The first report of human meningitis and pyogenic ventriculitis caused by Streptococcus suis: A case report.

Streptococcus suis, a gram-positive facultative anaerobe commonly found in pigs, is an emerging zoonotic pathogen. Herein, we describe a case of a 45-year-old male Japanese meat wholesaler with S. suis meningitis and pyogenic ventriculitis. S. suis was isolated from his blood and cerebrospinal fluid culture, and sequence type (ST) and serotype were confirmed to be ST1 and serotype 2, respectively, by multilocus sequence typing and the Quellung reaction. Magnetic resonance imaging (MRI) revealed right labyrinthitis and pyogenic ventriculitis. The patient was treated with ceftriaxone and ampicillin for 24 days; the treatment was deemed successful based on negative blood cultures on day 4. However, the patient experienced hearing loss and a vestibular nerve disorder. S. suis is a rare pathogen in Japan but can cause severe infection and sequelae. To the best of our knowledge, this is the first report of a human case of pyogenic ventriculitis caused by S. suis. Our findings suggest that S. suis infection should be considered when hearing impairment is present in a patient with bacterial infection and that MRI can help detect ventriculitis, which can necessitate a prolonged treatment duration.

Prognostic Factors for Japanese Adults With Acute Community-Acquired Bacterial Meningitis: A Retrospective Study.

Background This study aimed to determine if the cerebrospinal fluid (CSF) cell count is useful for predicting the infection severity or prognosis in Japanese adults with community-acquired bacterial meningitis. Methodology This study retrospectively evaluated the prognosis of patients diagnosed with community-acquired bacterial meningitis at our hospital from January 2004 to December 2021 using the modified Rankin scale (mRs) (Showa General Hospital; N = 39). Patients were classified into the following two groups: (i) favorable (mRs: 0-3) and (ii) unfavorable (mRs: 4-6). Eight factors were selected and compared with outcomes, and then two factors were evaluated from those, and a multivariate logistic regression was used to determine the significant variables. Results CSF cell count was observed to be associated with poor prognoses (odds ratio (OR) = 0.86, 95% confidence interval (CI) = 0.99995-0.99999, p = 0.0012). Glasgow coma scale (GCS) score on admission was also observed to be associated with poor prognoses (OR = 0.93, 95% CI = 0.89145-0.97290, p = 0.0029). Conclusions Low CSF cell count and low GCS on admission were observed as risk factors for poor prognoses in patients with bacterial meningitis.

Axonal projections of mechanoreceptive dorsal root ganglion neurons depend on Ret.

Establishment of connectivity between peripheral and central organs is essential for sensory processing by dorsal root ganglion (DRG) neurons. Using Ret as a marker for mechanoreceptive DRG neurons, we show that both central and peripheral projections of mechanoreceptive neurons are severely impaired in the absence of Ret. Death of DRG neurons in Ret-deficient mice can be rescued by eliminating Bax, although their projections remain disrupted. Furthermore, ectopic expression of the Ret ligand neurturin, but not Gdnf, in the spinal cord induces aberrant projection of mechanoreceptive afferents. Our results demonstrate that Ret expression in DRG neurons is crucial for the neurturin-mediated formation of precise axonal projections in the central nervous system.

Soluble C-type lectin-like receptor 2 in stroke (CLECSTRO) study: protocol of a multicentre, prospective cohort of a novel platelet activation marker in acute ischaemic stroke and transient ischaemic attack.

INTRODUCTION: Soluble C-type lectin-like receptor 2 (sCLEC-2) is a new biomarker for platelet activation, which can be easily measured by usual blood collection. We conducted the CLECSTRO, a prospective, observational cohort study, to evaluate the clinical implications of sCLEC-2 in patients with acute ischaemic stroke (AIS) and transient ischaemic attack (TIA). METHODS AND ANALYSIS: The participants are patients with AIS/TIA and control patients required for differentiation from AIS/TIA. The target population is 600, including the patients and controls, who would be recruited from eight stroke centres across Japan. The inclusion criteria are AIS within 24 hours of onset and a modified Rankin Scale (mRS) score of 0-2, TIA within 7 days of onset, and contemporary patients required for differentiation from AIS/TIA. Plasma sCLEC-2 will be measured by high-sensitive chemiluminescent enzyme immunoassay using residual blood samples from routine laboratory examinations at the first visit in all patients and 7 days later or at discharge in patients with AIS/TIA. The outcomes include plasma levels of sCLEC-2 in patients with AIS/TIA and controls, sCLEC-2/D-dimer ratio in non-cardioembolic and cardioembolic AIS/TIA, correlation of sCLEC-2 with recurrence or worsening of stroke, severity of stroke, infarct size, ABCD2 score in TIA and outcome (mRS) at 7 days and 3 months. ETHICS AND DISSEMINATION: This study was approved by the Ethical Committee of the University of Yamanashi as the central ethical committee in agreement with the ethical committees of all collaborative stroke centres. Informed consent will be obtained by an opt-out form from the patients at each stroke centre according to the Ethical Guidelines for Medical and Biological Research Involving Human Subjects by the Japanese Ministry of Health, Labour and Welfare. TRIAL REGISTRATION NUMBERS: NCT05579405, UMIN000048954.

Remote ischemic conditioning for acute ischemic stroke part 2: Study protocol for a randomized controlled trial.

Background: Remote ischemic conditioning (RIC) refers to the application of repeated short periods of ischemia intended to protect remote areas against tissue damage during and after prolonged ischemia. Aim: We aim to evaluate the efficacy of RIC, determined by the modified Rankin Scale (mRS) score at 90 days after stroke onset. Design and methods: This study is an investigator-initiated, multicenter, prospective, randomized, open-label, parallel-group clinical trial. The sample size is 400, comprising 200 patients who will receive RIC and 200 controls. The patients will be divided into three groups according to their National Institutes of Health Stroke Scale score at enrollment: 5-9, mild; 10-14, moderate; 15-20, severe. The RIC protocol will be comprised of four cycles, each consisting of 5 min of blood pressure cuff inflation (at 200 mmHg or 50 mmHg above the systolic blood pressure) followed by 5 min of reperfusion, with the cuff placed on the thigh on the unaffected side. The control group will only undergo blood pressure measurements before and after the intervention period. This trial is registered with the UMIN Clinical Trial Registry (https://www.umin.ac.jp/: UMIN000046225). Study outcome: The primary outcome will be a good functional outcome as determined by the mRS score at 90 days after stroke onset, with a target mRS score of 0-1 in the mild group, 0-2 in the moderate group, and 0-3 in the severe group. Discussion: This trial may help determine whether RIC should be recommended as a routine clinical strategy for patients with ischemic stroke.

[Intractable Neurological Disease].

Subacute intractable neurological diseases sometimes require patients to make various choices in life without accepting the disease. Therefore, doctors need to be diagnose these diseases early. However, in clinical settings, diagnosis is often sought only from the localized history and symptoms and not the overall picture of the disease. In addition, there are many findings that are difficult to interpret, and depending on the combination of findings, the diagnostic criteria or guidelines may be met. As a result, the disease fits only seemingly to the findings. Therefore, I would like to urge physicians to be aware of pitfalls by referring to the cases refferred to our neurology department for us to assess them.

[A case of motor and sensory polyneuropathy and respiratory failure with novel heterozygous mutation of the senataxin gene].

The patient was a 29-year-old male. He took his first steps at two-and-a-half years old, but his physical strength deteriorated and he became non-ambulatory at 12 years old. He had respiratory failure at the age of 20, and finally underwent tracheostomy with invasive positive-pressure ventilation (TPPV). He showed distal dominant muscle weakness and atrophy, including the face. Spinal scoliosis was recognized. He had peripheral predominance of sensory disorders. Nerve conduction studies showed a decrease of compound muscle action potential and a reduction of motor nerve conduction velocity. Sensory nerve action potential was not evoked. In genetic analysis, c.23 C> T (p. T8M) heterozygous mutation was found in the senataxin gene (SETX). Although SETX is a causative gene of familial amyotrophic lateral sclerosis type 4 (ALS4), this case suggests that SETX mutation can also cause motor and sensory polyneuropathy.

Two Japanese LGMDR25 patients with a biallelic recurrent nonsense variant of BVES.

We report two Japanese patients with autosomal recessive limb-girdle muscular dystrophy type R25 (LGMDR25), harboring a novel recurrent homozygous nonsense variant of BVES. Muscle symptoms manifested from childhood to adulthood, initiated in the proximal or distal muscles of the lower limbs, and displayed asymmetric muscle involvement. Similar to the patients in previous reports, these patients also lost ambulation in late middle age. The posterior compartment of the lower limb muscles (biceps femoris, adductor magnus, gastrocnemius, and soleus) was preferentially affected as was the paraspinal muscle. Muscles in the anterior compartment of the thigh were affected in more advanced stages. Both patients had symptomatic atrioventricular block. The POPDC1 protein was undetectable in the muscles of the patients. As observed by transmission electron microscopy, one of the patient samples had fewer caveolae along the sarcolemma than a control sample.

Artemin is a vascular-derived neurotropic factor for developing sympathetic neurons.

Artemin (ARTN) is a member of the GDNF family of ligands and signals through the Ret/GFRalpha3 receptor complex. Characterization of ARTN- and GFRalpha3-deficient mice revealed similar abnormalities in the migration and axonal projection pattern of the entire sympathetic nervous system. This resulted in abnormal innervation of target tissues and consequent cell death due to deficiencies of target-derived neurotrophic support. ARTN is expressed along blood vessels and in cells nearby to sympathetic axonal projections. In the developing vasculature, ARTN is expressed in smooth muscle cells of the vessels, and it acts as a guidance factor that encourages sympathetic fibers to follow blood vessels as they project toward their final target tissues. The chemoattractive properties of ARTN were confirmed by the demonstration that sympathetic neuroblasts migrate and project axons toward ARTN-soaked beads implanted into mouse embryos.

Clavus detected incidentally by positron emission tomography with computed tomography.

This is the first report showing that positron emission tomography with computed tomography (PET/CT) can detect clavus. A 37-year-old woman diagnosed with epithelioid sarcoma on her left inguinal region was investigated by whole-body PET/CT. The imaging showed some 18F-fluorodeoxyglucose uptake at the right lateral foot. There had been a clavus in exactly the same region. Every dermatologist, radiologist and oncologist needs to know about this finding for making an accurate diagnosis.

A Japanese male with a novel ANO5 mutation with minimal muscle weakness and muscle pain till his late fifties.

Limb girdle muscular dystrophy type 2L (LGMD2L) is an adult-onset slowly progressive muscular dystrophy associated with anoctamin 5 (ANO5) gene mutation, mainly reported from Northern and Central Europe. We report the case of a Japanese male patient with a novel homozygous mutation of c.2394dup, p.Arg799Thrfs in ANO5 gene, the second patient in the Asian population. He had had marked elevation of creatine kinase (CK) level for more than 10 years with minimal muscular symptoms consisting of muscle stiffness and occasional cramps, preceding the onset of proximal limb weakness. Calf hypertrophy and selective fatty replacement of the adductor magnus and gastrocnemius muscles were prominent clinical and muscle imaging features. This case suggests that LGMD2L may affect a broader population than has been previously thought, physicians should consider the possibility of ANO5 mutation even in patients showing elevated CK level with no apparent muscle weakness but muscle stiffness or cramps.

A case of meningococcal meningitis that was difficult to treat owing to concurrent ventriculitis.

A 64-year-old male came to our hospital emergency department with fever and consciousness disturbance. Culture tests of blood and spinal fluid samples revealed meningococci (Neisseria meningitidis), and we made a diagnosis of meningococcal meningitis. Brain magnetic resonance imaging (MRI) findings revealed ventriculitis. Ceftriaxone was administered for 17 days, however, relapse was noted after that was discontinued, with neutropenia and renal impairment thought to be adverse reactions to the beta-lactam antibiotic. Hence, treatment was switched to oral administration of moxifloxacin for a total of 12 weeks, including in an outpatient setting. After moxifloxacin was discontinued, no side effects or relapse were seen, and treatment was ended. Although antibacterial agents generally show favorable effects for meningococcal meningitis, we consider that sufficient antimicrobial therapy is difficult in cases complicated with ventriculitis.

Significance of 18 F-FDG PET and immunohistochemical GLUT-1 expression for cardiac myxoma.

Cardiac tumours are relatively rare and are difficult to diagnose merely with imaging techniques. We demonstrated an unusual case of left atrial myxoma, displaying the successful detection by positron emission tomography using 2-deoxy-2-[18 F] fluoro-D-glucose (18 F-FDG PET), correlated closely to more intense and enhanced immunoreactivity with glucose transporter-1 (GLUT-1) in a substantial number of cardiac myxoma cells. Further prospective studies are needed to validate the significance of 18 F-FDG PET findings for cardiac myxoma and the association with immunohistochemical GLUT-1 expression in its tumour cells, after collecting and investigating a larger number of surgical cases examined with both of them. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2991481941253449.