Use of the Superficialized Brachial Artery as Vascular Access for a Patient with Myasthenia Gravis with a Frequent Need for Plasmapheresis: A Case Report.

A 41-year-old woman diagnosed with seronegative myasthenia gravis struggled to maintain remission for a decade, facing crises every 3 months for several years. After repeated apheresis using a non-tunneled non-cuffed central venous dialysis catheter (NTNCC), complications such as catheter-related thrombus in the internal jugular veins and morbid obesity from steroids made the insertion of NTNCC increasingly difficult, leading to consideration of an alternative permanent vascular access (VA) approach. Thus, we created a subcutaneously superficialized brachial artery as the VA, which allowed the patient to undergo safe and uninterrupted apheresis therapy.

The Utility of Automated ASPECTS in Acute Ischemic Stroke for Intravenous Recombinant Tissue Plasminogen Activator (IV-rtPA) Therapy.

PURPOSE: This study aimed to investigate the accuracy and clinical significance of an artificial intelligence (AI)-based automated Alberta Stroke Program Early Computed Tomography (ASPECT) scoring software of head CT for the indication of intravenous recombinant tissue plasminogen activator (rt-PA) therapy. METHODS: This study included two populations of acute ischemic stroke: one comprised patients who had undergone head CT within 48 h of presentation (Population #1, n = 448), while the other included patients within 4.5 h from onset (Population #2, n = 132). The primary endpoint was the concordance rate of ASPECTS of the neurologists and AI software against the benchmark score. The secondary endpoints were to validate the accuracy of the neurologist and AI software in assessing the ability to rule out extensive infarction (ASPECTS of 0-5) in population #2. RESULTS: The reading accuracy of AI software was comparable to that of the board-certified vascular neurologists. The detection rate of cardiogenic cerebral embolism was better than that of atherothrombotic cerebral infarction. By excluding extensive infarction, AI-software showed a higher specificity and equivalent sensitivity compared to those of experts. CONCLUSIONS: The AI software for ASPECTS showed convincing agreement with expert evaluation and would be supportive in determining the indications of intravenous rt-PA therapy.

Antenatal Glucocorticoid Administration Promotes Cardiac Structure and Energy Metabolism Maturation in Preterm Fetuses.

Although the rate of preterm birth has increased in recent decades, a number of preterm infants have escaped death due to improvements in perinatal and neonatal care. Antenatal glucocorticoid (GC) therapy has significantly contributed to progression in lung maturation; however, its potential effects on other organs remain controversial. Furthermore, the effects of antenatal GC therapy on the fetal heart show both pros and cons. Translational research in animal models indicates that constant fetal exposure to antenatal GC administration is sufficient for lung maturation. We have established a premature fetal rat model to investigate immature cardiopulmonary functions in the lungs and heart, including the effects of antenatal GC administration. In this review, we explain the mechanisms of antenatal GC actions on the heart in the fetus compared to those in the neonate. Antenatal GCs may contribute to premature heart maturation by accelerating cardiomyocyte proliferation, angiogenesis, energy production, and sarcoplasmic reticulum function. Additionally, this review specifically focuses on fetal heart growth with antenatal GC administration in experimental animal models. Moreover, knowledge regarding antenatal GC administration in experimental animal models can be coupled with that from developmental biology, with the potential for the generation of functional cells and tissues that could be used for regenerative medical purposes in the future.

A case of autoimmune encephalitis with involuntary movements as the first symptom and suspected association with mumps virus infection.

This case involved a 72-year-old woman. From the day after mitral annuloplasty, a fever over 37°C and ballismus-like involuntary movements of the right upper and lower limbs appeared. A few month later, involuntary movements spread throughout the body, and she developed impairment of consciousness and difficulty speaking and eating. Levels of protein in cerebrospinal fluid were high. Positive results were seen for serum mumps immunoglobulin G and M antibody. Because steroid pulse therapy proved effective, we suspected autoimmune encephalitis associated with mumps virus infection.

Body Pitch Together With Translational Body Motion Biases the Subjective Haptic Vertical.

Accurate perception of verticality is critical for postural maintenance and successful physical interaction with the world. Although previous research has examined the independent influences of body orientation and self-motion under well-controlled laboratory conditions, these factors are constantly changing and interacting in the real world. In this study, we examine the subjective haptic vertical in a real-world scenario. Here, we report a bias of verticality perception in a field experiment on the Hong Kong Peak Tram as participants traveled on a slope ranging from 6° to 26°. Mean subjective haptic vertical (SHV) increased with slope by as much as 15°, regardless of whether the eyes were open (Experiment 1) or closed (Experiment 2). Shifting the body pitch by a fixed degree in an effort to compensate for the mountain slope failed to reduce the verticality bias (Experiment 3). These manipulations separately rule out visual and vestibular inputs about absolute body pitch as contributors to our observed bias. Observations collected on a tram traveling on level ground (Experiment 4A) or in a static dental chair with a range of inclinations similar to those encountered on the mountain tram (Experiment 4B) showed no significant deviation of the subjective vertical from gravity. We conclude that the SHV error is due to a combination of large, dynamic body pitch and translational motion. These observations made in a real-world scenario represent an incentive to neuroscientists and aviation experts alike for studying perceived verticality under field conditions and raising awareness of dangerous misperceptions of verticality when body pitch and translational self-motion come together.

[A case of meningoencephalitis that developed during nivolumab use].

A 72-year-old woman developed a fever and consciousness disturbance after completing 8 courses of nivolumab for lung adenocarcinoma. A cerebrospinal fluid test showed an increased cell count, but bacterial culture, herpes simplex virus-polymerase chain reaction, acid-fast staining, and cytology were negative; serum paraneoplastic syndrome-related antibody was also negative. Serum and cerebrospinal fluid specimens were positive for anti-glutamate receptor (GluR) antibody, and fluid-attenuated inversion recovery images on head magnetic resonance imaging showed a high signal intensity at the right parietal lobe. The condition was determined to be immune-mediated encephalitis, and pulse steroid therapy was performed. The symptoms promptly improved after treatment. The patient in the present case was anti-GluR antibody-positive but was determined to have nivolumab-related encephalitis based on the clinical course. The use of immune checkpoint inhibitors has become widespread in recent years, although it can occasionally lead to encephalitis. We herein report our experience with immune checkpoint inhibitor-related encephalitis, which is seldom reported in Japan.

[A case of nasal discharge resulting in recurrent bacterial meningitis].

A 47-year-old man who was previously hospitalized three times due to bacterial meningitis experienced a headache and posterior neck pain in May. He was admitted to our hospital because of a fever 3 h later. He was fully conscious and febrile, with a headache and signs of meningeal irritation. A cerebrospinal fluid examination showed an increased number of cells with polynuclear cell predominance and decreased glucose levels, leading to the diagnosis of bacterial meningitis. Steroid and antibiotic treatment was initiated, at which time, large amounts of nasal discharge were observed. Cisternal scintigraphy was performed, and cerebrospinal fluid was detected in the nasal discharge. The cause was idiopathic, and endoscopic repair was performed. The nasal fluid leakage was suggested to be the cause of the recurrent bacterial meningitis in this case.

IgG4-related hypophysitis diagnosed by retroperitoneal mass biopsy in a patient presenting with abducens nerve palsy: A case report (CARE-compliant article).

RATIONALE: Immunoglobulin G4 (IgG4)-related hypophysitis is a rare disorder which often requires invasive pituitary gland biopsy to confirm its diagnosis. We present a case whereby peripheral organ lesion biopsy and imaging findings were sufficient for the diagnosis. PATIENT CONCERNS: A 77-year-old man with diplopia was referred to our department by an opthomologist who had diagnosed the patient with right abducens nerve palsy. DIAGNOSES: Head magnetic resonance imaging revealed enlargement of the pituitary gland and pituitary stalk, while hormonal analysis revealed panhypopituitarism, thereby indicating a diagnosis of hypophysitis. Abdominal computed tomography imaging revealed a solid mass that encompassed the left kidney ureter. Although the patient did not have an increase in serum IgG4, a biopsy of the periureteral mass revealed infiltrating plasma cells that were positive when stained for IgG4. INTERVENTIONS: The patient was given corticosteroid pulse therapy (methylprednisolone: 1 g × 3 days), followed by oral corticosteroids (prednisolone, 0.5 mg/kg/d). OUTCOMES: The right abducens nerve palsy improved and the pituitary lesion shrank after the initiation of corticosteroid treatment. CONCLUSION: Based on the diagnosis of IgG4-related disease in the retroperitoneal organ and response to corticosteroid treatment, this patient was diagnosed with IgG4-related hypophysitis. This hypophysitis caused enlargement of the pituitary gland with resulting nerve compression, causing abducens nerve palsy. When IgG4-related hypophysitis is suspected, a thorough examination of other organ lesions and biopsy should be considered.

Severe Multiple Sclerosis Manifesting upon GnRH Agonist Therapy for Uterine Fibroids.

This case concerned a 39-year-old woman diagnosed with uterine fibroids. Upon initiation of gonadotropin-releasing hormone (GnRH) agonist therapy, she experienced various neurological deficits but did not seek medical attention because of gradual spontaneous symptom improvement. Upon completing four courses of GnRH agonist therapy, she began experiencing severe neurological symptoms and was diagnosed with multiple sclerosis (MS). Although her symptoms improved with steroid pulse therapy, serious sequelae remained. GnRH agonist therapy can exacerbate the disease activity of MS; therefore, awareness of the potential emergence of neurological symptoms during GnRH agonist therapy is important.

[Longitudinally extensive transverse myelitis involving fifteen vertebral bodies positive for anti-myelin oligodendrocyte glycoprotein (MOG) antibody: a case report].

A 16-year-old male with no previous medical history developed sudden fever and urinary dysfunction. He was admitted to our hospital due to bilateral leg weakness and sensory disturbance on the third day of weakness onset. A sagittal T2-weighted image displayed a longitudinal extensive lesion of transverse myelitis in the spinal column from the upper cervical (C2) to the thoracic region (Th9). The patient was diagnosed with autoimmune myelitis and treated with four courses of intravenous methylprednisolone (1 g/day for three consecutive days per week). This improved his signs, and his serum sample tested negative for anti-aquaporin-4 (AQP-4) antibody but positive for anti-myelin oligodendrocyte glycoprotein (MOG) antibody in cell-based assays. We report this case of longitudinally extensive transverse myelitis involving fifteen vertebral bodies positive for anti-MOG antibody.

Exposure of immature rat heart to antenatal glucocorticoid results in cardiac proliferation.

BACKGROUND: ATP synthesis and cardiac contraction-related protein production are accelerated in the immature fetal heart by antenatal glucocorticoids (GC). This study investigated the structural maturity of the myocardium and underlying signal pathway associated with cardiac growth in fetal rats that received antenatal GC. METHODS AND RESULTS: Dexamethasone (DEX) was given to pregnant rats for 2 days from day 17 or day 19 of gestation, and the hearts of 19 and 21 day fetuses and 1-day-old neonates were analyzed. Although irregular myofibril orientation was observed morphologically in 19 day fetal hearts, the myofibril components were organized in fetuses after DEX. The cross-sectional area of the myocardium and Ki-67-positive cells were significantly increased in fetal DEX groups, suggesting that cardiac enlargement resulted from myocyte proliferation. Glycogen synthase kinase-3ß (GSK-3ß) protein was significantly decreased in fetal DEX groups. ß-Catenin and vascular endothelial growth factor protein were also significantly increased. Furthermore, increased cardiomyocyte proliferation appeared to be mediated by GC receptors after culture with DEX in vitro. CONCLUSIONS: Antenatal DEX induces structural maturity accompanying cardiomyocyte proliferation in the premature fetal rat heart, and GSK-3ß and ß-catenin are thought to contribute to cardiac growth.

Erratum: Tactile Flow Overrides Other Cues To Self Motion.

A correction to this article has been published and is linked from the HTML version of this paper. The error has been fixed in the paper.

Reversible Cerebral Vasoconstriction Syndrome Presenting with Transient Global Amnesia.

A 65-year-old man who had been diagnosed with transient global amnesia (TGA) 15 years previously was admitted to hospital with complaints of amnesia and headache. His symptoms improved on day-2. The initial brain MRI and electroencephalography findings were normal. He was diagnosed with a recurrence of TGA and discharged. However, he returned with right leg weakness and complained of a thunderclap headache. MRI demonstrated subarachnoid hemorrhage and multifocal segmental narrowing of the left posterior cerebral artery (PCA) and large intracranial arteries, and he was diagnosed with reversible cerebral vasoconstriction syndrome (RCVS). He was discharged on day-30 without any neurological deficits. This case suggested that TGA should be interpreted as one of the symptoms of RCVS or a prodromal symptom of RCVS.

Tactile Flow Overrides Other Cues To Self Motion.

Vestibular-somatosensory interactions are pervasive in the brain but it remains unclear why. Here we explore the contribution of tactile flow to processing self-motion. We assessed two aspects of self-motion: timing and speed. Participants sat on an oscillating swing and either kept their hands on their laps or rested them lightly on an earth-stationary surface. They viewed a grating oscillating at the same frequency as their motion and judged its phase or, in a separate experiment, its speed relative to their perceived motion. Participants required the phase to precede body movement (with or without tactile flow) or tactile flow by ~5° (44 ms) to appear earth-stationary. Speed judgments were 4-10% faster when motion was from tactile flow, either alone or with body motion, compared to body motion alone (where speed judgments were accurate). By comparing response variances we conclude that phase and speed judgments do not reflect optimal integration of tactile flow with other cues to body motion: instead tactile flow dominates perceived self-motion - acting as an emergency override. This may explain why even minimal tactile cues are so helpful in promoting stability and suggests that providing artificial tactile cues might be a powerful aid to perceiving self-motion.

Growing potential of small aortic valve with aortic coarctation or interrupted aortic arch after bilateral pulmonary artery banding.

OBJECTIVES: Bilateral pulmonary artery banding (bPAB) is utilized for some patients with a ventricular septal defect (VSD) and aortic coarctation (CoA) or interrupted aortic arch (IAA). We evaluated aortic valve (AoV) diameter and patient outcomes following bPAB. METHODS: Between August 2010 and September 2015, 10 consecutive patients with VSD and patent ductus arteriosus-dependent CoA or IAA underwent bPAB because of an AoV diameter of approximately <50% of the normal value (n = 6), severe subaortic stenosis and poor patient condition (n = 1, respectively), or low birthweight (n = 2). RESULTS: Second-stage operations were conventional total repair in five and Damus-Kaye-Stansel anastomosis, aortic arch reconstruction and right ventricle-pulmonary artery shunt (modified Norwood) type repair in five. After modified Norwood-type repair, four patients were Yasui-type repair candidates and one was a Fontan candidate. For all patients, the mean AoV diameter increased from 3.7 ± 0.7 mm before bPAB to 4.6 ± 0.8 mm before the second-stage operation. In five patients with CoA or IAA type A, the AoV diameter significantly increased from 3.5 ± 0.3 mm to 4.5 ± 0.5 mm during the term between bPAB and the second-stage operation, with an AoV Z-score increase from -5.82 ± 0.92 to -4.28 ± 0.86. IAA type B showed a slight increase in the AoV diameter. CONCLUSIONS: Initial palliation with bPAB enables AoV diameter growth in some patients, improving the likelihood of conventional total repair adaptation rate, particularly for CoA or IAA type A.