Case reports of identical twins with developmental and epileptic encephalopathy with STXBP1 gene mutations for whom different CBD supplementations were markedly effective.

Cannabidiol (CBD) is a compound found specifically in the cannabis plant. Although a clinical trial for intractable epilepsy started in Japan in 2023, it is also available in the market as a dietary supplement. Herein, we report two cases of identical twins with developmental and epileptic encephalopathy with gene mutation who achieved seizure suppression through different regimens of CBD supplementation.

Epilepsy with Eyelid Myoclonia Complicated by Polycystic Ovary Syndrome: Valproate Withdrawal, Vagus Nerve Stimulation, and Seizure Reduction.

Epilepsy with eyelid myoclonia is a childhood-onset generalized epilepsy, which is more common in women. Over 90% of the patients continue antiseizure medications, especially valproate, and more than 60% of cases are refractory. The efficacy of vagus nerve stimulation in treating eyelid myoclonia is still unknown.

Incidence and Prevalence of Epilepsy in Japan: A Retrospective Analysis of Insurance Claims Data of 9,864,278 Insured Persons.

Introduction: The burden of epilepsy is thought to be high but is difficult to measure. Very few studies in Japan have attempted to estimate prevalence and incidence rates of epilepsy in Japan.

Methods: This retrospective cohort study used commercially collected nationwide insurance claims data from a cohort of 10 million persons between 2012 and 2019 among those aged 0 to 74 years.

[Creutzfeldt-Jakob Disease and Lyodura:A Special Reference to Prion Disease Control in the Field of Neurosurgery].

In Japan, 156 cases of dura mater-transplanted Creutzfeldt-Jakob disease(dCJD)with a history of Lyodura transplantation have been confirmed until February 2022, with only a few new cases still being identified. The history of Lyodura transplantation is one involving a neurosurgical procedure. The cumulative global number of cases of bovine spongiform encephalopathy-related variant CJD(BSE-related vCJD), which has shaken societies around the world, is 232 as of 2019.

Patient complexity, depression, and quality of life in patients with epilepsy at an epilepsy center in Japan.

Objective: The relationship between care complexity and quality of life among patients with epilepsy has not been assessed, especially in Japan. The aim of this study is to test the hypothesis that care complexity is associated with health-related quality of life (HRQOL) and mood disturbance.

Method: This was an observational cross-sectional study.

Left Transradial Neurointerventions Using the 6-French Simmons Guiding Sheath: Initial Experiences with the Interchange Technique.

Background: The transradial approach using a Simmons-shaped catheter has been increasingly used for diagnostic cerebral angiography and neurointervention. In the transradial approach for neurointervention, the right radial artery is mainly used. However, in interventional cardiology, there are apparent clinical benefits with the left transradial approach for right-handed patients.

Analysis of postprocedural microembolic infarctions and global oxygen extraction fraction during balloon-protected carotid artery stenting: Preliminary study.

Background: Atherosclerotic carotid stenosis with impaired cerebral perfusion is a risk factor for cerebral ischemia. In major carotid stenoocclusive diseases, increased oxygen extraction fraction (OEF) is associated with ischemic stroke. Balloon-protected carotid artery stenting (CAS) is valuable for high-grade carotid stenosis.

Calcium Phosphate Cement "Space Fill-in" Augmentation in Autologous Cranioplasty for Large Cranial Defect: Additional Technical Consideration and Its Long-term Follow-up.

In the skull tumor surgery that requires a large cranial reconstruction, economical one-time surgery is challenging. Calcium phosphate paste (CPC) alone is not applied in the large defect. Other plastic fill-in materials have each drawback.

Report of a 6-month-old Asian infant with early infantile epileptic encephalopathy whose seizures were eliminated by cannabidiol.

We observed that cannabidiol supplements were highly effective in treating an infant boy with drug-resistant early infantile epileptic encephalopathy, eliminating his intractable tonic seizures. The infant began suffering clusters of brief tonic seizures from birth at 39 weeks gestation. EEG showed burst-suppression and seizures could not be controlled by trials of phenobarbital, zonisamide, vitamin B6, clobazam, levetiracetam, topiramate, phenytoin, valproate, high-dose phenobarbital, and ACTH therapy.

Risk Factors of Cerebellar Microembolic Infarctions After Carotid Artery Stenting.

Background: This study analyzes the incidence of microembolic infarctions (MIs) in the cerebellum after carotid artery stenting (CAS) to determine the risk factors.

Methods: From 2012 to 2019, 162 CASs in 155 patients were performed at our hospital. Fifty-seven patients (35.

Effect of total callosotomy on KCNQ2-related intractable epilepsy.

Aim: To describe beneficial effects of callosotomy on KCNQ2-related intractable epilepsy.

Case Report: Our patient was a 10-year-old girl who had developed epilepsy during the neonatal period, accompanied by a suppression-burst pattern on the electroencephalography (EEG). The patient showed profound psychomotor developmental delay since early infancy.

A case of tandem stenoses at the proximal common and internal carotid arteries treated with transbrachial stenting: a case report.

The transfemoral approach is a common technique for carotid artery stenting. However, it has the risk of distal embolism when stenting for a stenosis of the proximal common carotid artery because of poor stability of the guiding catheter resulting in difficulty in setting the embolic protection device prior to stenting. We present a novel therapeutic approach and technique for the treatment of tandem carotid stenoses including the proximal common carotid artery.

Pedicle Galeo-pericranial Flap Augmentation in Salvage Frontotemporal Cranioplasty: Additional 'Neurosurgeon-friendly' Reconstruction Technique of Aesthetic Neurosurgery in Superficial Temporal Artery Branch Compromised Host.

This technical note aims to demonstrate the usefulness, indications and its limitations of augmentation technique by bipedicle galeo-pericranial rotation flap and by monopedicle galeo-pericranial flap, both in STA (superficial temporal artery) branch compromised hosts in salvage frontotemporal cranioplasty. Although these flaps are not always idealistically vascularized owing to accidental injuries to the STA branches during previous surgeries, they are properly augmenting after salvage frontotemporal craniotomy when infection is not active. The procedure is indicated for salvage frontotemporal craniotomy when vasculature is needed at the surgical site, such as beneath the skin incision line in a thin injured scalp, onto the titanium plates or beneath the fragile fibrous scar.

Use of an Inverted and Segmented Galea-Calvarial Flap for the Simultaneous Reconstruction of the Frontal Sinus and Forehead: A New Supplementary Method of Reconstructive Cranial Surgery.

We report on a new technical method of using an inverted and segmented galea-calvarial flap for simultaneous reconstruction of the frontal sinus and forehead. A 73-year-old man presented with the production of pus at the forehead, in which the frontal sinus had been involved 6 years postoperatively. The patient underwent surgery with our new simultaneous technique.

Ambient Temperature Change Increases in Stroke Onset: Analyses Based on the Japanese Regional Metrological Measurements.

Background: Relationships between various climate factors and stroke have long been a subject of investigation. The present study investigated in a single medical center the effects of periodic temperature changes on the onset of intracerebral hemorrhage (ICH), subarachnoid hemorrhage (SAH), atherothrombotic infarction (AI), lacunar infarction (LI), cardiogenic embolism (CE), and transient ischemic attack (TIA).

Methods: The subjects were 4,310 patients who had been hospitalized because of hemorrhagic or ischemic stroke from January 2000 through December 2005.

Indwelling Drains Are Not Necessary for Patients Undergoing One-level Anterior Cervical Fixation Surgery.

Background: Anterior cervical discectomy and fusion (ACDF) has become a common procedure for cervical spine surgeries, since it is safe and effective in most patients. However, some patients develop life-threatening problems such as respiratory obstruction arising from rare postoperative hematoma and edema, although intraoperative bleeding caused by the connective tissue splitting procedure or bleeding caused by postoperative insertion of a suction tube has rarely been reported. Investigation of the requirement for indwelling drains in patients who undergo cervical spine surgery is necessary because of the pain, anxiety, and discomfort caused despite the use of high-quality materials.

Descriptive epidemiology of prion disease in Japan: 1999-2012.

Background: Epidemiologic features of prion diseases in Japan, in particular morbidity and mortality, have not been clarified.

Methods: Since 1999, the Research Committee has been conducting surveillance of prion diseases, and the surveillance data were used to assess incident cases of prion diseases. For the observation of fatal cases, vital statistics were used.

Graft-related disease progression in dura mater graft-associated Creutzfeldt-Jakob disease: a cross-sectional study.

Objectives: Details of abnormal prion protein (PrP(Sc)) propagation in the human central nervous system (CNS) are unclear. To assess the spread of PrP(Sc) through the human CNS, we evaluated dura mater graft-associated Creutzfeldt-Jakob disease (dCJD) cases focusing on sites of grafting and dCJD pathological subtypes.

Design: A cross-sectional study.

Insight into the frequent occurrence of dura mater graft-associated Creutzfeldt-Jakob disease in Japan.

Objective: More than 60% of patients worldwide with Creutzfeldt-Jakob disease (CJD) associated with dura mater graft (dCJD) have been diagnosed in Japan. The remarkable frequency of dura mater grafts in Japan may possibly contribute to the elevated incidence of dCJD, but reasons for the disproportionate use of this procedure in Japan remain unclear. We investigated differences between dCJD patients in Japan and those elsewhere to help explain the more frequent use of cadaveric dura mater and the high incidence of dCJD in Japan.

Prediction of thrombolytic therapy after stroke-bypass transportation: the Maria Prehospital Stroke Scale score.

There is no prehospital stratification tool specifically for predicting thrombolytic therapy after transportation. We developed a new prehospital scale named the Maria Prehospital Stroke Scale (MPSS) by modifying the Cincinnati Prehospital Stroke Scale. Our objective is to evaluate its utility in a citywide bypass transportation protocol for intravenous (IV) tissue plasminogen activator (tPA).