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1.
Cereb Cortex ; 33(5): 2215-2228, 2023 02 20.
Article in English | MEDLINE | ID: mdl-35695785

ABSTRACT

The envelope is essential for speech perception. Recent studies have shown that cortical activity can track the acoustic envelope. However, whether the tracking strength reflects the extent of speech intelligibility processing remains controversial. Here, using stereo-electroencephalogram technology, we directly recorded the activity in human auditory cortex while subjects listened to either natural or noise-vocoded speech. These 2 stimuli have approximately identical envelopes, but the noise-vocoded speech does not have speech intelligibility. According to the tracking lags, we revealed 2 stages of envelope tracking: an early high-γ (60-140 Hz) power stage that preferred the noise-vocoded speech and a late θ (4-8 Hz) phase stage that preferred the natural speech. Furthermore, the decoding performance of high-γ power was better in primary auditory cortex than in nonprimary auditory cortex, consistent with its short tracking delay, while θ phase showed better decoding performance in right auditory cortex. In addition, high-γ responses with sustained temporal profiles in nonprimary auditory cortex were dominant in both envelope tracking and decoding. In sum, we suggested a functional dissociation between high-γ power and θ phase: the former reflects fast and automatic processing of brief acoustic features, while the latter correlates to slow build-up processing facilitated by speech intelligibility.


Subject(s)
Auditory Cortex , Speech Perception , Humans , Speech/physiology , Auditory Cortex/physiology , Speech Intelligibility , Acoustic Stimulation , Electroencephalography , Speech Perception/physiology
2.
Epilepsia ; 64(3): 667-677, 2023 03.
Article in English | MEDLINE | ID: mdl-36510851

ABSTRACT

OBJECTIVE: This study aimed to investigate the quantitative relationship between interictal 18 F-fluorodeoxyglucose-positron emission tomography (FDG-PET) and interictal high-frequency oscillations (HFOs) from stereo-electroencephalography (SEEG) recordings in patients with refractory epilepsy. METHODS: We retrospectively included 32 patients. FDG-PET data were quantified through statistical parametric mapping (SPM) t test modeling with normal controls. Interictal SEEG segments with four, 10-min segments were selected randomly. HFO detection and classification procedures were automatically performed. Channel-based HFOs separating ripple (80-250 Hz) and fast ripple (FR; 250-500 Hz) counts were correlated with the surrounding metabolism T score at the individual and group level, respectively. The association was further validated across anatomic seizure origins and sleep vs wake states. We built a joint feature FR × T reflecting the FR and hypometabolism concordance to predict surgical outcomes in 28 patients who underwent surgery. RESULTS: We found a negative correlation between interictal FDG-PET and HFOs through the linear mixed-effects model (R2  = .346 and .457 for ripples and FRs, respectively, p < .001); these correlations were generalizable to different epileptogenic-zone lobar localizations and vigilance states. The FR × T inside the resection volume could be used as a predictor for surgical outcomes with an area under the curve of 0.81. SIGNIFICANCE: The degree of hypometabolism is associated with HFO generation rate, especially for FRs. This relationship would be meaningful for selection of SEEG candidates and for optimizing SEEG scheme planning. The concordance between FRs and hypometabolism inside the resection volume could provide prognostic information regarding surgical outcome.


Subject(s)
Electroencephalography , Fluorodeoxyglucose F18 , Humans , Retrospective Studies , Electroencephalography/methods , Positron-Emission Tomography , Treatment Outcome
3.
Eur J Neurol ; 29(8): 2376-2385, 2022 Aug.
Article in English | MEDLINE | ID: mdl-35514068

ABSTRACT

BACKGROUND AND PURPOSE: Faciobrachial dystonic seizures (FBDS) and hyponatremia are the distinct clinical features of autoimmune encephalitis (AE) caused by antibodies against leucine-rich glioma-inactivated 1 (LGI1). The present study aims to explore the pathophysiological patterns and neural mechanisms underlying these symptoms. METHODS: We included 30 patients with anti-LGI1 AE and 30 controls from a retrospective observational cohort. Whole-brain metabolic pattern analysis was performed to assess the pathological network of anti-LGI1 AE, as well as the symptom networks associated with FBDS. Logistic regression was applied to explore independent predictors of FBDS. Finally, we used a multiple regression model to investigate the hyponatremia-associated brain network and its effect on serum sodium levels. RESULTS: The pathological network of anti-LGI1 AE involved hypermetabolism in the cerebellum, subcortical structures and Rolandic area, as well as hypometabolism in the medial prefrontal cortex. The symptom network of FBDS included hypometabolism in the cerebellum and Rolandic area (pFDR <0.05). Hypometabolism in the cerebellum was an independent predictor of FBDS (p < 0.001). Hyponatremia-associated network highlighted a negative effect on the caudate nucleus, frontal and temporal white matter. The metabolism of the hypothalamus was negatively associated with (Pearson's R = -0.180, p = 0.342), while not the independent predictor for serum sodium level (path c' = -7.238, 95% confidence interval = -30.947 to 16.472). CONCLUSIONS: Our results provide insights into the whole-brain metabolic patterns of patients with anti-LGI1 AE, including the symptom network associated with FBDS and the hyponatremia-associated brain network. The findings help us to understand the neural mechanisms underlying anti-LGI1 AE and to evaluate the progress of this disease.


Subject(s)
Autoimmune Diseases , Brain , Limbic Encephalitis , Autoantibodies/blood , Autoimmune Diseases/complications , Autoimmune Diseases/metabolism , Brain/metabolism , Humans , Hyponatremia/etiology , Hyponatremia/metabolism , Intracellular Signaling Peptides and Proteins/immunology , Limbic Encephalitis/complications , Limbic Encephalitis/metabolism , Retrospective Studies , Seizures/etiology , Seizures/metabolism , Sodium/blood
4.
Epilepsy Behav ; 114(Pt A): 107614, 2021 01.
Article in English | MEDLINE | ID: mdl-33277200

ABSTRACT

PURPOSE: The role of the Rolandic operculum in in mesial temporal lobe epilepsy (MTLE) is to produce oroalimentary automatisms (OAAs). In insulo-opercular epilepsy (IOE), the Rolandic operculum may produce perioral muscle clonic or tonic movements or contractions. This paper aims to confirm the symptomatogenic zone of facial symptoms in IOE and to explain this phenomenon. METHODS: A total of 45 IOE patients and 15 MTLE patients were analyzed. The patients with IOE were divided into facial (+) and (-) groups according to the facial symptoms. The interictal positron emission tomography (PET) data were compared among groups. Furthermore, electroclinical correlation, functional connectivity and energy ratio (ER) were analyzed with stereo-electroencephalography (SEEG). RESULTS: Intergroup PET differences were observed mainly in the Rolandic operculum. Electroclinical correlation showed that the Rolandic operculum was the only brain area showing any correlations. Compared with the facial (-) group, the facial (+) group showed stronger functional connectivity and a higher ER in the alpha 1, alpha 2 and beta sub-bands. In the Rolandic operculum, compared with those of the MTLE group, the h2 and ER of the facial (+) group were higher in the high frequency sub-bands. Intergroup comparison of the ER in the seizure onset zones (SOZ) showed no significant difference. SIGNIFICANCE: The symptomatogenic zone of facial symptoms in IOE is the Rolandic operculum. Seizure propagation to the Rolandic operculum generates different semiologies because of the different synchronization frequencies and energies of the sub-bands depending on the site of seizure origin. This may be due to the complex spreading pathway from the SOZ to the symptomatogenic zone.


Subject(s)
Epilepsy, Frontal Lobe , Epilepsy, Temporal Lobe , Cerebral Cortex , Electroencephalography , Epilepsy, Temporal Lobe/diagnostic imaging , Humans , Positron-Emission Tomography
5.
Epilepsy Behav ; 118: 107957, 2021 05.
Article in English | MEDLINE | ID: mdl-33872942

ABSTRACT

BACKGROUND: Radiofrequency thermocoagulation (RFTC) guided by stereo-electroencephalography (SEEG) has been proved to be a safe method to reduce seizure frequency in patients with drug-resistant epilepsy. However, there are few reports addressing the value and safety of this procedure in hypothalamic hamartoma (HH). OBJECTIVE: To present the results of our experience using SEEG-guided RFTC in HH patients with drug-resistant epilepsy, and identify outcome predictors. METHODS: We retrospectively reviewed the clinical and surgical characteristics of 27 HH-related patients with epilepsy in our center between 2015 and 2019. All patients underwent invasive recordings with SEEG before RFTC was performed. We reported surgical outcome predictors and postoperative follow-up concerning safety and efficacy (mean follow-up, 27.3 months; range, 12-63). Surgical strategy was also analyzed. RESULTS: Nineteen patients (70.4%) achieved Engel's class I outcome, while 4 patients (14.8%) did not show significant improvement. Of all observed seizures, two different onset patterns of intracranial electrophysiology recorded by SEEG were observed. Patients presented with focal low-voltage fast activity were more likely to obtain seizure freedom (p = 0.045), while classification (p = 0.478), volume (p = 0.546), history of resection (p = 0.713), seizure types (p = 0.859), or seizure duration (p = 0.415) showed no significant effect on the outcome. Weight gain was the most common long-term complication (18.5%). CONCLUSION: The SEEG can guide the ablation of HH and serve as an important factor to predict favorable seizure outcomes. Radiofrequency thermocoagulation guided by SEEG can offer a minimally invasive and low-risk surgical approach with excellent outcomes. Disconnecting the attachment of HH should be the appropriate strategy to obtain the best seizure outcome.


Subject(s)
Hamartoma , Electrocoagulation , Electroencephalography , Hamartoma/surgery , Humans , Hypothalamic Diseases , Magnetic Resonance Imaging , Prognosis , Retrospective Studies , Stereotaxic Techniques , Treatment Outcome
6.
Epilepsy Behav ; 122: 108130, 2021 09.
Article in English | MEDLINE | ID: mdl-34153637

ABSTRACT

OBJECTIVE: Hyperkinetic seizures (HKS) are characterized by complex movements that commonly occur during seizures arising from diverse cortical structures. A common semiology network may exist and analyzing the anatomo-electrical mechanisms would facilitate presurgical evaluation. Here, quantitative positron emission tomography (PET) and stereoelectroencephalography (SEEG) analysis was used to explore the underlying mechanism of HKS. METHODS: We retrospectively collected patients with epilepsy with HKS between 2014 and 2019. The interictal PET data of patients with epilepsy with HKS were compared with those of 25 healthy subjects using statistical parametric mapping to identify regions with significant hypometabolism. Then, regions of interest (ROI) for SEEG analysis were identified based on the results of PET analysis. Patients in which the ROIs were covered by intracerebral electrodes were selected for further analysis. Stereoelectroencephalography -clinical correlations with latency measurements were analyzed, and we also performed coherence analysis among ROIs both before and during HKS. RESULTS: Based on the inclusion criteria, 27 patients were analyzed. In the PET analysis, significant hypometabolism was observed in the ipsilateral dorsoanterior insular lobe, bilateral mesial frontal lobes (supplementary motor area/middle cingulate cortex, SMA/MCC), and the bilateral heads of the caudate nuclei in patients with HKS compared with the control group (p < 0.001). We selected dorsoanterior insula and SMA/MCC as ROIs for SEEG analysis. Eight patients with 23 HKS events were selected for further analysis. There was a linear correlation between the ictal involvement of both the dorsoanterior insula and SMA/MCC with the onset of HKS. Stereoelectroencephalography analysis indicated alpha range activity seemed more often associated with dorsoanterior insula and SMA/MCC involvement during HKS. CONCLUSIONS: The dorsoanterior insular lobe, mesial frontal lobes (SMA/MCC), and the bilateral heads of the caudate nuclei were probably involved in the generation of HKS. The SEEG analysis further indicated that the occurrence of HKS might be partly associated with synchronized rhythmical alpha activity between dorsoanterior insula and SMA/MCC.


Subject(s)
Electroencephalography , Positron-Emission Tomography , Humans , Neural Networks, Computer , Retrospective Studies , Seizures/diagnostic imaging
7.
Epilepsy Behav ; 121(Pt A): 108028, 2021 08.
Article in English | MEDLINE | ID: mdl-34058496

ABSTRACT

OBJECTIVE: To summarize the clinical and electrophysiological observations of epilepsy originating from the inferior perisylvian cortex, and analyze the potential epileptic networks underlying the semiological manifestations. METHODS: We retrospectively analyzed patients with refractory inferior perisylvian epilepsy (IPE) who had undergone resective surgery, and then reviewed the demographic, clinical, neuroelectrophysiological, neuroimaging, surgical, histopathological, and follow-up data of the patients from the respective medical records. The selected patients were then categorized in accordance with the results of semiological analysis. Quantitative 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) analysis was performed to investigate the underlying neural network. RESULTS: Of the 18 IPE patients assessed in this study, ipsilateral frontotemporal epileptic discharges or its onsets were the dominant interictal or ictal scalp EEG observations. In addition, oroalimentary or manual automatism was the most frequently documented manifestation, followed by facial tonic or clonic movements. Moreover, the semiological analysis identified and classified the patients into 2 patterns, and the PET statistical analyses conducted on these 2 groups revealed differences in the neural network between them. CONCLUSION: Inferior perisylvian epilepsy possesses semiological manifestations similar to those of mesial temporal lobe epilepsy or rolandic opercular epilepsy, hence these conditions should be carefully differentiated. Performing lesionectomy or cortectomy, sparing the mesial temporal structures, was found to be an effective and safe treatment modality for IPE.


Subject(s)
Epilepsy, Frontal Lobe , Epilepsy, Temporal Lobe , Electroencephalography , Fluorodeoxyglucose F18 , Humans , Magnetic Resonance Imaging , Positron-Emission Tomography , Retrospective Studies , Treatment Outcome
8.
Epilepsy Behav ; 115: 107661, 2021 02.
Article in English | MEDLINE | ID: mdl-33434884

ABSTRACT

OBJECTIVE: Mesial temporal lobe epilepsy (MTLE) is one of the most common types of intractable epilepsy. The hippocampus and amygdala are two crucial structures of the mesial temporal lobe and play important roles in the epileptogenic network of MTLE. This study aimed to explore the effective connectivity among the hippocampus, amygdala, and temporal neocortex and to determine whether differences in effective connectivity exist between MTLE patients and non-MTLE patients. METHODS: This study recruited 20 patients from a large cohort of drug-resistant epilepsy patients, of whom 14 were MTLE patients. Single-pulse electrical stimulation (SPES) was performed to acquire cortico-cortical evoked potentials (CCEPs). The root mean square (RMS) was used as the metric of the magnitude of CCEP to represent the effective connectivity. We then conducted paired and independent sample t-tests to assess the directionality of the effective connectivity. RESULTS: In both MTLE patients and non-MTLE patients, the directional connectivity from the amygdala to the hippocampus was stronger than that from the hippocampus to the amygdala (P < 0.01); the outward connectivity from the amygdala to the cortex was stronger than the inward connectivity from the cortex to the amygdala (P < 0.01); the amygdala had stronger connectivity to the neocortex than the hippocampus (P < 0.01). In MTLE patients, the neocortex had stronger connectivity to the hippocampus than to the amygdala (P < 0.01). No significant differences in directional connectivity were noted between the two groups. CONCLUSIONS: A unique effective connectivity pattern among the hippocampus, amygdala, and temporal neocortex was identified through CCEPs analysis. This study may aid in our understanding of physiological and pathological networks in the brain and inspire neurostimulation protocols for neurological and psychiatric disorders.


Subject(s)
Epilepsy, Temporal Lobe , Epilepsy , Neocortex , Amygdala , Evoked Potentials , Hippocampus , Humans
9.
BMC Neurosci ; 21(1): 13, 2020 03 30.
Article in English | MEDLINE | ID: mdl-32228575

ABSTRACT

BACKGROUND: Patients positive for anti-glutamic acid decarboxylase 65 (GAD65) antibodies have attracted increasing attention. Their clinical manifestations are highly heterogeneous and can be comorbid with tumors. Currently, there is no consensus on the therapeutic regimen for anti-GAD65-associated neurological diseases due to the clinical complexity, rarity and sporadic distribution. We reported six anti-GAD65 autoimmune encephalitis (AE) patients who received intravenous methylprednisolone (IVMP) or immunoglobulin (IVIG) or both. Then, we evaluated the therapeutic effect of both by summarizing results in previous anti-GAD65 AE patients from 70 published references. RESULTS: Our six patients all achieved clinical improvements in the short term. Unfortunately, there was no significant difference between IVMP and IVIG in terms of therapeutic response according to the previous references, and the effectiveness of IVMP and IVIG was 45.56% and 36.71%, respectively. We further divided the patients into different subgroups according to their prominent clinical manifestations. The response rates of IVMP and IVIG were 42.65% and 32.69%, respectively, in epilepsy patients; 60.00% and 77.78%, respectively, in patients with stiff-person syndrome; and 28.57% and 55.56%, respectively, in cerebellar ataxia patients. Among 29 anti-GAD65 AE patients with tumors, the response rates of IVMP and IVIG were 29.41% and 42.11%, respectively. There was no significant difference in effectiveness between the two regimens among the different subgroups. CONCLUSION: Except for stiff-person syndrome, we found that this kind of AE generally has a poor response to IVMP or IVIG. Larger prospective studies enrolling large numbers of patients are required to identify the optimal therapeutic strategy in the future.


Subject(s)
Encephalitis/drug therapy , Glucocorticoids/administration & dosage , Glutamate Decarboxylase/immunology , Hashimoto Disease/drug therapy , Immunoglobulins/administration & dosage , Immunologic Factors/administration & dosage , Methylprednisolone/administration & dosage , Administration, Intravenous , Adult , Autoantibodies/blood , Autoantibodies/cerebrospinal fluid , Brain/diagnostic imaging , Brain/pathology , Encephalitis/diagnostic imaging , Encephalitis/immunology , Female , Hashimoto Disease/diagnostic imaging , Hashimoto Disease/immunology , Humans , Male , Middle Aged , Retrospective Studies , Treatment Outcome
10.
Epilepsia ; 61(7): 1341-1351, 2020 07.
Article in English | MEDLINE | ID: mdl-32544279

ABSTRACT

Seizures are a well-recognized and often prominent manifestation of autoimmune encephalitic syndromes. Progress in detection of pathogenic neural autoantibodies has led to increased awareness of autoimmune causes of seizures. Clinical studies of patients with these autoantibodies have improved our understanding of the seizure characteristics, treatments, and seizure prognosis in these disorders. The International League Against Epilepsy (ILAE) Autoimmunity and Inflammation Taskforce proposes conceptual definitions for two main diagnostic entities: (a) acute symptomatic seizures secondary to autoimmune encephalitis, and (b) autoimmune-associated epilepsy, the latter of which suggests an enduring predisposition to seizures. Such a distinction is relevant when discussing the pathophysiology, treatment, prognosis, and social consequences of these disorders. We discuss the role of biomarkers in the application of these conceptual definitions and illustrate their use in patients cared for by members of the task force.


Subject(s)
Encephalitis/blood , Encephalitis/diagnosis , Epilepsy/blood , Epilepsy/diagnosis , Hashimoto Disease/blood , Hashimoto Disease/diagnosis , Seizures/blood , Seizures/diagnosis , Acute Disease , Adolescent , Adult , Autoantibodies/blood , Biomarkers/blood , Encephalitis/complications , Epilepsy/complications , Female , Hashimoto Disease/complications , Humans , Male , Middle Aged , Seizures/etiology , Young Adult
11.
Epilepsia ; 61(5): 984-994, 2020 05.
Article in English | MEDLINE | ID: mdl-32314372

ABSTRACT

OBJECTIVE: Insulo-opercular seizures are characterized by diverse semiology, related to the insula's multiple functional roles and extensive connectivity. We aimed to identify semiologic subgroups and correlate these with insulo-opercular subregions based on connectional architecture. METHODS: We retrospectively collected a large series of 37 patients with insulo-opercular seizures explored by stereoelectroencephalography (SEEG) from three epilepsy centers. A new human brain atlas (Brainnetome Atlas, BNA) based on both anatomic and functional connections was employed to segment insulo-opercular cortex. Semiology and SEEG changes were carefully reviewed and quantified. Principal component analysis and cluster analysis were used to correlate semiologic characteristics with insulo-opercular subregions. RESULTS: Four main semiologic subgroups were identified, organized along an anteroventral to posterodorsal axis based on BNA. Group 1 was characterized by epigastric sensation and/or integrated gestural motor behaviors with or without feelings of fear or rage, involving the anteroventral insular regions and mesial temporal lobes. Group 2 was characterized by auditory sensations and symmetric proximal/axial tonic signs involving the posteroventral temporal operculum. The characteristics of group 3 were orofacial and laryngeal signs, involving the intermediate insulo-opercular regions. The features of group 4 were somatosensory signs followed by nonintegrated gestural motor behaviors and/or asymmetric tonic signs involving the posterodorsal insulo-opercular regions with propagation to the mesial frontal lobes. Thus anteroventral seizure organizations predominantly showed limbic system semiology, whereas more posterodorsal regions were associated with semiology involving mainly the sensorimotor system. Subjective symptoms proved to be particularly discriminating factors. SIGNIFICANCE: Insulo-opercular seizures can be categorized in terms of clinical semiology and correlate with connectional architecture subregions along an anteroventral-posterodorsal axis in line with the cytoarchitectonic gradient rather than the gyral anatomy of the insula cortex. This provides new insights into facilitating differential diagnosis and presurgical localization but also highlights the importance of considering connectional architecture in determining neural correlates of complex semiologic patterns.


Subject(s)
Cerebral Cortex/pathology , Epilepsy, Frontal Lobe/pathology , Neural Pathways/pathology , Seizures/pathology , Adolescent , Cerebral Cortex/physiopathology , Electroencephalography , Epilepsy, Frontal Lobe/classification , Epilepsy, Frontal Lobe/physiopathology , Female , Humans , Male , Neural Pathways/physiopathology , Principal Component Analysis , Retrospective Studies , Seizures/classification , Seizures/etiology , Seizures/physiopathology
12.
Epilepsia ; 60(6): 1150-1159, 2019 06.
Article in English | MEDLINE | ID: mdl-31095733

ABSTRACT

OBJECTIVE: Oroalimentary automatisms (OAAs) are common clinical manifestations of medial temporal lobe epilepsy. Nevertheless, the location of the symptomatogenic zone of OAAs remains unclear. The generation mechanism of OAAs also has not been clarified. We attempt to explain these problems by analyzing interictal [18 F]-fluorodeoxyglucose positron emission tomography (18 FDG-PET) imaging and ictal stereo-electroencephalography (SEEG) recordings in patients with medial temporal lobe epilepsy. METHODS: Fifty-seven patients with mesial temporal lobe epilepsy were analyzed retrospectively. All underwent anterior temporal lobectomy (ATL) and were seizure-free. The patients were divided into OAA (+) and OAA (-) groups according to the occurrence of consistent stereotyped OAAs. The interictal PET data were compared with those of 18 healthy controls and were then compared between groups using statistical parametric mapping (SPM). Functional connectivity using linear regression analysis was performed between the target brain regions. To clarify the network of OAAs, ictal epileptogenicity index (EI) values, and the nonlinear correlation method h2 were performed with SEEG on patients. RESULTS: Compared to OAAs (-), the rolandic operculum was the only area with significant differences. Hippocampus and rolandic operculum showed significant correlations in the OAA (+) group (y = 0.758x+0.470, R2  = 0.456, P = 0.000). No correlation was found in the OAA (-) group (P = 0.486). The EI values of the OAA (+) group (median 0.20) were significantly higher (P < 0.0001) than those of the OAA (-) group (median 0). The h2 in the OAA (+) group (h2  = 0.23 ± 0.13) showed stronger functional connectivity (t = 6.166, P < 0.0001) than that of the OAA (-) group (h2  = 0.08 ± 0.05). SIGNIFICANCE: The rolandic operculum is most likely to be the symptomatogenic zone of OAAs. In medial temporal lobe epilepsy, unilateral functional connection from the hippocampus to the rolandic operculum during seizure onset is the basis for the generation of OAAs.


Subject(s)
Automatism/diagnostic imaging , Automatism/etiology , Epilepsy, Temporal Lobe/complications , Epilepsy, Temporal Lobe/diagnostic imaging , Adolescent , Adult , Anterior Temporal Lobectomy , Child , Child, Preschool , Electroencephalography , Epilepsy, Temporal Lobe/surgery , Female , Fluorodeoxyglucose F18 , Hippocampus/diagnostic imaging , Hippocampus/physiopathology , Humans , Infant , Male , Mouth/physiopathology , Positron-Emission Tomography , Radiopharmaceuticals , Retrospective Studies , Treatment Outcome , Young Adult
13.
Epilepsy Behav ; 77: 90-95, 2017 12.
Article in English | MEDLINE | ID: mdl-29050866

ABSTRACT

OBJECTIVE: The objective of this study was to advance the characterization of seizure semiology in leucine-rich glioma-inactivated protein 1 (LGI1) antibody-associated limbic encephalitis (LE). METHODS: Eighteen patients diagnosed with LGI1 LE were identified. Seizure semiology, demographic features, MRI and fluorodeoxyglucose positron emission tomography (FDG-PET), electroencephalograms, and outcomes following immunotherapy were evaluated. RESULTS: Patients were divided into the following groups based on seizure semiology: faciobrachial dystonic seizure only (FBDS-only, n=4), epileptic seizure without FBDS (Non-FBDS, n=6), and FBDS plus epileptic seizure (FBDS+, n=8). In the group with Non-FBDS, the majority of patients (5/6) manifested mesial temporal lobe epilepsy (MTLE) like semiology (i.e., fear, epigastric rising, staring, and automatisms) with a frequency of 7±5 times per day and a duration of 15.3±14.3s. In the group with FBDS+, the distinctive symptom was FBDS followed by epileptic events, especially automatisms (7/8), with a frequency of 16±12 times per day and a duration of 13.0±8.0s. In these cases, 67% and 50% of the patients showed abnormalities on MRI and FDG-PET, respectively, and the mesial temporal lobe structures were most often involved. Ictal discharges were observed in 0/4, 6/6, and 8/8 of the patients in the groups with FBDS only, Non-FBDS, and FBDS+, respectively. The temporal lobe was mainly affected. Immunotherapy had favorable therapeutic effects. SIGNIFICANCE: The LGI1 LE should be considered as one disease syndrome with a series of clinical manifestation. Identifying types of unique semiology features will facilitate the early diagnosis and the timely initiation of immunotherapy.


Subject(s)
Autoantibodies , Limbic Encephalitis/complications , Proteins/immunology , Seizures/etiology , Adult , Aged , Electroencephalography/methods , Female , Humans , Immunotherapy , Intracellular Signaling Peptides and Proteins , Limbic Encephalitis/immunology , Limbic Encephalitis/therapy , Magnetic Resonance Imaging , Male , Middle Aged , Seizures/immunology , Seizures/therapy , Treatment Outcome
14.
Int J Neurosci ; 127(8): 726-734, 2017 Aug.
Article in English | MEDLINE | ID: mdl-27619342

ABSTRACT

Circular RNAs (circRNAs) are novel endogenous non-coding RNAs characterized by the presence of a covalent bond linking the 3' and 5' ends generated by backsplicing. In this review, we summarize a number of the latest theories regarding the biogenesis, properties and functions of circRNAs. Specifically, we focus on the advancing characteristics and functions of circRNAs in the brain and neurological diseases. CircRNAs exhibit the characteristics of species conservation, abundance and tissue/developmental-stage-specific expression in the brain. We also describe the relationship between circRNAs and several neurological diseases and highlight their functions in neurological diseases.


Subject(s)
Nervous System Diseases/genetics , Nervous System Diseases/metabolism , RNA/biosynthesis , RNA/genetics , Animals , Brain/metabolism , Brain/pathology , Humans , Nervous System Diseases/pathology , RNA, Circular
15.
Neuromodulation ; 19(8): 818-823, 2016 Dec.
Article in English | MEDLINE | ID: mdl-26399458

ABSTRACT

OBJECTIVES: The aim of this study was to report on four patients with craniocervical dystonia (CCD) treated with deep brain stimulation (DBS). In addition, we investigated the treatment efficacy and surgical outcome predictors by the review and analysis of previously published studies. METHODS: Four patients with CCD underwent DBS of the globus pallidus internus (Gpi) or subthalamus nucleus (STN). PubMed and MEDLINE searches were performed to obtain detailed information on patients who underwent DBS for CCD. The primary efficacy endpoint was the change in the Burke-Fahn-Marsden Dystonia Rating Scale (movement and disability scores, BFMDRS-M/D) after surgery. RESULTS: Seventy-five patients were included in the pooled analysis, including 69 patients with Gpi-DBS and 6 patients with STN-DBS. The mean follow-up of time was 28.0 months after surgery. The mean BFMDRS-M score was 24.5 ± 11.2 preoperatively and 8.1 ± 5.7 postoperatively at the final follow-up evaluation, with a mean improvement of 66.9% (p < 0.001). The mean BFMDRS-D score was 8.1 ± 4.6 preoperatively and 3.6 ± 2.5 postoperatively, with a mean percentage improvement of 56.0% (p < 0.01). Positive correlations were found between each of the preoperative movement and disability scores and percentage of postoperative improvement (r = 0.247, p = 0.034; r = 0.331, p = 0.034, respectively). CONCLUSION: GPi/STN-DBS is an effective treatment for patients with medically refractory CCD, including those with severe preoperative symptoms. The age at CCD onset and the disease duration do not predict improvement in movement scores.


Subject(s)
Craniofacial Abnormalities/therapy , Deep Brain Stimulation/methods , Globus Pallidus/physiology , Osteochondrodysplasias/therapy , Subthalamic Nucleus/physiology , Adult , Aged , Databases, Bibliographic/statistics & numerical data , Female , Humans , Male , Middle Aged , Treatment Outcome
16.
J Stroke Cerebrovasc Dis ; 24(2): 455-64, 2015 Feb.
Article in English | MEDLINE | ID: mdl-25511617

ABSTRACT

BACKGROUND: To determine whether the presence of seizures in patients with spontaneous intracerebral hemorrhage (ICH) was associated with in-hospital complications and measured outcomes. METHODS: This prospective cohort study from the China National Stroke Registry included consecutive patients with ICH between August 2007 and September 2008. In-hospital complications, functional outcomes, and mortality at 3, 6, and 12 months were compared between patients with seizures and those without seizures occurring at ICH onset and during hospitalization. Poor functional outcome was defined as a modified Rankin Scale score between 3 and 6. Poor functional outcome and mortality were stratified by stroke severity using Glasgow Coma Scale scores on admission. RESULTS: The study included 3216 patients with ICH and 139 of them (4.3%) experienced seizures. The presence of seizures was associated with high in-hospital complications including atrial fibrillation (P = .004), pneumonia (P = .001), as well as lower rehabilitation assessment rates (P = .033) compared with patients without seizures. ICH patients with seizures had poorer functional outcome at 3-month (P = .012), 6-month (P = .007), and 12-month (P = .001) follow-up. They also had higher mortality at 3 months (P = .045), 6 months (P = .005), and 12 months (P = .002). Patients with mild strokes had poorer functional outcome and higher mortality (P < .005) if seizures occurred. CONCLUSIONS: The presence of seizures in patients with ICH was associated with high in-hospital complications and indicates poor outcomes at 3-, 6-, and 12-month follow-up. Quality improvement strategies targeting ICH patients with seizures especially mild stroke may help improve prognoses.


Subject(s)
Intracranial Hemorrhages/epidemiology , Seizures/epidemiology , Stroke/epidemiology , Aged , Aged, 80 and over , Cerebral Hemorrhage/mortality , China , Cohort Studies , Female , Humans , Intracranial Hemorrhages/mortality , Male , Middle Aged , Prognosis , Recovery of Function , Registries , Risk Factors , Seizures/mortality , Stroke/mortality
17.
BMC Neurol ; 14: 194, 2014 Oct 02.
Article in English | MEDLINE | ID: mdl-25269594

ABSTRACT

BACKGROUND: Some recent studies suggest that some imaging-negative temporal lobe epilepsy (TLE) had significant amygdala enlargement (AE). Contradictory data were also reported in previous studies regarding the association between AE and TLE. The present study was to investigate the clinical characters of a group of TLE with AE and compare the amygdala volume of the same patient before and after antiepileptic drugs treatment by a larger sample size. METHODS: This study recruited 33 mesial TLE patients with AE and 35 healthy volunteers. The clinical history, seizure semiology, electroencephalogram (EEG), fluorodeoxyglucose-positron emission tomography (FDG-PET) and amygdala volume were investigated. The amygdala volume were compared between ipsilateral and contralateral sides, TLE patients and 35 healthy controls, and patients at first and follow-up visit by 3.0 T MRI. RESULTS: Average seizure onset age was 42.0 years (SD 14.3). All patients had complex partial seizures, fourteen had occasional generalized tonic-clonic seizures which often happened during sleep. Ninety percent patients suffered from anxiety or depression. Thirty percent patients had memory decline. Interictal epileptiform discharges appeared predominantly in the anterior or inferior temporal area ipsilateral to AE. Interictal FDG-PET showed regional glucose hypometabolism in the ipsilateral temporal lobe. No hippocampal sclerosis (HS) was suspected in all patients. 22 patients demonstrated good seizure control and significantly reduced volume of the enlarged amygdala after treatment (P < 0.01). The other 11 patients showed initial response to treatment, followed by a gradual increase in seizure frequency over time, and no volume change of the enlarged amygdala after treatment. CONCLUSIONS: TLE with AE probably represents a distinct nosological and probably less homogeneous syndrome which is most likely a subtype of TLE without ipsilateral HS. The chronic and long lasting inflammatory processes or focal cortical dysplasia could lead to amygdala enlargement possibly.


Subject(s)
Amygdala/pathology , Epilepsy, Temporal Lobe/pathology , Adult , Aged , Epilepsy, Temporal Lobe/classification , Epilepsy, Temporal Lobe/physiopathology , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Young Adult
18.
Neurol Sci ; 35(5): 777-80, 2014 May.
Article in English | MEDLINE | ID: mdl-24366242

ABSTRACT

Somatosensory-evoked reflex epilepsy is characterized by seizures in response to specific stimuli. It is highly uncommon for somatosensory-evoked focal seizures to be caused by movement or a change in posture. Reflex epilepsy induced by both somatosensory and proprioceptive stimulations has not been previously reported. In this study, we present a case of reflex epilepsy evoked by somatosensory and proprioceptive stimulation in a patient with hypertrophic cranial pachymeningitis. After comparing our patient with other cases of previously reported somatosensory-evoked reflex epilepsy, we determined that our patient had an unusual cause of reflex epilepsy.


Subject(s)
Epilepsy, Reflex/complications , Epilepsy, Reflex/diagnosis , Meningitis/complications , Meningitis/diagnosis , Adult , Brain/pathology , Electroencephalography , Epilepsy, Reflex/pathology , Epilepsy, Reflex/physiopathology , Humans , Magnetic Resonance Imaging , Male , Meningitis/pathology , Meningitis/physiopathology , Tomography, X-Ray Computed
19.
Int J Surg ; 110(1): 306-314, 2024 Jan 01.
Article in English | MEDLINE | ID: mdl-37800596

ABSTRACT

BACKGROUND: Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) and traditional open surgery (OS) are effective and safe options for patients with drug-resistant mesial temporal lobe epilepsy (DR-mTLE). However, their superiority in seizure control and preservation of functional abilities remains unclear. This study aimed to compare the surgical outcomes of MRgLITT and OS. MATERIALS AND METHODS: This multicenter retrospective cohort study included patients with DR-mTLE who underwent MRgLITT or OS at three centres between 2015 and 2023. The data on patient demographics, presurgical non-invasive evaluation, stereoelectroencephalography (SEEG) implantation, memory alteration, and seizure outcomes were collected. Propensity score matching (PSM) analysis was conducted for the comparison of seizure control and functional preservation between two surgical approaches. RESULTS: Of the 244 individuals who met the study criteria, 33 underwent MRgLITT and 211 OS. The median (interquartile range) age at seizure onset was 22.0 (13.0) and 12.3 (10.0) years in the MRgLITT and OS groups, respectively. The first PSM, based on demographic and non-invasive information, resulted in 26 matched pairs for the primary analysis. There were no significant differences in memory preservation ( P = 0.95) or surgical outcomes ( P = 0.96) between the groups. The second PSM, based on demographics and SEEG implantation, yielded 32 matched pairs for the sensitivity analysis, showing similar results. Subset analysis of early and late MRgLITT cases revealed no statistically significant differences in the proportion of patients with memory decline ( P = 0.42) or seizure control ( P = 1.00). Patients who underwent SEEG implantation were 96% less likely to achieve seizure freedom after MRgLITT ( P = 0.02). CONCLUSION: Minimally invasive MRgLITT is associated with memory preservation and seizure control, similar to traditional OS. MRgLITT is effective and safe for DR-mTLE and is relevant for future prospective randomized trials on dominant-side mTLE, providing practical implications for guiding neurosurgeons in the selection of surgical approaches.


Subject(s)
Drug Resistant Epilepsy , Epilepsy, Temporal Lobe , Laser Therapy , Humans , Epilepsy, Temporal Lobe/surgery , Retrospective Studies , Propensity Score , Treatment Outcome , Laser Therapy/methods , Drug Resistant Epilepsy/surgery , Magnetic Resonance Imaging/methods , Seizures , Magnetic Resonance Spectroscopy , Lasers
20.
Neurol Ther ; 13(1): 107-125, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38019380

ABSTRACT

INTRODUCTION: Cognitive impairment (CI) is a common comorbidity in patients with late-onset epilepsy of unknown origin (LOEU). However, limited data are available on effective screening methods for CI at an early stage. We aimed to develop and internally validate a nomogram for identifying patients with LOEU at risk of CI and investigate the potential moderating effect of education on the relationship between periventricular white matter hyperintensities (PVHs) and cognitive function. METHODS: We retrospectively reviewed the clinical data of 61 patients aged ≥ 55 years diagnosed with LOEU. The main outcome was CI, reflected as an adjusted Montreal Cognition Assessment score of < 26 points. A nomogram based on a multivariable logistic regression model was constructed. Its discriminative ability, calibration, and clinical applicability were tested using calibration plots, the area under the curve (AUC), and decision curves. Internal model validation was conducted using the bootstrap method. The moderating effect of education on the relationship between PVH and cognitive function was examined using hierarchical linear regression. RESULTS: Forty-four of 61 (72.1%) patients had CI. A nomogram incorporating seizure type, total cerebral small vessel disease burden score, and PVH score was built to identify the risk factors for CI. The AUC of the model was 0.881 (95% confidence interval: 0.771-0.994) and 0.78 (95% confidence interval: 0.75-0.8) after internal validation. Higher educational levels blunted the negative impact of PVH on cognitive function. CONCLUSION: Our nomogram provides a convenient tool for identifying patients with LOEU who are at risk of CI. Moreover, our findings demonstrate the importance of education for these patients.

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