Assessing the usefulness of inner ear contrast-enhanced magnetic resonance imaging and pure-tone audiometry in estimating the prognostic value of hearing improvement in patients with definite Meniere's disease.

PURPOSE: Meniere's disease (MD) is characterized by combined cochlear and vestibular symptoms. However, its underlying cause remains unclear, with low-to-mid-tone hearing impairment being predominantly reported. Moreover, predicting hearing improvement after disease onset is not possible. This study aimed to assess whether hearing improvement in patients with definite MD (DMD) could be predicted using inner ear contrast magnetic resonance imaging (IEC-MRI) and pure-tone audiometry (PTA) at disease attack. MATERIALS AND METHODS: Between April 2020 and March 2022, seven outpatients with DMD were enrolled based on the Bárány Society DMD criteria. The patients were divided into two groups: low-tone hearing loss (LTL) group and low-to-mid-tone hearing loss (LMTL) group. Hearing improvement rates as well as the possible inter-relation between endolymphatic hydrops and hearing improvement were evaluated. RESULTS: Endolymphatic hydrops was found in two of four LTL cases. One of three LMTL cases had prominent lymphedema. All patients with LTL but only one patient with LMTL had hearing improvement. Endolymphatic hydrops was not found to be correlated with hearing improvement. CONCLUSIONS: Estimating hearing improvement in patients with DMD using IEC-MRI was not possible. However, PTA showed better hearing prognosis in LTL than in LMTL. Therefore, estimating hearing improvement using PTA was possible at disease attack.

Extranodal Natural Killer/T-cell Lymphoma, Nasal Type Occurring After Actinomyces Infection: A Case Report.

The pathogenesis of extranodal natural killer/T-cell lymphoma (ENKTL) remains largely unknown. Herein, we present a case of ENKTL that may have occurred during the treatment of Actinomyces infection. A 69-year-old woman was admitted to our hospital with nasal bleeding, and a nasopharyngeal mass was observed. The patient was diagnosed with Actinomyces infection on biopsy, and oral antibiotics were administered. The tumor decreased in size; however, swelling of the nasal mucosa and perforation of the nasal septum were observed. A biopsy revealed a recurrence of Actinomyces infection, and oral antibiotics were again administered. The mucosal swelling improved temporarily, but the condition gradually deteriorated. The patient was diagnosed with ENKTL based on a third biopsy. Retrospective evaluation of the biopsies showed that there were no CD56-positive cells in the first specimen; however, the number of CD56-positive cells gradually increased in the second and third specimens. We retrospectively observed the occurrence of ENKTL under chronic inflammatory conditions due to Actinomyces infection in this case. In addition, this case suggests that the possibility of malignancy must be considered when managing such patients with Actinomyces infection.

Management of anaplastic thyroid cancer and proposed treatment guidelines-A 5-year case series study.

BACKGROUND: Anaplastic thyroid cancer is a rare and rapidly progressive cancer with an extremely poor prognosis. Besides surgical control, no clear treatment has been found, mainly due to the small population affected and high mortality rate. AIMS: To propose evidence-based treatment guidelines based on a 5-year retrospective study of patients with anaplastic thyroid cancer treated at our facility. There have been no clearly defined guidelines for treatment plan for undifferentiated thyroid cancer. Our paper presents a retrospective analysis on the treatment of patients with undifferentiated thyroid cancer at our hospital. METHODS AND RESULTS: We retrospectively evaluated the data of patients diagnosed with anaplastic thyroid cancer from April 2017 to March 2022. The total number of patients diagnosed and treated was seven. Two of these patients had operable cancer; five were inoperable and treated with lenvatinib or paclitaxel maintenance therapy. The median time from the first visit to death was 3.84 months, and six of the seven patients died before this study started. Three of them had Stage IVB cancer and died due to deterioration of their general condition, including lung metastasis; the other three had Stage IVC cancer and died of suffocation. The survivor had Stage IVB cancer, was treated by surgery combined with chemical radiotherapy, and survived >240 days. CONCLUSION: Considering the above findings, personalized surgical treatment should be prioritized to prevent suffocation. Especially in Stage IVB cancer, local control can be achieved by surgical and anticancer drug treatment to avoid death from suffocation.

Idiopathic common carotid artery laceration: A case report.

Carotid artery injury is a rare disease often caused by neck trauma or irradiation of head and neck cancer. It is not easy to diagnose quickly without these backgrounds. Herein, we report a case of a 52-year-old man with no history of trauma or irradiation whose carotid artery was found to be injured. It was suggested that patients without any other disease but hypertension could have carotid artery injury. Additionally, it was challenging to detect extravasation from the carotid artery by computed tomography scan and ultrasonography in the emergency department. We should consider the possibility of carotid artery injury when examining patients with sudden neck swelling, even without any history of trauma or irradiation.

Choice of Aspiration Prevention Surgery for Patients With Neuromuscular Disorders: Report of Three Cases.

Dysphagia, one of the major complications of neuromuscular diseases such as Parkinson's disease and amyotrophic lateral sclerosis (ALS), decreases quality of life and may lead to malnutrition or aspiration pneumonia. Although recent reports have suggested that surgical aspiration prevention improves quality of life and enables oral intake, the selection of appropriate aspiration prevention techniques has rarely been discussed. In this report, we present the cases of three patients with neuromuscular diseases who underwent surgical aspiration prevention; we selected the surgical techniques based on analysis of the dysphagia mechanisms, disease progression, and general condition in each case. Case 1 was a 55-year-old man with multiple system atrophy (MSA) and presented with dysphagia associated with insufficient upper esophageal sphincter (UES) relaxation. We performed central-part laryngectomy, which was able to improve UES relaxation. Case 2 was a 79-year-old man with progressive supranuclear palsy who presented with respiratory disorder and dysphagia. Glottic closure under local anesthesia was selected because he also had acute hepatobiliary dysfunction and methicillin-resistant Staphylococcus aureus pneumonia with pleural effusion. Case 3 was a 75-year-old man with ALS and presented with respiratory disorder and mild dysphagia. Subglottic closure with total cricoidectomy was selected because his dysphagia was expected to worsen due to tracheostomy and disease progression. We also summarize the characteristics of the aspiration prevention surgical techniques based on our cases and on literature review. The causes of dysphagia, including insufficient UES opening during swallowing, weak pharyngeal constriction, velopharyngeal insufficiency, and inadequate laryngeal elevation, should be assessed by detailed examination before surgery, and the type of aspiration prevention surgery should be selected based on patient swallowing function and general condition.

Polymorphic regulation of mitochondrial fission and fusion modifies phenotypes of microglia in neuroinflammation.

Microglia are the resident macrophages of the central nervous system and play complex roles in the milieu of diseases including the primary diseases of myelin. Although mitochondria are critical for cellular functions and survival in the nervous system, alterations in and the roles of mitochondrial dynamics and associated signaling in microglia are still poorly understood. In the present study, by combining immunohistochemistry and 3D ultrastructural analyses, we show that mitochondrial fission/fusion in reactive microglia is differentially regulated from that in monocyte-derived macrophages and the ramified microglia of normal white matter in myelin disease models. Mouse cerebral microglia in vitro demonstrated that stimulation of TLR4 with lipopolysaccharide, widely used to examine microglial reactions, caused the activation of the mitochondrial fission protein, dynamin-related protein 1 (Drp1) and enhanced production of reactive oxygen species (ROS). The increase in the ROS level activated 5' adenosine monophosphate-activated protein kinase (AMPK), and facilitated elongation of mitochondria along the microtubule tracks. These results suggest that the polymorphic regulation of mitochondrial fission and fusion in reactive microglia is mediated by distinct signaling under inflammatory conditions, and modulates microglial phenotypes through the production of ROS.

Recent advancement in the challenges to connectomics.

Advancement of microscopic technologies established significant progress in our understanding of the brain. In the recent effort to elucidate the complete wiring map of the brain circuitry termed 'connectome', the different modalities of imaging technology, including those of light and electron microscopy, have started providing essential contribution in multiple organisms. The contribution would be impossible without the recent innovation in both acquisition and analyses of the big connectomic data. The current data demonstrated complicated networks with unidirectional and reciprocal connections of the cerebral circuits at the macroscopic and light microscopic ('mesoscopic') levels, and the unimaginable complexity of synaptic connections between axons and dendrites at the electron microscopic ('microscopic') level. At the same time, the data highlighted the necessity to make substantial advancement in methodology of the connectomic studies, including efficient handling and automated analyses of the acquired dataset. Further understanding about structural and functional connectome seems to be facilitated by combinations of the different imaging modalities. Such multidisciplinary approaches will give us the clues to address whether the complete connectome can elucidate fundamental mechanisms processing the basic and higher functions of human brains.

Three-dimensional volume imaging with electron microscopy toward connectome.

Ultrastructural analyses with electron microscopy have provided indispensable information to understand physiology and pathology of the nervous system. Recent advancement in imaging methodology paved the way for complete reconstruction of the neuronal connection map in the central nervous system, which is termed 'connectome' and would provide key insights to understand the functions of the brain. The critical advancement includes serial ultrastructural observation with scanning electron microscopy (SEM) instead of conventional serial sectioning transmission electron microscopy along with specific tissue preparation methods to increase heavy metal deposition for efficient SEM imaging. The advanced imaging methods using SEM have distinct advantages and disadvantages in multiple aspects, such as resolution and imaging speed, and should be selected depending on the observation conditions, such as target tissue sizes, required spatial resolution and necessity for re-observation. Dealing with the huge dataset remained to be a major obstacle, and automation in segmentation and 3D reconstruction would be critical to understand neuronal circuits in a larger volume of the brain. Future improvement in acquisition and analyses of the morphological data obtained with the advanced SEM imaging is awaited to elucidate the significance of whole connectome as the structural basis of the consciousness, intelligence and memory of a subject.