Statement of the Problem: Childhood epilepsy is one of the most common CNS diseases [1]. From the point of view of optimizing care for children with epilepsy and their families during and after the crisis caused by the COVID-19 pandemic, it is important not only the high frequency and severity of the pathology, but also its social consequences - stigmatization, some isolation of the child in many cases [2,3] and economic aspects [4,5].

The Cl--extruding transporter KCC2 (SLC12A5) critically modulates GABAA receptor signaling via its effect on neuronal Cl- homeostasis. Previous studies have shown that KCC2 was downregulated in both epileptic patients and various epileptic animal models. We discovered that the in vitro dual phosphorylation of Thr906 and Thr1007 in the intracellular carboxyl (C)-terminal domain of KCC2, mediated by the Cl--sensitive WNK-SPAK serine-threonine protein kinase complex, maintains the depolarizing action of GABA in immature neurons by antagonizing KCC2 Cl- extrusion capacity. GABAAR-mediated inhibition confines KCC2 to the plasma membrane, while antagonizing inhibition reduces KCC2 surface expression by increasing the lateral diffusion and endocytosis of the transporter. This mechanism utilizes Cl- as an intracellular secondary messenger and is dependent on phosphorylation of KCC2 at threonines 906 and 1007 by the Cl- -sensing kinase WNK1. We propose this mechanism contributes to the homeostasis of synaptic inhibition by rapidly adjusting neuronal [Cl-]i to GABAAR activity. We further demonstrate here that this signaling pathway is rapidly and massively activated in an acute epilepsy model. This indicates that dephosphorylation of KCC2 at Thr906 and Thr1007 is a potent activator of KCC2 activity, and small molecular targets WNK-SAPK kinase signaling may be a novel therapeutic strategy for epilepsy.

Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy characterized by Parkinsonism, vertical gaze palsy, and early falls. The neuropathology is characterized by neurofibrillary tangles, tufted astrocytes, and coiled bodies, but some brains show other pathologic processes. To investigate the frequency of a-synuclein pathology in PSP with immunohistochemistry and to report the clinical and pathological features of a case of PSP with concomitant Multiple system atrophy (MSA) (PSP/MSA), 290 cases of PSP were screened for a-synuclein pathology with immunohistochemistry. Double-labeling immunohistochemistry was performed on a case of PSP/MSA. Among the PSP cases screened for a-synuclein pathology, a single case of PSP/MSA was detected. The patient was an 86-year-old woman with clinical features consistent with PSP. She had no documented dysautonomia or cerebellar signs, and imaging studies were not diagnostic of MSA. Pathological examination showed s-immunoreactive neuronal and glial lesions consistent with PSP as well as a-synuclein immunoreactive glial cytoplasmic inclusions diagnostic of MSA. Double immunolabeling studies showed no co-localization of a-synuclein and s in most neuronal and glial lesions. Based upon the findings in this case, the neuropathologic changes of PSP and MSA are distinct and independent processes, but they can occasionally coexist.

There are many causes of seizures in autoimmune disorders of brain, and the first clinical Seizures are among the most common neurological manifestation. Occasionally seizures can be the presenting symptom, convert into epilepsy, may be detected up to 14% of epilepsy patient, which could herald a life-threatening progression of the underlying illness. However, existing criteria for autoimmune epilepsy are too reliant on antibody testing and response to immunotherapy, which might delay the diagnosis. Because autoantibody test results and response to therapy are not available at disease onset, the initial diagnostic approach should be on neurological assessment and conventional tests that are accessible to most clinicians. Through logical differential diagnosis, levels of evidence for autoimmune epilepsy (possible, probable, or definite) may be achieved, which can lead to prompt immunotherapy. Prompt recognition of these disorders is mandatory to offer the patient adopted therapeutic options. Neuronal surface antibody and intracellular antibody syndromes encompass a variety of disorders associated with severe epilepsy. These share clinical and neuroradiological features that pose challenges related to their recognition and treatment. On that basis, a target treatment can be started, anti-seizure drugs augmented with corticosteroids and intravenous immunoglobulin or plasma exchange as a first-line immunotherapy, followed by second-line drugs including rituximab, cyclophosphamide or mycophenolate mophetil, if the case. In children a prompt diagnosis and a targeted treatment may lead to a better clinical outcome.

Epilepsy in one of the most common neurological diseases and may result in poor quality of life (QOL) in case of lack of knowledge, negative attitude, and reduced practice. The objective of this study is the assessment of knowledge, attitude, and practice (KAP) of patients taking anti-epileptic drugs (AEDs). An observational cross-sectional study was conducted on the Lebanese patients taking AEDs. The QOL score was assessed and calculated using the quality of life 10 instruments (QOLIE-10) questionnaire. Among the 150 patients included in the study, 60% took carbamazepine as AEDs and 62% were on a monotherapy. The mean of the QOLIE-10 score is 23.92 ± 7.17 which is indicates a good QOL. The mean of the knowledge sub-score is 5.14 ±1.68, the mean of the attitude sub-score is 6.72 ± 1.15, and the majority of patients (82%) always follow health care provider’s instructions about medication. The mean of the practice sub-score is 8.13 ± 1.78. The multiple linear regression carried out revealed that practice (p-value <0.001) is significantly associated with QOLIE-10 score. Being married (p-value < 0.001) and not being employed (p-value = 0.009) also affected the QOLIE-10 score. Treatment of epilepsy should include other health dimensions in addition to maintaining seizure free intervals. This study may be helpful to health care professionals in identifying the areas where the QOL of PWE can be improved; especially the impact of the practice that should be taken by them since it significantly affected the QOL as well as increasing their knowledge and favoring positive attitude towards epilepsy.