities are frequent and vary with duration of illness. While median nerve is most frequently and more severely affected, involvement of sural nerve may have prognostic value.
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Síndrome de Guillain-Barré , Humanos , Adulto Joven , Adulto , Persona de Mediana Edad , Estudios Prospectivos , Nervio Sural , Conducción Nerviosa/fisiología , Respiración ArtificialRESUMEN
Background: Myasthenia gravis (MG) is an immune-mediated disorder of the neuromuscular junction. About 10% are refractory to immunosuppressive therapy. Aims: To analyze the response of patients with generalized MG to rituximab. Methods and Materials: A retrospective review of patients with MG who received rituximab was carried out (n = 13, M:F = 6:7, mean age: 44.84 ± 15.73 years). Myasthenia Gravis Foundation of America (MGFA), MGFA post-intervention status (MGFA-PIS), and Myasthenia Gravis Status and Treatment Intensity (MGSTI) were assessed before and after rituximab. Results: The duration of MG was 104.07 ± 92.25 months. Before rituximab, the MGFA was IIA/IIB/IIIA/IIIB/IVB/V in 1/1/2/6/2/1 patients and MGSTI was four in eight patients and six in three patients. The mean duration of follow up was 20.92 ± 14.06 months (range, 4 to 42 months). Dose reduction or discontinuation of cholinesterase inhibitors could be achieved 12 patients. Complete stable remission (CSR) and pharmacologic remission (PR) were achieved in one and four patients respectively and five patients had minimal manifestations. Most patients attained level 0, 1 or 2 MGSTI at last follow up. No rituximab infusion-related adverse events were noted. Three patients had exacerbation of MG between one to five weeks after rituximab administration. Three patients died, one each due to a cardiac event unrelated to MG or treatment, complications related to myasthenic crisis, and coronavirus disease. Conclusions: Rituximab was effective in bringing about remission in MG and can be considered as a first-line agent. However, it has to be administered under close supervision as some patients develop exacerbation of MG akin to steroid-induced worsening.