Detection of anaerobic carbon monoxide-oxidizing thermophiles in hydrothermal environments.

Carboxydotrophic anaerobic thermophiles have been isolated from various hydrothermal environments and are considered to be important carbon monoxide (CO) scavengers or primary producers. However, the ecological factors that influence the distribution, abundance and CO-oxidizing activities of these bacteria are poorly understood. A previous study detected the carboxydotrophic bacteria Carboxydothermus spp. in a hot spring sample and found that they constituted up to 10% of the total bacterial cells. In this study, we investigated environmental features, potential microbial CO-oxidation activities and the abundance of Carboxydothermus spp. in various hot springs to determine environmental factors that affect CO oxidizers and to see whether Carboxydothermus spp. are common in these environments. We detected potential microbial CO-oxidation activities in samples that showed relatively high values of total organic carbon, total nitrogen, oxidation-reduction potential and soil-water content. The abundance of Carboxydothermus spp. did not correlate with the presence of potential microbial CO-oxidation activities; however, Carboxydothermus spp. were detected in a wide range of environments, suggesting that these bacteria are widely distributed in spite of the relatively low population size. This study implies that thermophilic CO oxidizers occur in a wide range of environments and oxidize CO in somewhat oxidative environments rich in organic matter

Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6–dependent Epilepsy Than Pyridoxal 5′-Phosphate

Background We aimed to demonstrate the biochemical characteristics of vitamin B6–dependent epilepsy, with a particular focus on pyridoxal 5′-phosphate and pyridoxal in the cerebrospinal fluid. Methods Using our laboratory database, we identified patients with vitamin B6–dependent epilepsy and extracted their data on the concentrations of pyridoxal 5′-phosphate, pyridoxal, pipecolic acid, α-aminoadipic semialdehyde, and monoamine neurotransmitters. We compared the biochemical characteristics of these patients with those of other epilepsy patients with low pyridoxal 5′-phosphate concentrations. Results We identified seven patients with pyridoxine-dependent epilepsy caused by an ALDH7A1 gene abnormality, two patients with pyridoxal 5′-phosphate homeostasis protein deficiency, and 28 patients with other epilepsies with low cerebrospinal fluid pyridoxal 5′-phosphate concentrations. Cerebrospinal fluid pyridoxal and pyridoxal 5′-phosphate concentrations were low in patients with vitamin B6–dependent epilepsy but cerebrospinal fluid pyridoxal concentrations were not reduced in most patients with other epilepsies with low cerebrospinal fluid pyridoxal 5′-phosphate concentrations. Increase in 3-O-methyldopa and 5-hydroxytryptophan was demonstrated in some patients with vitamin B6–dependent epilepsy, suggestive of pyridoxal 5′-phosphate deficiency in the brain. Conclusions Low cerebrospinal fluid pyridoxal concentrations may be a better indicator of pyridoxal 5′-phosphate deficiency in the brain in vitamin B6–dependent epilepsy than low cerebrospinal fluid pyridoxal 5′-phosphate concentrations. This finding is especially helpful in individuals with suspected pyridoxal 5′-phosphate homeostasis protein deficiency, which does not have known biomarkers

Direct Evidence that 4ʼ-O-methylpyridoxine Induces Hyperactivity and Convulsions due to Pyridoxal Phosphate Deficiency in the Brain

Ginkgo biloba seed (GBS) poisoning is caused by the toxic substance 4’-O-methylpyridoxine (MPN). Gammaaminobutyric acid (GABA) in the brain is suggested to be involved in the convulsion-inducing effect of MPN. 　　To clarify the direct effects of MPN in the brain on behavior and changes in vitamin B6 and GABA concentration, we intrathecally administered MPN (5-80 nmol) to mice. Some of them developed hyperactivity and seizures. Incidence of behavioral abnormalities were correlated with the MPN dose/body weight. In the analysis of the brain, mice with hyperactivity shown decreased Pyridoxal-5’-phosphate (PLP) and decreased GABA/Glutamae (Glu) ratio. Administered MPN dose/body weight was negatively correlated with PLP (p＜0.001) and GABA/ Glu ratio (p＝0.004) in the brain. MPN concentration in the brain negatively correlated with PLP (p＜0.001) and GABA/Glu ratio (p＜0.001), and a positive correlation between concentration of MPN and phosphorylated MPN (4’-O-methylpyridoxine-5’-phosphate, MPNP) was found (p＝0.01). We report for the first time that MPNP is produced from MPN in vivo and MPN decreases PLP concentrations in the brain, resulting in the decrease of GABA/Glu ratio and this change leads to behavioral changes.Article信州医学雑誌 72(3) : 159-167, (2024)journal articl

Characteristics of an advanced epilepsy treatment gap in a region in Japan

To investigate the quality of epilepsy care in a region in Japan that lacked specialised care, we retrospectively evaluated patients who visited our newly established epilepsy division between April 2018 and March 2021, and had been treated with anti-seizure medications (ASMs) for at least 1 year prior.Of the 231 patients included, 169 had ongoing seizure episodes at first visit (seizure-persist group) and 62 had no seizure episodes for more than a year (seizure-free group). Eighty-three patients in the seizure-persist group had not received specialised epilepsy care, 15 had been treated with unnecessary medications, and seven had experienced side effects from ASMs. Twelve patients in the seizure-free group had been treated with unnecessary ASMs, 10 had been treated with ASMs with teratogenic potential and four had experienced ASM side effects. These patients could be classified as having an advanced epilepsy treatment gap (ETG) because they had not previously received necessary specialised care. The progressive decline in the number of patients with advanced ETG suggests that our new epilepsy division has addressed this issue.This study highlights that a significant number of patients with advanced ETGs exist in Japan and that proper countermeasures are required to address this gap

Frontal Encephalocele Plus Epilepsy: A Case Report and Review of the Literature

An encephalocele is a pathological brain herniation caused by osseous dural defects. Encephaloceles are known to be regions of epileptogenic foci. We describe the case of a 44-year-old woman with refractory epilepsy associated with a frontal skull base encephalocele. Epilepsy surgery for encephalocele resection was performed; however, the epilepsy was refractory. A second epilepsy surgery for frontal lobectomy using intraoperative electroencephalography was required to achieve adequate seizure control. Previous reports have shown that only encephalocele resection can result in good seizure control, and refractory epilepsy due to frontal lobe encephalocele has rarely been reported. To the best of our knowledge, this is the first report of frontal encephalocele plus epilepsy in which good seizure control using only encephalocele resection was difficult to achieve. Herein, we describe the possible mechanisms of encephalocele plus epilepsy and the surgical strategy for refractory epilepsy with encephalocele, including a literature review