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Objective:To investigate the clinical and electrophysiological characteristics of patients with sudden unexpected death of epilepsy (SUDEP).Methods:Using "epilepsy" as the keyword, the relevant cases entered from October 2011 to March 2012 were searched in the database of the Electroencephalogram (EEG) Monitoring Center, Xijing Hospital, the Air Force Military Medical University. Telephone follow-up was conducted for all confirmed epilepsy patients, and for the death cases confirmed by telephone follow-up, the patients identified as consistent with SUDEP diagnosis were included in this study based on their past medical history, clinical data, death details, etc, and their clinical and neuroelectrophysiological characteristics were summarized and analyzed.Results:Among the 1 232 patients who underwent 24-hour video-EEG monitoring during the study period, 354 patients were successfully followed up by telephone interview, of whom 17 patients were died (4.8%), 12 individuals met the diagnosis of SUDEP (7 men, 5 women). The duration of the disease in 9 patients exceeded 10 years. Eight cases presented with focal-bilateral tonic clonic seizures. Nine patients were treated with anti-seizure drug monotherapy. All the 24-hour video EEG of 12 patients were abnormal. There were 8 occasions when the EEG occipital α background rhythm slowed down compared with the standard frequency of peers or was dominated by slow waves. Interictal epileptic discharge (IED) located in temporal lobe were found in 12 EEG records, of which 9 EEG records were found with frontal IED. One of the 12 cases received 24-hour video EEG twice within 6 years, and his EEG background rhythm was significantly slower and the IED region was expanded compared with the first EEG record. At the third year after reexamination of EEG, SUDEP developed in this patient.Conclusions:SUDEP patients have a long course of disease and bilateral tonic-clonic seizure. The interictal EEG shows occipital slow α activity and temporofrontal epileptiform discharges, which may increase the risk of SUDEP.
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Objective To illustrate the semiological characteristics of the three sub-types within the broad bilateral asymmetric tonic seizures (BATS),summarize their predictive values on lateralization and localization of seizure onset zone (SOZ),and analyze the difference between BATS and asymmetrical tonic limb posturing (ATLP).Methods A retrospective review of 385 patients who underwent stereotactic electrode implantation in the Sanbo Brain Hospital,Capital Medical University from September 2011 to May 2018 was performed.As long as there was a clinical epileptic seizure in the presence of BATS or ATLP,the patients were classified into the corresponding groups.Postoperative prognosis was assessed using Engel's grading criteria for a follow-up of no less than six months.Seizure descriptions were based on the classification of epileptic seizures introduced by Lüiders,which used arrows to connect the symptoms in chronological order.Results There was no statistically significant difference between the classic BATS and bilateral proximal tonic seizure in terms of whether it could be an independent seizure,as the onset and end of the seizure,with version and generalized tonic-clonic seizure (P＞0.05).Compared with the ATLP,except for whether it could be an independent seizure (P=1.000) and onset before versive seizure (P=0.068),the BATS showed significantly different semiological features (P＜0.05).The classic BATS and secondary motor area epilepsy had a 100.0％ predictive accuracy on the lateralization of SOZ.In the patients with broad BATS,the SOZ distribution was more extensive,but it was rare in the orbitofrontal gyrus,frontal pole and mesial temporal lobe.Compared with the bilateral proximal tonic seizures from the other regions,those originated from supplementary somatosensory motor area and its adjacent areas were rare and showed no statistically significant difference (0/8 vs 40.0％ (18/45),x2=3.226,P=0.072) but a low trend.The predictive value of BATS on lateralization of SOZ was higher than that of ATLP (84.9％ (45/53) vs 57.1％ (24/42),x2=9.086,P=0.003),and BATS was less originated from temporal lobe than ATLP (3.8％ (2/53) vs 23.8％ (10/42),x2=8.523,P=0.004).Conclusion Different from ATLP,the broad BATS are characterized by tonic proximal upper limb posturing,and have a higher predictive value on lateralization and localization of SOZ.
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Objective To analyze the clinical features and prognosis of anti-leucine rich glioma inactivated protein 1 (LGI1) encephalitis.Methods Twelve encephalitis patients with anti-LGI1 antibodies were collected from the First Affiliated Hospital of Zhengzhou University from June 2015 to December 2016.The clinical manifestations,electroencephalogram,laboratory examination and imaging findings were summarized and the prognosis was observed.The modified Rankin Scale (mRS) was used for evaluation before and after treatment.Results The major clinical features included memory deficit (10/12),spatial disorientation (7/12),epilepsy with generalized tonic-clonic seizures (9/12),faciobrachial dystonic seizures (7/12),hyponatremia (5/12),mental and behavioral abnormalites (1/12),light sleep (1/12),increased sleep (3/12),aphasis (4/12),dysphagia,choking (2/12),headache (1/12),dizziness (2/12),fatigue (2/12),ataxia (2/12),bradycardia (3/12),urinary disorders (2/12),intestinal obstruction (1/12),diarrhea (1/12).Admission mRS score was found to be three in eight cases,four in four cases.The abnormal electroencephalogram was found in six cases,mainly manifested as focal or diffuse slow wave,some accompanied by epileptic wave.MRI scan of brain showed abnormal signals in four cases,mainly involved medial temporal lobe,hippocampus,basal ganglia,while one patient avoided MRI scan due to implantation of pacemaker.Two patients presented with pulmonary nodules,one case with positive thyroid antibody and increased rheumatoid factor.The follow-up after treatment showed no one died;mRS score was two in two cases,one in nine cases and zero in one case;the sequelae were memory deficit,increased sleep,faciobrachial dystonic seizures.Conclusions Anti-LGI1 encephalitis is a treatable disease,cardinal clinical features of which are seizures,cognitive disorders,hyponatremia.Immunotherapy can improve the symptoms of the disease significantly,and the prognosis is better comparatively.
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Objective To investigate the lateralizing value of figure"4"sign,an asymmetrical tonic posturing observed in secondarily generalized tonic-clonic seizures(sGTC),in patients with mesial temporal lobe epilepsy.Methods Presurgical videotapes of 54 sGTC from 33 patients who were seizure-free for at least 4 years after temporal lobectomy were retrospectively reviewed.Attention was paid to the relationship between the extended upper extremity in figure"4"sign and the resected side.Results The "4"sign was observed in 10(30%)of 33 patients and 23(43%)of sGTC.The extended arm in figure "4"sign was always contralateral to the epileptogenic foci(resected side).Conclusion"4"sign has a significant lateralizing value,when present,in this selected homogeneous group of patients.
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Objective To investigate whether the vohage-gated chloride channel CLC-2 gene— CLCN2 is associated with idiopathic generalized tonie-clonic seizures(often called a grand mal seizure, GME)of Jinuo people and Han people from Yunnan province.Methods Three regions,including Intron 2, Exon 5 and Exon 19(Intron 18),of CLCN2 were selected to conduct sequence analysis.The case-control study design was used to detect association between gene polymorphism and idiopathic generalized tonic- clonic seizures of Jinuo people and Han people from Yunnan province.Results No previously reported susceptible mutations were found in Intron 2,Exon 5 and Exon 19 in Jinuo people and Han people from Yunnan province.However we found a single nucleotide polymorphism(SNP)at site 146 of Intron 18. Case-control study were carried out,using this SNP.Distribution of the 3 genotypes(TT,TC,CC)has a significant difference between the IGTCS patients of Han people and the normal controls of Han people(9, 3,29 cases and 22,9,26,respectively,x~2=16.079,P
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Objective To explore sub-clinical items in evaluating the prognosis of epileptic patients,the study on differences of regional cerebral blood flow (rCBF) in both effective treated and incomplete-controlled patients with idiopathic generalized tonic clonic seizure (GTCS) was carried out.Methods Interictal rCBF measurements using 99m Tc-ethyl cysteinates dimmer (ECD) SPECT was performed on 29 effective treated idiopathic GTCS patients and 12 incomplete controlled idiopathic GTCS patients. The rCBF distribution was semi-quantitatively analyzed by regions of interest (ROIs) comparing with abnormal rate of interictal hypoperfusion rCBF,clinical seizure time and EEG.Results ROI analysis showed that rCBF decreased in basal ganglia and thalamus of incomplete controlled patients with idiopathic GTCS compared to that of effective treated ones′ significantly ( P