Drug resistant epilepsy remains a major clinical challenge. Diverse criteria have been used to defi ne drug resistance by different researchers, making it diffi cult or even impossible to compare the results across different studies. To improve patient care and facilitate clinical research, the International League Against Epilepsy recently proposed a consensus defi nition to defi ne drug resistant epilepsy as failure of adequate trials of two tolerated, appropriately chosen and used antiepileptic drug schedules (whether as monotherapies or in combination) to achieve sustained seizure freedom. This article outlines the framework of the consensus defi nition, explains how to apply it in practice, and discusses the future development in its use.

We reported the experience of epilepsy su�rgery program in 3 centers in Hong Kon��� g They are Prince of Wales Hospital, Queen Elizabeth Hospital and Tuen Mun Hospital. The results of surgery are comparable to best centre elsewhere in the world. Hong Kong has a population of 7 million people with estimated surgical candidates of 3,500����. With e�stimated le�ss than 150 epilep�sy ��sur�geries performed in Hong Kong so far, there is room for further development of epilepsy surgery service in Hong Kong

Strong association between HLA B*1502 and carbamazepine-induced Steven-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) was demonstrated among Han Chinese in 2004. Studies from Europe showed that the HLA B*1502 is not a universal marker for SJS/TEN, but is ethnicity specific for Asians. Reports across Asia has shown that the prevalence of HLA B*1502 is high among Han Chinese (5-15%), Malays (12-15%), and Thais (8-27%), but low among Japan, Korea, Sri Lanka, and most ethnic groups in India. Other than Han Chinese, the association between HLA B*1502 and carbamazepine-induced SJS-TEN is also seen among the Thais and Malay. There is urgent need for further studies to determine the prevalence of SJS/TEN, and HLA B*1502 in the various ethnic groups in Asia, and its association with carbamazepine-induced SJS-TEN in each of these ethnic groups. In view of the significant morbidity and mortality in SJS-TEN, facilities should be developed to allow for screening of HLA B*1502 before carbamazepine is prescribed to the Hans Chinese, Malays and Thais. For those who experience no adverse cutaneous reaction after 3 months use of carbamazepine, the risk of SJS/TEN is low, and the drugs can be continued.

In the treatment of refractory epilepsy,especially in Dravet syndrome and Lennox-Gastaut syndrome,cannabidiol,an anticonvulsant component of marijuana,has shown significant efficacy and safety.However,its mechanism of action and interaction with other drugs are still unclear.For refractory patients who have been treated with a variety of antiepileptic drugs,cannabidiol makes their treatment more promising but also more complicated.This review focuses on the mechanism of cannabidiol and its clinical research progress in the treatment of refractory epilepsy.

BACKGROUNDChildhood cancer survivors were at risk of development of second malignant neoplasms. The aim of this study is to evaluate the incidence, risk factors and outcome of second malignant neoplasms in childhood cancer survivors in a tertiary paediatric oncology centre in Hong Kong, China.

METHODSWe performed a retrospective review of patients with childhood cancer treated in Children's Cancer Centre in Prince of Wales Hospital, Hong Kong, China between May 1984 and June 2009. Case records of patients who developed second malignant neoplasms were reviewed.

RESULTSTotally 1374 new cases aged less than 21-year old were treated in our centre in this 25-year study period. Twelve cases developed second malignant neoplasms with 10-year and 20-year cumulative incidence of 1.3% (95% confidence interval 0.3% - 2.3%) and 2.9% (95% confidence interval 1.1% - 4.7%) respectively. Another 4 cases were referred to us from other centres for the management of second malignant neoplasms. In this cohort of 16 children with second malignant neoplasms, the most frequent second malignant neoplasms were acute leukemia or myelodysplastic syndrome (n = 6) and central nervous system tumor (n = 4). Median interval between diagnosis of primary and second malignant neoplasms was 7.4 years (range 2.1 - 13.3 years). Eight patients developed second solid tumor within the previous irradiated field. Radiotherapy significantly increased the risk of development of second solid tumor in patients with acute lymphoblastic leukemia (P = 0.027). Seven out of 16 patients who developed second malignant neoplasms had a family history of cancer among the first or second-degree relatives. Nine patients died of progression of second malignant neoplasms, mainly resulted from second central nervous system tumor and osteosarcoma.

CONCLUSIONSCumulative incidence of second cancer in our centre was comparable to western countries. Radiotherapy was associated with second solid tumour among patients with acute lymphoblastic leukemia. Patients who developed second brain tumor and osteosarcoma had a poor outcome.

Adolescent ; Adult ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Hong Kong ; epidemiology ; Humans ; Infant ; Infant, Newborn ; Male ; Neoplasms ; epidemiology ; Neoplasms, Second Primary ; epidemiology ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Retrospective Studies ; Survivors ; statistics & numerical data ; Young Adult

Epilepsy is a common, chronic neurological disorder that has been associated with impaired neurodevelopment and immunity. The chemokine receptor CXCR5 is involved in seizures via an unknown mechanism. Here, we first determined the expression pattern and distribution of the CXCR5 gene in the mouse brain during different stages of development and the brain tissue of patients with epilepsy. Subsequently, we found that the knockdown of CXCR5 increased the susceptibility of mice to pentylenetetrazol- and kainic acid-induced seizures, whereas CXCR5 overexpression had the opposite effect. CXCR5 knockdown in mouse embryos via viral vector electrotransfer negatively influenced the motility and multipolar-to-bipolar transition of migratory neurons. Using a human-derived induced an in vitro multipotential stem cell neurodevelopmental model, we determined that CXCR5 regulates neuronal migration and polarization by stabilizing the actin cytoskeleton during various stages of neurodevelopment. Electrophysiological experiments demonstrated that the knockdown of CXCR5 induced neuronal hyperexcitability, resulting in an increased number of seizures. Finally, our results suggested that CXCR5 deficiency triggers seizure-related electrical activity through a previously unknown mechanism, namely, the disruption of neuronal polarity.
Animals ; Humans ; Mice ; Actin Cytoskeleton/metabolism* ; Actins/metabolism* ; Epilepsy/metabolism* ; Neurons/metabolism* ; Receptors, CXCR5/metabolism* ; Seizures/metabolism*

Background: and Purpose Recent studies suggested an increased incidence of cerebral venous thrombosis (CVT) during the coronavirus disease 2019 (COVID-19) pandemic. We evaluated the volume of CVT hospitalization and in-hospital mortality during the 1st year of the COVID-19 pandemic compared to the preceding year. Methods: We conducted a cross-sectional retrospective study of 171 stroke centers from 49 countries. We recorded COVID-19 admission volumes, CVT hospitalization, and CVT in-hospital mortality from January 1, 2019, to May 31, 2021. CVT diagnoses were identified by International Classification of Disease-10 (ICD-10) codes or stroke databases. We additionally sought to compare the same metrics in the first 5 months of 2021 compared to the corresponding months in 2019 and 2020 (ClinicalTrials.gov Identifier: NCT04934020). Results: There were 2,313 CVT admissions across the 1-year pre-pandemic (2019) and pandemic year (2020); no differences in CVT volume or CVT mortality were observed. During the first 5 months of 2021, there was an increase in CVT volumes compared to 2019 (27.5%; 95% confidence interval [CI], 24.2 to 32.0; P<0.0001) and 2020 (41.4%; 95% CI, 37.0 to 46.0; P<0.0001). A COVID-19 diagnosis was present in 7.6% (132/1,738) of CVT hospitalizations. CVT was present in 0.04% (103/292,080) of COVID-19 hospitalizations. During the first pandemic year, CVT mortality was higher in patients who were COVID positive compared to COVID negative patients (8/53 [15.0%] vs. 41/910 [4.5%], P=0.004). There was an increase in CVT mortality during the first 5 months of pandemic years 2020 and 2021 compared to the first 5 months of the pre-pandemic year 2019 (2019 vs. 2020: 2.26% vs. 4.74%, P=0.05; 2019 vs. 2021: 2.26% vs. 4.99%, P=0.03). In the first 5 months of 2021, there were 26 cases of vaccine-induced immune thrombotic thrombocytopenia (VITT), resulting in six deaths. Conclusions During the 1st year of the COVID-19 pandemic, CVT hospitalization volume and CVT in-hospital mortality did not change compared to the prior year. COVID-19 diagnosis was associated with higher CVT in-hospital mortality. During the first 5 months of 2021, there was an increase in CVT hospitalization volume and increase in CVT-related mortality, partially attributable to VITT.