Taste disorders and alopecia in myasthenia gravis.

BACKGROUND: Non-motor symptoms in myasthenia gravis (MG) are rarely confirmed. Although there are some small cohort studies, a large-systemic survey has not yet been performed. METHODS: We investigated the incidence and clinical characteristics of patients with MG who had taste disorders and alopecia using data of 1710 patients with MG enrolled in the Japan MG Registry 2021. RESULTS: Among them, 104 (6.1%) out of 1692 patients and 138 (8.2%) out of 1688 patients had histories of taste disorders and alopecia, respectively. Among the patients with MG, taste disorders were significantly more common in women, those with severe symptoms, refractory MG, or thymoma-associated MG, and were less common in those with ocular MG. The taste disorders often occurred after the onset of MG and often responded to MG treatments. Alopecia was more common in MG patients with a history of bulbar palsy and thymoma, and it often occurred before the onset of MG and sometimes responded to MG treatments. Multivariate logistic regression analysis revealed taste disturbance was associated with worst quantitative MG score and thymoma-associated MG; and alopecia was associated with thymoma-associated MG. CONCLUSION: Clinicians should be aware of the non-motor symptoms in MG, especially in patients with severe myasthenic symptoms and thymoma-associated MG.

Clinical features and outcomes of patients with muscle-specific kinase antibody-positive myasthenia gravis in Japan.

This study included 51 patients with muscle-specific kinase antibody-positive myasthenia gravis (MuSK-MG) from a Japanese multicenter survey to examine clinical features and outcomes. Median onset age was 37 years and female predominance was observed. All patients developed generalized symptoms and almost all (50/51) patients had bulbar symptoms. About half of the patients met the criteria for refractory MG. The refractory group had a lower age of onset, higher severity scores, and higher maximum daily doses of oral prednisolone compared to the nonrefractory group. The outcomes for MuSK-MG patients in Japan are not favorable, indicating the need for more aggressive treatment.

Streptococcus Agalactiae Meningitis in an Immunocompetent Adult: A Case Report and Literature Review.

Streptococcus agalactiae is a rare cause of meningitis in healthy adults. We herein report a case of culture-negative Streptococcus agalactiae meningitis in a healthy adult which was diagnosed using the FilmArray Meningitis and Encephalitis Panel. A healthy 22-year-old man presented with a fever, headache, and neck stiffness. Despite negative results from blood and cerebrospinal fluid cultures, the diagnosis was confirmed using the FilmArray Meningitis and Encephalitis Panel. The patient was treated with dexamethasone, vancomycin, and ceftriaxone, and thereafter recovered completely. This report highlights the importance of being aware that Streptococcus agalactiae meningitis can occur in healthy individuals, and summarizes these features.

Impact of Early Treatment with Intravenous High-Dose Methylprednisolone for Ocular Myasthenia Gravis.

The efficacy of intravenous high-dose methylprednisolone (IVMP) in ocular myasthenia gravis (MG) has not been fully established. This study aimed to elucidate the effects of early intervention with IVMP for achieving the therapeutic targets (minimal manifestations [MM] or MM or better status with prednisolone ≤ 5 mg/day [MM5mg]) in ocular MG. In this observational study, we included a total of 1710 consecutive patients with MG enrolled in the Japan MG Registry in 2021. Of these, 204 patients with ocular MG who received immunotherapy were analyzed. The clinical course and time to first achieve MM or MM5mg after starting immunotherapy were compared between the early IVMP group (treated with IVMP within 3 months of treatment initiation) and the non-early IVMP group. Despite having greater clinical severity before immunotherapy and lower oral prednisolone doses throughout the course, the early IVMP group (n = 55) showed a higher rate of achievement of MM (P = 0.0040, log-rank test; hazard ratio 1.58, 95% confidence interval [CI] 1.13-2.20, P < 0.0001) and MM5mg (P = 0.0005, log-rank test; hazard ratio 1.78, 95% CI 1.27-2.51, P < 0.0001) compared with the non-early IVMP group (n = 149). In conclusion, an early intervention with IVMP is likely to increase the probability of achieving a better long-term outcome and reducing the total dose of corticosteroids in ocular MG.

Effectiveness of early cycles of fast-acting treatment in generalised myasthenia gravis.

BACKGROUND: Early fast-acting treatment (EFT) is the aggressive use of fast-acting therapies such as plasmapheresis, intravenous immunoglobulin and/or intravenous high-dose methylprednisolone (IVMP) from the early phases of treatment. EFT is reportedly beneficial for early achievement of minimal manifestations (MM) or better status with ≤5 mg/day prednisolone (MM5mg), a practical therapeutic target for myasthenia gravis (MG). OBJECTIVE: The current study aimed to clarify which specific EFT regimen is efficacious and the patient characteristics that confer sensitivity to EFT. METHODS: We recruited a total of 1710 consecutive patients with MG who enrolled in the Japan MG Registry for this large-cohort study. Among them, 1066 with generalised MG who had received immunotherapy were analysed. Prognostic background factors were matched in a 1:1 ratio using propensity score matching analysis between patients treated with EFT (n=350) and those treated without EFT (n=350). The clinical course and time to first achieve MM5mg after starting immunotherapy was analysed in relation to treatment combinations and patient characteristics. RESULTS: Kaplan-Meier analyses showed that EFT had a significant effect on the achievement of MM5mg (p<0.0001, log-rank test; HR 1.82, p<0.0001). Notably, EFT was efficacious for any type of MG, and the inclusion of IVMP resulted in earlier and more frequent achievement of MM5mg (p=0.0352, log-rank test; HR 1.46, p=0.0380). In addition, early administration of calcineurin inhibitors also promoted MM5mg achievement. CONCLUSION: Early cycles of intervention with EFT and early use of calcineurin inhibitors provides long-term benefits in terms of achieving therapeutic targets for generalised MG, regardless of clinical subtype.

Meat consumption and consumer attitudes in Japan: An overview.

Among Asian countries, Japan has the shortest history of eating meat in its food culture. It was only after 2007 that household fresh meat consumption surpassed fresh fish consumption in Japan. Although it was forecast that slowing population and economic growth would limit the demand for meat, household meat consumption in 2020 increased remarkably from that in 2019, perhaps due to COVID-19. Meat consumption has regional variations within Japan attributable to historical background. Japanese consumers prefer domestically produced meat and focus on the visual characteristics of meat, in particular marbling. Extrinsic factors such as information also contribute to Japanese consumers' meat choices more than the quality of the meat. Although Japanese consumers are concerned about the safety of meat, reassurance from appropriate inspection will restore their purchasing intention. Novel factors such as animal welfare and environment-friendly production are also recognized in Japan. Also notable is that wild animal meat consumption is beginning to increase in recent years.

Guillain-Barre Syndrome Caused by Mycoplasma pneumoniae Infection in an Elderly Patient Initially Misdiagnosed As Frailty.

A 73-year-old man with a history of hypertension was referred to our department because of progressive frailty while being treated for acute heart failure. Physical examination revealed loss of tendon reflexes in the lower extremities and muscle weakness in all extremities. After close examination, he was diagnosed with Guillain-Barre syndrome (GBS), which was preceded by a Mycoplasma pneumoniae infection. He was given high-dose intravenous immunoglobulin (IVIg) therapy, and his muscle weakness improved. This case suggests that symptoms of neurological disorders in the elderly may be viewed as frailty. Being elderly is a poor prognostic factor for GBS; therefore, early diagnosis and consultation with a neurologist are necessary. Physical examination is essential to differentiate frailty from neurological disorders, and deep tendon reflexes are instrumental in making a diagnosis of GBS.

Estimation of discrimination threshold for emulsified chicken oil in chicken broth.

Although fat content affects the texture and flavor of meat and meat products, the fat discrimination thresholds are unknown. This study aimed to elucidate the discrimination threshold of fat in meat products using broth from chicken thigh meat as a meat taste model. In Experiment 1, triangle tests were conducted between 1%, 0.2%, 0.04%, and 0.008% (w/v) chicken oil-supplemented broth and broth without chicken oil. The results indicated the discrimination threshold to be between 0.04% and 0.008% (w/v). In Experiment 2, similar triangle tests were conducted using oil supplementation at 0.1%, 0.0333%, 0.0111%, and 0.0037% to refine the discrimination threshold. Logistic regression analysis revealed that the discrimination threshold of emulsified chicken oil in chicken broth was 0.0387% (w/v). We demonstrated that chicken oil made a sensory difference in chicken broth at a low concentration and indicates that the chicken oil strongly affects the sensory characteristics of chicken broth.

Myelin oligodendrocyte glycoprotein-IgG-positive, steroid-responsive combined central and peripheral demyelination with recurrent peripheral neuropathy.

Myelin oligodendrocyte glycoprotein (MOG)-IgG detected by the cell-based assay is associated with demyelinating diseases of the central nervous system, such as optic neuritis, myelitis, and acute disseminated encephalomyelitis, but rarely with peripheral neuropathy. Here, we describe the case of a 32-year-old MOG-IgG+ woman who developed central and peripheral demyelinating lesions. In contrast to previous similar cases, she uniquely presented with repeated subsequent relapses in the peripheral nerve, mimicking chronic inflammatory demyelinating polyneuropathy. Possible pathogenic implications of MOG-IgG in combined central and peripheral nervous system diseases are considered.

[MPO-ANCA-associated hypertrophic pachymeningitis with monoclonal gammopathy of undetermined significance: a case report].

A 66-year-old woman presented with dysesthesia over the right side of her face, hypoglossal nerve dysfunction, dysphagia, and dysgeusia of the right side. A MRI scan of the brain revealed cerebral dural thickening on the right side of the skull base, and histopathological examination revealed granulomatous inflammation of the dura. Based on paranasal sinusitis, bronchodilatation, laboratory tests showing weakly positive MPO-ANCA, intact renal function, and the patient's favorable response to steroids, we diagnosed the patient with limited granulomatosis with polyangiitis (GPA). Reportedly, autoimmune disease might occur in patients with exacerbation of monoclonal gammopathy of undetermined significance, which was observed in this case. This suggests the utility of immunoelectrophoresis.

Reduction in dietary lysine increases muscle free amino acids through changes in protein metabolism in chickens.

Taste is crucial to meat quality, and free Glu is an important taste-active component in meat. Our recent study showed that the short-term feeding of a low-Lys diet increases the concentration of free Glu and other free amino acids in chicken muscle and improves its taste. Here, we investigated the mechanisms by which the feeding of a low-Lys diet increases free Glu in chicken muscle. Two groups (n = 10 per group) of 28-day-old female Ross strain broiler chickens were fed diets with a graded Lys content of 90% or 100% of the recommended Lys requirement (according to National Research Council [1994] guidelines) for 10 D. Free amino acid concentrations and the mRNA abundance of protein metabolism-related genes were measured in breast muscle, and breast muscle metabolome analysis was conducted. Free Glu in muscle was increased by 51.8% in the Lys 90% group compared with the Lys 100% group (P < 0.01). Free threonine, glutamine, glycine, valine, isoleucine, leucine, tyrosine, phenylalanine, histidine, and 3-methyl-histidine concentrations in breast muscle were also increased in the Lys 90% group (P < 0.05). Metabolome analysis also showed that free amino acids were increased in the Lys 90% group. The mRNA abundance of µ-calpain, caspase-3, and 20S proteasome C2 subunit were increased in the Lys 90% group (P < 0.05). Moreover, the free Glu concentration in muscle was correlated with mRNA abundance of µ-calpain (r = 0.74, P < 0.01), caspase 3 (r = 0.69, P < 0.01), 20S proteasome C2 subunit (r = 0.65, P < 0.01), and cathepsin B (r = 0.52, P < 0.05). Our study suggests that the feeding of a low-Lys diet to chickens increased the free Glu content of breast muscle by promoting protein degradation.

[Intravascular large B-cell lymphoma with multiple intracranial hemorrhages diagnosed by brain biopsy].

Central nervous system intravascular lymphoma sometimes includes multiple lesions mimicking cerebral infarction. Herein, we report our experience with a case of intravascular large B-cell lymphoma (IVLBCL) with multiple hemorrhages. A 53-year-old woman was admitted to our hospital with clonic convulsions of the left upper limb. Brain MRI revealed a large number of high-intensity areas on FLAIR and low-intensity areas on susceptibility-weighted imaging (SWI). Chest CT showed bilateral multiple high-density lesions in the lungs. Biopsy of pulmonary lesions revealed no abnormal cells. Levetiracetam was administered to prevent the seizures that were assumed to occur due to the cerebral cortex lesions; however, convulsive seizure recurred with a depressed level of consciousness. On a repeat brain MRI examination, severe, multiple new lesions were shown to have developed bilaterally in the cerebral cortex and white matter, exhibiting spotty low intensities on SWI. Biopsy of a new cerebral lesion was carried out and the lesion was pathologically diagnosed as IVLBCL with hemorrhages. IVLBCL should be noted as one of the differential diagnoses not only in case with multiple infarct lesions, but also in case with multiple hemorrhages.

Epstein-Barr Virus-induced Meningitis-Retention Syndrome.

Meningitis-retention syndrome (MRS) is a rare disorder where aseptic meningitis is accompanied by urinary retention, which can be easily misdiagnosed as urinary tract infection. We present the case of a 55-year-old man with fever and dysuria. At the time of hospitalization, the patient had no symptoms of meningitis, but signs of meningeal irritation appeared later during the course of the disease. Investigation revealed that this was a case of MRS due to Epstein-Barr virus. We have to consider MRS when examining patients with fever and urinary retention, as dysuria may precede meningitis symptoms. LEARNING POINTS: Meningitis-retention syndrome (MRS) is a rare disorder where aseptic meningitis is accompanied by urinary retention, which can be easily misdiagnosed as urinary tract infection.MRS should be considered when examining patients with fever and urinary retention, as dysuria may precede the meningitis symptoms.The presence of Epstein-Barr virus can also complicate MRS.

Peculiar Unpleasant Dysgeusia as the Sole Initial Symptom of Guillain-Barré Syndrome.

Dysgeusia is rare in Guillain-Barré syndrome, particularly as the initial symptom. We herein report the case of a 59-year-old woman who presented with only dysgeusia as the initial symptom of Guillain-Barré syndrome, followed by gradually worsening muscle weakness and bilateral sensory disturbances in the extremities. Her dysgeusia was so unpleasant that she could not eat anything, so she received nasogastric tube feeding without dysphasia. We speculate that the dysgeusia in our patient was mainly caused by inflammation of the chorda tympani nerves. Guillain-Barré syndrome should be considered a possible cause of acute dysgeusia.

Assessment of the dynamics of sensory perception of Wagyu beef strip loin prepared with different cooking methods and fattening periods using the temporal dominance of sensations.

In this study, we assessed the relative sensory perception of Wagyu beef using temporal dominance of sensations (TDS), which is a dynamic sensory method that captures the "dominance of sensation" throughout food consumption. In addition, we checked the integrity of the TDS by comparing the TDS results with a physicochemical analysis. Strip loins were obtained from 24- and 28-month-old Japanese Black cattle ("Wagyu") and were cooked by grilling (yakiniku) or boiling (shabu-shabu). Temporal dominance of sensations was then used to evaluate the four types of samples. "Tender and/or soft," "juicy," "dry," "fat melting," "fat taste," "umami," "sweet taste," and "butter odor" were dominant in at least one of the sample types, with the yakiniku cooking method highlighting texture- and fat-related sensory characteristics, and the shabu-shabu cooking method highlighting flavor-related sensory characteristics. In addition, beef obtained from the 24-month-old Wagyu was significantly more "dry" than that of the 28-month-old cattle, reflecting their different cooking loss. Temporal dominance of sensations successfully demonstrated the dominant sensory perceptions of Wagyu beef prepared with different cooking methods and fattening periods.

Relative contribution of sensory characteristics for different types of pork loin, assessed by temporal dominance of sensations.

BACKGROUND: Sensory perception is a dynamic process occurring sequentially. The dynamic analysis of the perception of sensory characteristics in meat is important to understand complex meat sensory characteristics. Temporal dominance of sensations (TDS) is a dynamic sensory method that captures the 'dominance of sensations' throughout food consumption, and it is possible to calculate the relative contribution of various sensory characteristics by 'dominance rate' over time. The objective of this study was to assess the major sensory characteristics of the dynamic sensory perception of pork loin. Using the TDS, we evaluated pork loins derived from pigs with different genetic and feeding backgrounds. Moreover, we checked the integrity of the TDS by comparing the TDS results with a descriptive sensory analysis and a physico-chemical analysis. RESULTS: Seven types of pork loin were evaluated by TDS, and 'tender and/or soft,' 'tough and/or hard,' 'juicy,' 'dry,' 'fat melting,' 'smoothness,' 'sourness,' 'umami,' and 'beast odors' became dominant in at least one type of pork loin. The dynamics of the perception of pork sensory characteristics was also depicted by TDS. In addition, TDS and descriptive analysis demonstrated similarity in principal component analysis. CONCLUSION: TDS demonstrated the major sensory characteristics on the dynamic sensory perception of pork loin. © 2019 Society of Chemical Industry.

Descriptive Sensory Traits of Cooked Eggs Laid from Hens Fed Rice Grain.

Descriptive sensory characteristics of eggs produced by conventional corn-based feeding and unhulled whole rice grain-feeding were compared in two cooking procedures using a trained panel. Rice-feeding significantly decreased brothy and roasted odor in eggs cooked into half-cooked egg yolks, and decreased the creamy odor, smoothness and moisture of eggs cooked into custard puddings. However, a statistical interaction between rice-feeding and production farm was not observed in every sensory attribute. These findings indicated that replacing corn with unhulled whole rice grain in diets for laying hens alters the sensory attributes of eggs.

Acute Intracranial and Spinal Subdural Hematoma Associated with Vardenafil.

A 28-year-old healthy man was admitted to our hospital because of right-sided headache, vomiting, and lower back pain after the administration of vardenafil. Computed tomography and magnetic resonance imaging of the brain showed a small, right-sided, subdural hematoma. A lumbar magnetic resonance imaging showed a longitudinally extended subdural hematoma. He had no history of trauma. We speculated that vardenafil might have had an association with the bleeding. Several reports have suggested a relationship between phosphodiesterase-5 inhibitors and intracerebral or subarachnoid hemorrhage. Our case suggested that there may also be risks of bleeding into the subdural space. Although headache and nausea are common side effects of vardenafil, hemorrhagic diseases should also be considered when symptoms are severe or prolonged.

In situ characterization of acidic and thermal protein denaturation by infrared microspectroscopy.

Foods meet acid pH during gastric digestion after cooking. An in situ infrared microspectroscopy approach was developed to detect the effects of heat and acid treatments on protein structure separately. Infrared spectra were obtained from meat samples treated with heat and/or acid, and wavenumbers accounting independently for the treatments were extracted by principal component regression. Extreme-acid treatment (pHinitial 2.0) was well predicted (0.5% error) by a simple ratio of as-observed spectral intensities at 1211 and 1396 cm-1, reflecting a perturbation in the vibration of amino acid residues (phenylalanine, tyrosine and aspartic acid) by protein unfolding and protonation. Using the imaging mode of an IR microscope, meat protein acidification was evidenced with high spatial resolution. The heat effect was well discriminated from the acid effect by the ratio of as-observed intensities at 1666 and 1697 cm-1 (0.9% error), indicating content of aggregated ß-sheets relative to α-helix structure.

Relationship between water-holding capacity and intramuscular fat content in Japanese commercial pork loin.

OBJECTIVE: The relationship between water-holding capacity (WHC) and intermuscular fat (IMF) was studied in Japanese commercial pork. METHODS: Longissimus muscles of pigs (n = 62), obtained from two meat packing plants, were analyzed for IMF content, moisture content, drip loss, cooking loss, and pH. Pairwise relationships among these traits were determined using correlation analyses. RESULTS: IMF content was significantly correlated with moisture content (r = -0.88; p<0.01) and pH (r = 0.32; p<0.05), but not with drip loss (r = -0.23; p = 0.07) or cooking loss (r = -0.10; p = 0.42). In contrast, drip loss was significantly (and negatively) correlated with pH (r = -0.57; p<0.01). CONCLUSION: IMF content was not significantly correlated with WHC in pork, and so ultimately, we consider pH to be one of the most important factors influencing WHC in pork meat.