Original scoring system of safety reports for ensuring medical security-trajectory for 18 years at a single center.

Objectives: Safety reports are core components of secure medical systems, but their significance have remained obscure, mostly owing to limited quantitative approaches other than the numbers of reports. In 2005, we developed "management levels" and their equally converted points (p) of scores, which indicated the grades of required correspondences of medical systems. Moreover, products of those scores multiplied by risk levels (equally converted scores), "total scores" were also proposed for weighting harmful events from biphasic aspects; severity of patient damage and the required levels of correspondence as medical systems.

[Herpes simplex encephalitis complicated with cerebral salt wasting syndrome: a case study].

A 78-year-old man was admitted to the hospital with a 4-day history of fever and confusion. Physical examination revealed oral dryness and decreased skin turgor. Blood tests showed hyponatremia (121.

Unusual Presentation of Double-seronegative Myasthenia Gravis with Positive Anti-LRP4 Antibody: Diagnostic Utility of a Videofluoroscopic Swallowing Study.

An 86-year-old woman was admitted to our hospital with cryptogenic progressive dyspnea and dysphagia following a tracheostomy procedure 4 months prior to presentation. She exhibited fluctuating diplopia, bilateral vocal fold paralysis, normal nerve test results, negative findings for serum anti-acetylcholine receptor and anti-muscle-specific kinase antibodies, and positive findings for anti-LDL receptor-related protein 4 (LRP4). A videofluoroscopic swallowing study (VFSS) with edrophonium revealed an improvement in bulbar paralysis.

Delayed Superior Orbital Fissure Syndrome Arising More than One Month after Herpes Zoster Ophthalmicus and Meningitis.

A 79-year-old woman developed herpes zoster ophthalmicus (HZO) with a vesicular rash on the nasal root, which developed soon after intravenous acyclovir therapy. Although varicella zoster virus DNA was undetectable in the cerebrospinal fluid, she presented with ophthalmoplegia without optic nerve dysfunction 32 days after the onset of HZO. We diagnosed the patient with superior orbital fissure syndrome and administered intravenous immunoglobulin and systemic corticosteroids.

Pembrolizumab-induced Myopathy with Anti-striated Muscle Antibodies Successfully Treated by Plasma Exchange.

A 70-year-old woman with advanced endometrial cancer developed right ptosis and muscle weakness in the right quadriceps after pembrolizumab administration. Serum creatine kinase (CK) levels were elevated, and anti-striated muscle antibodies were positive. On magnetic resonance imaging, the right vastus lateral muscle showed an abnormal signal.

[Immune-Mediated Necrotizing Myopathy(IMNM)].

Immune-mediated necrotizing myopathy (IMNM) has recently been classified from polymyositis, and it clinically shows subacute progressive proximal dominant muscle weakness. Laboratory examinations show a great increase in serum creatine kinase and prominent necrotic muscle fibers without any pathological invasion of inflammatory cells. It is thought to be an autoimmune disease because SRP and HMGCR antibodies have been detected in many cases.

[Therapeutic Strategies for Idiopathic Inflammatory Myopathies].

Almost all types of idiopathic inflammatory myopathy (IIM) are treated by immune-modulating therapies. Corticosteroids, such as prednisolone and methylprednisolone, are used as first-line therapy for IIM. When the symptoms cannot be sufficiently improved, immunosuppressive agents, such as azathioprine, methotrexate, or tacrolimus, should be administered approximately two weeks after starting corticosteroid therapy.

[Treatment of Dermatomyositis and Immune-Mediated Necrotizing Myopathy with Poor Muscle Recovery with Steroids and IVIg: Therapeutic Strategies for Refractory Inflammatory Myositis].

The majority of inflammatory myositis cases can be cured by immunomodulatory therapies. We recently observed that the phenotype and response to therapies differed according to myositis-specific autoantibodies; therefore, it is essential to select a suitable therapy after thoroughly evaluating the autoantibody, clinical severity, and complications. In some cases, the symptoms can be controlled by steroid monotherapy, but some cases exhibit steroid resistance and require other therapies.

Successful treatment of ischemic stroke associated with brachiocephalic artery stenosis using alteplase.

Brachiocephalic artery stenosis rarely causes right hemispheric infarction with associated left hemiparesis. To date, there have been no reported cases of stroke associated with brachiocephalic artery stenosis that were successfully treated with recombinant tissue-type plasminogen activator (rt-PA), alteplase. An 80-year-old woman presented with left hemiparesis.

[Immune-Mediated Necrotizing Myopathy: IMNM].

Immune-mediated necrotizing myopathy (IMNM) is a heterogeneous disease that presents with subacute progressive proximal dominant muscle weakness clinically and prominent necrotic muscle fibers without invasion of inflammatory cells pathologically. IMNM is a disease that exclusively affects skeletal muscles, so the serum creatine kinase level usually increases prominently. It is thought to be an autoimmune disease because of the presence of two autoantibodies, anti-SRP and anti-HMGCR.

Distribution of Deep Gray Matter Lesions on Magnetic Resonance Imaging in Lymphomatosis Cerebri.

We herein report the distribution of gray matter lesions on magnetic resonance imaging (MRI) in two patients with lymphomatosis cerebri (LC). In our patients, the fluid-attenuated inversion recovery sequence of brain MRI demonstrated a bilateral and diffuse high signal intensity, not only in the white matter but also in the thalamus, globus pallidus, putamen, and hippocampus. Among the deep gray matter, the caudate head and putamen (striatum) were relatively spared when compared with the globus pallidus, thalamus, and hippocampus.

Treatment consensus for management of polymyositis and dermatomyositis among rheumatologists, neurologists and dermatologists.

Although rheumatologists, neurologists and dermatologists see patients with polymyositis (PM) and dermatomyositis (DM), their management appears to vary depending on the physician's specialty. The aim of the present study was to establish the treatment consensus among specialists of the three fields to standardize the patient care. We formed a research team supported by a grant from the Ministry of Health, Labor and Welfare, Japan.

Treatment consensus for management of polymyositis and dermatomyositis among rheumatologists, neurologists and dermatologists.

Although rheumatologists, neurologists and dermatologists see patients with polymyositis (PM) and dermatomyositis (DM), their management appears to vary depending on the physician's specialty. The aim of the present study was to establish the treatment consensus among specialists of the three fields to standardize the patient care. We formed a research team supported by a grant from the Ministry of Health, Labor and Welfare, Japan.

Retrobulbar optic neuropathy associated with sphenoid sinus mucormycosis.

Because fungi usually spread from the paranasal sinuses to the orbital apex in invasive fungal sinusitis (IFS), IFS often presents as an orbital apex syndrome (OAS) characterized by dysfunction of cranial nerves II, III, IV, V1, and VI. We report a case of sphenoid sinus mucormycosis that presented as isolated retrobulbar optic neuropathy. A 94-year-old woman presented with acute blindness in the right eye.

Acute pancreatitis is a very rare comorbidity of acute ischemic stroke.

Although acute pancreatitis is listed among the exclusion criteria for the administration of recombinant tissue plasminogen activator according to the Japanese Guideline for the Management of Stroke, the co-occurrence of acute pancreatitis and acute ischemic stroke has not been investigated. The present study aimed to assess the incidence rate of acute pancreatitis in patients with acute ischemic stroke. This study consecutively enrolled all patients with ischemic stroke admitted to the Department of Neurology, JA Toride Medical Center between April 2014 and March 2016.

[Dropped head syndrome as first manifestation of primary hyperparathyroid myopathy].

75 years old woman presented with 6-month history of progressive dropped head syndrome. Neurological examination revealed moderate weakness of flexor and extensor of neck and mild weakness of proximal appendicular muscles with normal deep tendon reflexes. The needle electromyography showed short duration and low amplitude motor unit potential.

Bilateral Frontal Lobe Vasogenic Edema Resulting from Hypertrophic Pachymeningitis due to Granulomatosis with Polyangiitis.

A 61-year-old woman presented with a 1-month history of decreased activities of daily living. Magnetic resonance imaging revealed abnormal intensities of the bilateral frontal lobes and enhancement of the thickened dura matter. A biopsy of the dura mater revealed multinucleated giant cells.

Pneumococcal Pyomyositis of the Neck Muscles.

High fever, severe neck pain and neck stiffness can result from meningitis. We report a case of pneumococcal pyomyositis of the neck muscles. A 72-year-old man developed high fever and severe neck pain.

Disappearance of the Hummingbird Sign after Shunt Surgery in a Case of Idiopathic Normal Pressure Hydrocephalus.

A 79-year-old man presented with a slowly progressive gait disturbance. Brain MRI demonstrated ventriculomegaly and the hummingbird sign. A lumbar puncture showed no abnormalities of the cerebrospinal fluid.

Epidemiologic analysis of the clinical features of Japanese patients with polymyositis and dermatomyositis.

Objectives: This study aimed to investigate the clinical characteristics of polymyositis/dermatomyositis (PM/DM) in Japan by analyzing data from the nationwide registration system.

Methods: The data of the registration system in 2009 were analyzed to investigate patient numbers, sex, clinical symptoms, therapies, complications, and prognosis of PM/DM.

Results: The total number of PM/DM cases was approximately 17,000, and the female/male sex ratio was 2.

Significance of Development and Reversion of Collaterals on MRI in Early Neurologic Improvement and Long-Term Functional Outcome after Intravenous Thrombolysis for Ischemic Stroke.

Background And Purpose: Predicting response to rtPA is essential in the era of endovascular therapy for stroke. The purpose of this study was to elucidate prognostic factors of early neurologic improvement and long-term outcome with respect to the development and reversion of leptomeningeal collaterals in recanalization therapy after acute ischemic stroke.

Materials And Methods: We analyzed consecutive patients with proximal MCA occlusion treated with rtPA from 2007 to 2012 at 2 hospital stroke centers.