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Objective To evaluate the clinical and paraclinical features of Chinese patients with anti- LGI1 encephalitis. Methods Patients with memory deficits, psychiatric symptoms, seizures or altered level of consciousness, suspicious of encephalitis, at presentation to Peking Union Medical College Hospital were recruited between July 2013 and January 2018, and tested for anti-LGI1 antibodies in their serum and/or cerebrospinal fluid(CSF) samples. Patients with anti-LGI1 antibodies were enrolled. The demographic characteristics, clinical manifestations, laboratory examination results, neuroimaging features, immunotherapy, follow-up practices and outcomes for included patients were registered and analyzed. Results The study enrolled 120 patients, of whom 66.7% were male. The median age was 61 years (interquartile range [IQR]: 49-66 years). Seizures(65.0%) were the most common initial symptoms. Most patients developed seizures (95.0%), including faciobrachial dystonic seizures (54.2%), memory deficits (92.5%), and psychiatric symptoms (69.1%). Brain MRI and 18F-FDG PET / CT showed that the lesions were mainly located in unilateral or bilateral medial temporal lobes, and (or) basal ganglia. Of the patients, 95.0% received intravenous immunoglobulin (IVIg) or corticosteroids, 47.5% received mycophenolate mofetil as long-term immunotherapy, and no one received second-line immunotherapy. The median follow-up was 34.2 months(IQR: 22.0-45.6 months). 91.2% had a good outcome (modified Rankin Scale score≤2 points). Residual mild memory deficits were present in 47.8% of the patients. Nine deaths were documented. Relapses occurred in 24.8% of the patients in the first year. In total, 24 (20%)cases were young patients(onset age ≤45 years).There were fewer males among the younger patients(37.5% vs. 74.0%, P < 0.01). Besides, there were fewer younger patients with psychiatric symptoms(50.0% vs. 74.0%, P=0.02), hyponatremia(33.3% vs. 68.8%, P < 0.01), and abnormalities on brain 18F-FDG PET/CT(20.8% vs. 47.9%, P=0.02). The relapse-free survival rate was significantly higher in the young patients. Conclusions Elderly males were predominant in patients with anti-LGI1 encephalitis. Most patients developed symptoms of limbic encephalitis and/or FDBS during the disease course. Several patients were young adults and lacked typical symptoms. Neuroimaging features were consistent with the involvement of limbic system or basal ganglia. Patients with anti-LGI1 encephalitis respond well to immunotherapy, irrespective of the age.
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Objective To analyze the clinical and histology characteristics of a patient with frontal lobe epilepsy diagnosed with mild malformation of cortical development with oligodendroglial hyperplasia, and to recognize the new neuropathological entity. Methods Clinical history, seizure types, neuroimaging, electroencephalography as well as macroscope, histology and immunohistochemistry characteristics were collected from a frontal lobe epilepsy patient and were compared with cases from literature. Results It was a female patient aged 16 years with 12 years history of epilepsy. The seizures manifested as episodes of conscious loss with automatism including grope and voice lasting for seconds. About 10 episodes a day were found and sometimes with secondary generalized tonic-clonic seizures. MRI showed blurring of grey-white matter interface in left orbital frontal cortex. Video-encephalography revealed left frontal lobe origin of seizures. So left prefrontal lobe was removed. Histology showed almost normal cortex neuropil and neurons. Blurring of grey-white interface in some area with patches of proliferation of oligodendrocytes in the corresponding sub-cortical white matter was found. The density of oligodendrocytes was significantly higher in sub-cortical than in deep white matter both shown in HE and Oligo-2 staining. Obvious oligodendrocytes increase and satellite phenomenon in deep cortical layer as well as increased ectopic neurons in sub-cortical white matter were found in the lesion. In proliferation area, there were some nuclei stained with Ki-67, but not as high as tumor. Subsequent follow up for two years proved the operation efficacy and benign prognosis. Conclusions There are special and undiscovered histopathological entities in epilepsy etiology. Although known as grey matter disease, white matter pathology plays an important role in epilepsy pathophysiology which needs further research.
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Objective To evaluate the efficacy and safety of mycophenolate mofetil (MMF) in antileucine-rich glioma-inactivated 1 (LGI1) encephalitis by analyzing the clinical and immunologic data of patients treated with MMF in this prospective cohort of anti-LGI1 encephalitis.Methods Patients treated with MMF for more than one year in Peking Union Medical College Hospital were included in this study.MMF was given at a dosage of 1.5-3.0 g/d in the induction period (two to four months) and 0.75-2.00 g/d in the maintenance period.All the patients were followed up regularly.Modified Rankin Scale (mRS) score evaluation,serum IgG and peripheral CD19-positive B cells,CD4-positive T cells and CD8-positive T cells testing were performed every two months.Results Fifteen patients were included in this study who received first-line immunotherapy combined with MMF.No other second-line therapy including rituximab was used.Thirteen patients responded well to MMF combined with first-line immunotherapy (a decrease in mRS score of more than 1).All 15 patients had a good outcome (i.e.,a mRS score of 0-2),including nine patients without residual symptoms (a mRS score of 0).After 12 months of MMF treatment,CD19-positive B cells were significantly decreased (median 320 (227,628) × 106/L vs 152 (105,223) × 106/L;Z=-2.028,P=0.043),while serum IgG (9.07 (6.70,11.32) g/L vs 8.35 (6.63,10.69) g/L,P=0.144)),CD4-positive T cells (1 136 (736,1 432) × 106/L vs 1 055 (802,1 072) × 106/L,P =0.866) and CD8-positive T cells (627 (413,784) × 106/L vs 568 (393,743) × 106/L,P =0.735) were not significantly changed.Three patients relapsed and were treated with additional cycle of first-line immunotherapy and increased dosage of MMF (induction dosage) resulting in remission.CD19-positive B cells were tested to be increased during the patients' relapse.No serious adverse event was noted in all these patients.Conclusions MMF is safe and effective as a long-term immunotherapy in patients with anti-LGI1 encephalitis.MMF can be used as an add-on therapy to first-line immunotherapy for autoimmune encephalitis.CD19-positive B cell count should be monitored and used as a parameter to individualize dosage of MMF.
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Objective To find out whether conversation analysis helps to differentiate psychogenic nonepileptic seizure (PNES) from epileptic seizure in Chinese patients.Methods Twelve unselected patients from Peking Union Medical College Hospital during 2014 to 2016 with diagnostic uncertainty were included.Interactions following standard protocol were carried out.A linguist blinded to all medical data and a neurologist studied videos and transcripts of the interactions.Using a diagnostic scoring aid which includes 17 conversation features summarized from previous researches, they attempted to predict the medical diagnosis of those patients independently.Results Accurate diagnosis was predicted in 10/12 patients by both raters.Average scores of patients with epileptic seizures were 8.00 (linguist) and 6.75 (neurologist), while average scores of paitents with PNES were-5.75 (linguist) and-7.88 (neurologist).Both raters agreed on most individual items (81.86%, 167/204).To demonstrate different features between these two groups, a case comparison was made between one patient with frontal lobe epilepsy and one patient with PNES.Conclusion In Chinese patients, conversation analysis can help differentiate between epileptic seizure and PNES.
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Objective To investigate the results and complications of hemispherotomy for drug resistant hemispheric epilepsy.Methods The authors reviewed 5 patients who were diagnosed as drug resistant hemispheric epilepsy and operated in the neurosurgery department of Peking Union Medical College Hospital from 2011 to 2013.All the 5 patients were underwent hemispherotomy after carefully multidisciplinary pre-operation evaluation.Results All patients tolerated the procedure well and the postoperative course was smooth.All the 5 patients didn`t have seizure in the period of following up of 46 to 69 months.Conclusions According to literatures and the authors` experience, hemispherotomy is as safe and efficient for hemispheric epilepsy as hemispherectomy.It is most important advance for hemispherectomy.The procedure of hemispherotomy is complex but not very difficult, illustrating a good prospect of application and extension.
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Objective To investigate the lateralizing value of head deviation(HD) during complex partial seizures (CPS) in patients with refractory mesial temporal lobe epilepsy (mTLE).Methods Presurgical videotypes of 43 patients who were seizure-free for at least one year after temporal lobectomy were retrospectively reviewed.Attention was paid to the relationship between time and type of HD and the side of epileptogenic zone.Results HD was seen in 88 CPS from 43 patients who had total 206 CPS with or without secondary generalization.Both versive and non-versive HD displayed high positive predictive value (83％ (33/40) and 88％ (22/25)) for localization of an ipsilateral and contralateral seizure onset,respectively.Conclusion Both non-versive HD and versive HID during CPS in patients with mTLE are reliable lateralizing signs that can complement other diagnostic modalities in presurgical evaluation.
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Objective To compare the classification rate of three classifications of epilepsy syndromes proposed by International League Against Epilepsy(ILAE),and analyze their stuctural changes.Methods All patients with epilepsy who consecutively visited the epilepsy center of Peking Union Medical College Hospital between Aug.1st,2007 and Mat.31st.2008 were included.Thtee classifications of epilepsy syndromes were used in order.Results In this study,we could categorize 75.5 % of 1356 patients by applying the 1989 international classification of epilepsy syndromes.89.0 % of them by the 2001 proposed diagnostic scheme and 88.1 % of them by the 2006 report.In this aspect,the 2001 and 2006 classifications were better than the 1989 classification(x2=116.3,P<0.01).However,only 11.6 % (157),12.O % (162)and 11.9 % (160)of patients with specific epilepsy syndromes were identified from the 1356 epileptic patients by three classifications.respectively.This data based on the 2001 and 2006 classifications did not change markedly in comparison with the 1989 classification(x2=0.09,P>0.05).Conclusions The 2006 report involve mole scientific mode of classification and systematic evaluation,and can classify more patients with epilepsy.It can be ased in clinical and scientific research.which can not only accumulate data for developing more scientific classification but also stimulate research especially in the fields of genetics and functional morphology.
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Objective To investigate clinical features and the pathophysiology of 43 children with atypical idiopathic partial epilepsies of childhood(IPEC)that is unclassified according to the International League Against Epilepsy classification.Methods All the children with partial epilepsy of childhood in out hospital were followed up,including those age-related cases with benign process and excluding those with benign epilepsy of childhood with centro-temporal EEG foci,Panayiotopoulos type and Gastaut type.We reviewed their EEGs,seizures and therapeutic data to analyze the semiologieal characters and the interietal activities during they were awake and sleeping.Results The average age of onset of epilepsy was 8.84years old.Frontal absences(43.2%),adversive seizures(47.7%)were more common.Everyday seizures occurred in 38.6%of patients and monthly seizures in 56.8%of patients.Atypical focal(43.2%),multifocal(27.3%),and generalized spike and waves(29.5%),were more predominant in frontal location when they were awake.During sleep,sharp waves generalized or the amplitude increased.At the last follow-up,88.6%of patients were in complete clinical remission and EEG in 22.7%of cases was normal.Among them 2 patients had stopped taking antiepileptic drugs(AEDs)and 6 patients were reducing the doses of AEDs.EEG Was abnormal only in sleep or decreased synchronization.The patients were more responsive to earbamazepine combined with sodium valproate(P<0.01).Conclusion Special partial epileptic syndrome is age-related,having excellent prognosis,which might origin from the frontal lobe.
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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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A reappraisal was made with respect to a classical observation of the mode of instrumental phase reversals on inter-ictal EEG of seemingly bilateral synchronous spike-wave discharges in patients with either idiopathic generalized epilepsies (IGE) or symptomatic localization-related frontal lobe epilepsies (FLE). It was pointed out in the original observation by Tukel and Jasper that one phase reversal at midline or near the midline on the side of the parasagittal epileptogenic lesion designated as secondary bilateral synchrony (SBS) was found in patients with frontal lobe epilepsy (FLE), whereas a double phase reversal was found over the homologous frontal electrodes (F3 and F4) designated as primary bilateral synchrony (PBS) in patients with IGE. Twenty-three patients (IGE: 15, and FLE: 8) revealing bursts of seemingly bisynchronous spike-wave discharges in interictal EEGs were retrospectively studied. Discharge patterns were defined as stable phase reversal pattern if the site of phase-reversal was consistent, and as unstable pattern if the site of phase-reversal was not consistent but shifting in the same patient. Stable one phase-reversal pattern was found more frequently in FLE (50%) than in IGE patients (26.7%), and stable double phase-reversal pattern more frequently in the IGE (33.3%) than in the FLE group (12.5%). Notably, unstable pattern was found almost equally in both IGE and FLE patients (40% and 37.5%, respectively). Recognition of SBS or PBS in accordance with original observation was found not to clearly differentiate FLE from IGE in patients showing seemingly bisynchronous spike-wave complexes. The variability of instrumental phase-reversals can be accounted for by the fact that the localization of maxima of negative spike of the spike-and-wave complexes varies considerably.
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Immunoglobulin E , Tidal WavesABSTRACT
Objective To assess the value of ictal video-electroencephalography monitoring (IVEEG) in presurgical evaluation for medically intractable nonlesional temporal lobe epilepsy(TLE). Methods The data of presurgical evaluation and postsurgical follow-up from 15 patients with intractable TLE were retrospectively analyzed.Results In this selected patients group, all 15 patients manifested clinically seizures during long term VEEG monitoring, with initial rhythmic activity from one side of temporal lobe in 13 cases. Of these 13 patients, the side of initial ictal focal pattern was concordant with the side of anterior temporal lobectomy or selective amygdalohippocampectomy in all cases and excellent postsurgical outcome were attained in 11 patients.Conclusion IVEEG monitoring may not only help to confirm the diagnosis and seizure type(s),but also more important to correctly localize the side of epileptogenic region in most patients with intractable TLE.
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Objective To investigate the features of clinical and EEG changes during follow up in patients with BECCT. Correlative analysis between the disease duration and some factors were also performed.Methods The clinical and EEG data of 87 recovered patients with BECCT were retrospectively analyzed.Results The EEG and clinical manifestations are mostly characteristic. Clinical seizures disappeared early before the rolandic spikes of EEG in most patients,showing a longest duration of 6 years. There was only the onset age being correlative with the duration of disease in both seizures and abnormal EEG. Medication was withdrawn after there appeared seizures free for more than two years and a recovery of EEG. However medication can also be stopped even at patient′s EEG still remaining abnormal.Conclusion BECCT is an age dependent, idiopathic epilepsy syndrome in childhood, comprehensively recognizing the clinical and EEG features should be helpful in clinical practice.