Clinical Course of Residual Ventricular Septal Defects After Congenital Heart Disease Repair.

The clinical course of residual ventricular septal defects after congenital heart disease repair is not completely elucidated in the medical literature. This study assessed the incidence, size, and clinical course of residual defects.This single-center retrospective study included 132 patients who survived after ventricular septal defect patch closure (n = 107) and intracardiac repair of double-outlet right ventricle (n = 16) and tetralogy of Fallot (n = 9).

Frontotemporal lobar degeneration in the "Annual of the Pathological Autopsy Cases in Japan".

Background: Good accuracy for the clinical diagnosis of frontotemporal lobar degeneration (FTLD) by specialists in an early onset dementia clinic has been reported.

Objective: To assess the diagnostic accuracy of FTLD in an entire population, without restrictions related to patient age or diagnosing physician.

Methods: Volumes of the "Annual of the Pathological Autopsy Cases in Japan," with reports of 130,105 autopsies throughout Japan from 2007 to 2016, were descriptively analyzed.

Atrioventricular conduction recovery immediately after the re-operation in a repaired CHD patient.

This is a case of a 2.7-year-old girl with trisomy 21 and double outlet right ventricle who underwent epicardial pacemaker system placement for a surgical atrioventricular block and achieved atrioventricular conduction recovery immediately after residual ventricular septal defect closure. Although ventricular pacing ratio was 100% before re-operation, it declined to approximately 25% on the 6 post-operative day and was <1% 3 years after re-operation.

A descriptive study of Parkinson disease and atypical parkinsonisms in the Annuals of the Pathological Autopsy Cases in Japan.

Although many entities have been established within the broad spectrum of Parkinson disease (PD) and atypical parkinsonisms, they are often difficult to differentiate. To clarify the current clinical diagnostic conditions and problems in PD and atypical parkinsonisms, we analyzed volumes of the Annuals of the Pathological Autopsy Cases in Japan. Among 130 105 autopsies conducted from 2007 to 2016 throughout Japan, patients were included in the study if they had been either clinically or pathologically diagnosed with PD, multiple system atrophy (MSA), progressive supranuclear palsy (PSP), or corticobasal degeneration (CBD).

dose associates with increased brain iron and β-amyloid via blood-brain barrier dysfunction.

Objective: To examine the effect of apolipoprotein E () dose on blood-brain barrier (BBB) clearance function, evaluated using an advanced MRI technique and analyse its correlation with brain iron and β-amyloid accumulation in the early stages of the Alzheimer's continuum.

Methods: In this single-centre observational prospective cohort study, 24 non-carriers, 22 heterozygotes and 20 homozygotes in the early stages of the Alzheimer's continuum were scanned with diffusion-prepared arterial spin labelling, which estimates the water exchange rate across the BBB (k). Participants also underwent quantitative susceptibility mapping, [C]Pittsburgh compound B-positron emission tomography and neuropsychological testing.

Clinical investigation in non-liver cirrhosis portosystemic shunt encephalopathy-four case series.

We reported here four cases presenting with disturbance of consciousness over long periods of time and hyperammonemia. Two patients were on maintenance hemodialysis. Contrast-enhanced computed tomography (CT) of abdomen and balloon-occluded retrograde contrast venography revealed existence of a non-cirrhotic portosystemic shunt.

Effects of repetitive transcranial magnetic stimulation on cerebral glucose metabolism.

Objective: To investigate the mechanisms underlying the effect of repetitive transcranial magnetic stimulation (rTMS) on post-stroke hemiplegia, we assessed alterations in cerebral glucose metabolism.

Methods: Five post-stroke hemiplegic patients (three targeted for upper limb impairment and two targeted for lower limb impairment) aged 62.6 ± 6.

Prion diseases reported in the "Annual of the Pathological Autopsy Cases in Japan".

Background: For surveillance projects to be successful, it is important to accurately diagnose all patients, without overlooking any cases. Here, we investigated the present clinical diagnostic accuracy for prion diseases in Japan.

Methods: We analyzed volumes of the "Annual of the Pathological Autopsy Cases in Japan", which reported details on 130,105 autopsies conducted from 2007 to 2016 throughout Japan.

Dopaminergic function in spinocerebellar ataxia type 6 patients with and without parkinsonism.

Background: Although pure cerebellar ataxia is usually emphasized as the characteristic clinical feature of spinocerebellar ataxia type 6 (SCA6), parkinsonism has been repeatedly described in patients with genetically confirmed SCA6.

Methods: We conducted a positron emission tomography study using a combination of [F]fluoro-L-dopa for dopamine synthesis and [C]raclopride for dopamine D2 receptor function on six genetically confirmed SCA6 patients, both with and without parkinsonism. To the best of our knowledge, this is the first dopamine receptor imaging study of patients with SCA6.

[Therapeutic outcome of onabotulinum toxin type A in patients with upper limb spasticity].

Onabotulinum toxin type A treatment for post-stroke upper limb spasticity was investigated to contribute to establishing a standard dosage for Japanese patients. A total of 100 patients participated in the study. The outcome one month (33.

"Possible primary antiphospholipid syndrome" with concurrent diffuse alveolar hemorrhaging and Libman-Sacks endocarditis mimicking catastrophic antiphospholipid syndrome.

Although antiphospholipid antibody syndrome (APS) is an autoimmune condition that is primarily characterized by arterial or venous thrombosis or pregnancy morbidity and the presence of antiphospholipid antibodies (aPL), recent reviews have introduced non-thromboembolic manifestations. We describe the case of a 58-year-old woman with vegetation on the aortic valve, whose initial presentation of APS abruptly developed into diffuse pulmonary hemorrhage. Despite consecutive plasma exchange procedures and the administration of corticosteroids and high-dose intravenous immunoglobulin, multiple brain infarctions developed, and the patient died of pneumonia.

Longitudinal study on MRI intensity changes of Machado-Joseph disease: correlation between MRI findings and neuropathological changes.

To disclose the neuropathological progression course of Machado-Joseph disease (MJD), magnetic resonance imaging (MRI) findings of six genetically confirmed MJD cases (four males and two females, including an autopsied female, all unrelated to one another) were further investigated on neurodegeneration. Brain MRI studies were repeated in all cases at different stages of the disease. Ages at the first MRI study ranged from 47 to 65 years (55.

Effects of pitavastatin on cerebral blood flow.

Background: Hypercholesterolemia has been identified as an important risk factor for stroke. It has been reported that statins might reduce the risk for new or recurrent cardiovascular events and strokes.

Objective: This paper reports on the effects of pitavastatin on cerebral blood flow in 2 elderly patients.

Cognitive conditions of pathologically confirmed dementia with Lewy bodies and Parkinson's disease with dementia.

The relationship between dementia with Lewy bodies (DLB) and Parkinson's disease with dementia (PDD) has been insufficiently described, and it is still problematic. Twenty-nine cases of DLB and 10 cases of PDD were investigated in the present study. DLB cases disclosed a significantly older disease onset and shorter disease duration than PDD cases (p<0.

Autonomic dysfunctions in dementia with Lewy bodies.

Twenty-nine cases of both clinically and neuropathologically diagnosed dementia with Lewy bodies (DLB) were retrospectively examined for autonomic symptoms. Twenty-eight cases showed some kind of autonomic dysfunction. Urinary incontinence (97 %) and constipation (83 %) were the two most common.

Longitudinal MRI study of multiple system atrophy - when do the findings appear, and what is the course?

Several investigators have revealed features of multiple system atrophy (MSA) by magnetic resonance imaging (MRI). For use in clinical diagnosis, we determined the exact time when two main features of pontine and putaminal intensity changes appeared. Furthermore, in order to reveal the course from when the disorder first appeared and how it spread, we also investigated the course of MRI findings and differences between clinical subtypes.