Successful Control of Myasthenic Crisis After the Introduction of Ravulizumab in Myasthenia Gravis: A Case Report.

This case study describes the successful use of ravulizumab in treating a 71-year-old woman with myasthenia gravis experiencing a myasthenic crisis. The patient initially presented with hypernasality and dysphagia; her medical history included untreated, complicated type 1 diabetes. The patient received several treatments approved in Japan for general myasthenia gravis, including immunoadsorption plasmapheresis, tacrolimus, intravenous immunoglobulin, and intravenous methylprednisolone.

Long-Term Bone Density Changes and Fracture Risk in Myasthenia Gravis: Implications for FRAX Tool Application.

Myasthenia gravis (MG) patients often require long-term glucocorticoid therapy, which may affect bone health. This study aimed to assess long-term changes in bone mineral density (BMD), evaluate osteoporotic fracture incidence, and examine the relationship between MG-specific factors and bone health outcomes over a 10-year period. This single-center, prospective cohort study included 28 MG patients.

Real-World Case Series of Efgartigimod for Japanese Generalized Myasthenia Gravis: Well-Tailored Treatment Cycle Intervals Contribute to Sustained Symptom Control.

Introduction: Myasthenia gravis (MG), an immune disorder affecting nerve-muscle transmission, often necessitates tailored therapies to alleviate longitudinal symptom fluctuations. Here, we aimed to examine and compare the treatment cycle intervals and efficacy of efgartigimod in four patients. This case series mainly offers insights into personalized treatment cycle intervals and the efficacy of efgartigimod for patients with MG in our facility in Japan.

Ten-year fracture risk in Japanese patients with myasthenia gravis: A comprehensive assessment using the fracture risk assessment tool.

Background: Myasthenia gravis (MG) is an immune disorder that causes muscle weakness with an increasing prevalence, particularly among the elderly in Japan. Glucocorticoid treatment for MG is problematic for bone health because of reduced bone density and increased fracture risk. The fracture risk assessment tool (FRAX®) can estimate fracture risk, but its applicability in patients with MG remains uncertain.

A Multifaceted Approach to Seizure Management in a Patient With Down Syndrome, Obstructive Sleep Apnea, and Hypothyroidism: A Case Report.

In this case study, a 16-year-old male with Down syndrome (DS) faced persistent nocturnal seizures despite anti-seizure medications and treatment for concurrent hypothyroidism. Obstructive sleep apnea (OSA), a common issue in patients with Down syndrome, was revealed as a trigger of the seizures. The implementation of continuous positive airway pressure (CPAP) therapy along with medication adjustments led to a significant decrease in seizure frequency, highlighting the importance of a comprehensive approach to seizure management in patients with complex medical conditions.

Comprehensive Management of Takayasu Arteritis Using Immunologic and Antithrombotic Interventions With Cerebral Circulation Support: A Case Report.

A 20-year-old woman with bilateral common carotid artery stenosis was diagnosed with Takayasu arteritis (TA). She suffered from a recurrent stroke, and repeated transient ischemic attacks (TIAs) occurred due to middle cerebral artery (MCA) stenosis. The clinical course indicated that TA contributed to MCA stenosis despite the negative results for serological inflammation markers.

Thiamine deficiency unrelated to alcohol consumption presented with urinary retention and Wernicke's encephalopathy: A case report.

Thiamine deficiency can present with rare neurological symptoms such as urinary retention, along with common symptoms like ataxia and decreased limb muscle strength. Early recognition and treatment are crucial to improve symptoms and prevent complications.

Anti-leucine-rich Glioma-inactivated 1 Protein-antibody Positive Encephalitis with Extensive Unilateral Cerebral Cortex and White Matter Lesions.

Encephalitis caused by antibodies targeting the leucine-rich glioma-inactivated 1 protein receptor, which belongs to the anti-voltage-gated potassium channel receptor complex, is characterized by hyponatremia, progressive cognitive impairment, seizures, and psychiatric disorders. The patient initially presented with faciobrachial dystonic seizures and subsequently developed encephalopathy. Brain magnetic resonance imaging revealed atypical unilateral hyperintense signals in the cerebral cortex and white matter.

Siponimod ameliorates experimental autoimmune neuritis.

Background: Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are human autoimmune peripheral neuropathy. Besides humoral immunity, cellular immunity is also believed to contribute to these pathologies, especially CIDP. Sphingosine-1-phosphate receptor 1 (S1PR1) regulates the maturation, migration, and trafficking of lymphocytes.

Myasthenia Gravis Complicated by Eosinophilic Granulomatosis with Polyangiitis: A Case Report.

A 55-year-old woman with a history of allergic sinusitis was being administered cyclosporine for ptosis and diplopia due to myasthenia gravis since age 46 years. She developed painful dysesthesia that began in her feet and later spread to her palms, leading to difficulty in walking. Eosinophils were markedly increased in the peripheral blood.

Refractory and Severe Hepatogenous Diabetes in a Patient with Cirrhosis Improved by Balloon-Occluded Retrograde Transvenous Obliteration of a Large Portosystemic Shunt.

A 54-year-old male with liver cirrhosis (Child-Pugh score 5) presented with severe hepatogenous diabetes (HbA1c 12.6%). Contrast-enhanced CT showed a large portosystemic shunt from the inferior mesenteric vein to the left internal iliac vein.

Unexpected discovery of a diffuse astrocytoma of the conus medullaris in an elderly NF1 patient.

Neurofibromatosis type 1 (NF1) is one of the most common genetic neurocutaneous disorders, and it is well known to be associated with peripheral or central nervous system malignancies. The most common malignant tumors are malignant peripheral nerve sheath tumors (MPNSTs); MPNSTs are the most common cause of death in patients with NF1. Central nervous system malignancies rarely occur.

Percutaneous dye image cardioscopy for detection of endocardial lesions.

Endocardial lesions are caused not only by inflammatory processes but also by myocardial ischemia, resulting in endocardial thrombosis and cerebral embolism. We deviced a method for direct visualization of endocardial damages by a novel dye image cardioscopy with Evans blue and examined its feasibility in patients with heart disease. The dye was injected into the left ventricle before and after endomyocardial biopsy.

Association between preheparin serum lipoprotein lipase mass and acute myocardial infarction in Japanese men.

A sensitive immunoassay system using a specific monoclonal antibody against lipoprotein lipase (LPL) recently demonstrated the presence of an LPL mass in preheparin serum. We reported that a preheparin serum LPL mass (pre-LPL mass) reflected the level of functioning LPL activity in the whole body and could be deeply involved in the progression of coronary atherosclerosis of stable organic angina pectoris. We examined the relation between the pre-LPL mass and acute myocardial infarction (AMI).

[Clinical significance of preheparin serum lipoprotein lipase mass in normocholesterolemic patients with coronary artery disease].

Objectives: Some normocholesterolemic patients have coronary artery disease (CAD) in Japan. This study evaluated the clinical significance of preheparin lipoprotein lipase mass as a risk factor for normocholesterolemic patients with CAD.

Methods: This study included 89 normocholesterolemic male patients with CAD (CAD group, 40 with stable organic angina pectoris, 19 with vasospastic angina pectoris, and 30 with acute myocardial infarction), and 13 normocholesterolemic males with normal coronary arteries (control group) with no stenotic lesion and negative reaction to intracoronary administration of acetylcholine.

Preheparin serum lipoprotein lipase mass is negatively related to coronary atherosclerosis.

In preheparin serum, there exists lipoprotein lipase (LPL) mass with little activity. The clinical significance of this preheparin serum LPL mass (preheparin LPL mass) is unclear. We studied the levels of preheparin LPL mass in patients with coronary atherosclerosis, comparing the results with those in healthy men.

Preventive effects of an antiallergic drug, pemirolast potassium, on restenosis after percutaneous transluminal coronary angioplasty.

Background: We recently confirmed that pemirolast potassium, an antiallergic agent, markedly inhibits migration and proliferation of vascular smooth muscle cells. It has also been reported that pemirolast inhibits intimal hyperplasia in animal experiments.

Methods And Results: To elucidate the preventive effects of pemirolast on restenosis after percutaneous transluminal coronary angioplasty (PTCA), 227 patients were enrolled in this prospective, randomized trial.

[Effects of repetitive myocardial ischemia on collateral circulation, ST deviation and epicardial wall motion].

The effects of repetitive myocardial ischemia on collateral circulation, ST deviation and epicardial wall motion were examined in 12 patients undergoing percutaneous transluminal coronary angioplasty (PTCA) for single left anterior descending artery disease. Rentrop collateral filling grade was assessed using contrast injection to the contralateral artery during the first and final episodes of coronary occlusion. ST deviation was measured by intracoronary electrocardiography.

Partial type of common atrioventricular canal defect associated with mitral stenosis.

We report a 63-year-old woman, with a partial type of common atrioventricular canal and mitral stenosis, who was hospitalized because of dyspnea on exertion. Two-dimensional echocardiogram showed an ostium primum atrial septal defect with two well-formed AV valves located at the same level. However, both anterior and posterior mitral leaflets were markedly thickened with a thickened subvalvular apparatus, and the commisures were fused.

Study of the efficacy of nicorandil in patients with ischaemic heart disease using Exercise-T1-201 myocardial tomography.

The effect of nicorandil on myocardial perfusion in ischaemic heart disease has been studied using exercise-load T1-201 myocardial SPECT (Ex-SPECT). Ex-SPECT was carried out in 12 patients with previous myocardial infarction (OMI) and 9 with angina pectoris (AP) before and after administration of nicorandil 15 mg/day, for three or more weeks; % T1 uptake and the washout rate in infarcted or ischaemic areas were calculated from short axial images using the Bull's eye method. In the OMI group, % T1 uptake and washout rates in the infarction areas improved significantly from 52.

[The usefulness of three dimensional imaging of the myocardium using three heads SPECT camera (PRISM-3000)].

The SPECT unit using a 3 heads camera (PRISM-3000) has an advantage over the conventional one head camera SPECT unit because it can complete full circle scans (360 degrees scans) within a short time, and because the non-circular orbit of the camera heads fitting the individual form of the body has improved the spatial resolution. In addition, the high speed data processing has reduced the time for scan, enabling the three dimensional imaging system clinically applicable. In the present study, this modern technique was used to examined the myocardium in patients with ischemic heart disease so as to evaluate it for usefulness and depict problem to be solved.

[Evaluation of myocardial disorders in patients with dilated cardiomyopathy and left ventricular eccentric hypertrophy--by 201Tl myocardial SPECT].

201Tl myocardial SPECT was performed in cases of dilated cardiomyopathy and valvular heart disease with left ventricular eccentric hypertrophy, and the two groups were compared from the standpoint of the mechanism of onset of myocardial disorders. Significant coefficients of correlation were seen between the Tl score and LVDd (r = 0.792, r = 0.

[Evaluation of efficacy of PTCA in patients with angina pectoris by exercise Tl-201 myocardial SPECT].

Exercise 201Tl single photon emission computed tomography (Ex-SPECT) was performed before and after percutaneous transluminal coronary angioplasty (PTCA) to detect ischemia and determine the response of the ischemic myocardium. In a series of 132 patients with angina pectoris, 43 were followed up by Ex-SPECT and coronary angiography. Before PTCA, ischemia was detected in 85% of the patients, and in all cases a diameter stenosis (%DS) of 90% or more was seen.