Idiopathic hypersomnia with a video recording of a spontaneous sleep attack: A case report.

RATIONALE: Although patients with central disorders of hypersomnolence (CDH) exhibit characteristic symptoms of hypersomnia frequently, it takes 5 to 15 years from the onset for its diagnosis due to the lack of symptom recognition. Here, we present a case of idiopathic hypersomnia (IH), a CDH, wherein early diagnosis was aided by a video footage of a spontaneous sleep attack. PATIENT CONCERNS: A 21-year-old man lost consciousness while driving and experienced an accident. He had complained of excessive daytime sleepiness (EDS) over half a year. During his hospitalization for close monitoring of the loss of consciousness, an in-room surveillance camera captured a 14-minutes long spontaneous sleep attack, during which he experienced general muscle weakness and loss of consciousness without warnings or convulsions leading to a fall from the bed. There were no abnormalities in vital signs. DIAGNOSES: There was no significant cataplexy and less than 2 sleep-onset rapid eye movements (SOREM) in 2 sleep latency tests, with a mean sleep latency of 2.1 and 4.6 minutes. Other sleep deprivation syndromes were excluded from differential diagnosis and finally, a diagnosis of IH was confirmed according to the criteria of the Third Edition of the International Classification of Sleep Disorders. During the course of the disease, attention-deficit/hyperactive disorder (ADHD) and a gaming disorder also diagnosed. INTERVENTIONS: Pharmacological treatment with modafinil was administered for IH and methylphenidate for ADHD. Cognitive behavioral therapy was performed for the gaming disorder. OUTCOMES: The EDS improved, and sleep attacks were no longer observed. The disruption of daily life caused by the gaming disorder was also reduced. LESSONS: Video recordings of sleep attacks are beneficial for identifying the cause of loss of consciousness. Home video recordings may be helpful in the early diagnosis of IH.

Efficacy of denosumab in the treatment of hypercalcemic renal dysfunction in sarcoidosis: a case report.

A 70-year-old female patient was admitted for close examination and treatment of hypercalcemia (corrected serum calcium levels: 3.04 mmol/L) and renal dysfunction (serum creatinine levels: 254.59 µmol/L). The patient had a history of sarcoidosis, diagnosed based on epithelioid cell granulomas in subcutaneous nodule biopsies, uveitis, and bilateral hilar lymphadenopathy, which had spontaneously remitted 10 years before admission. Because the patient was diagnosed with hypercalcemia associated with recurrent sarcoidosis, prednisone (20 mg/day) was initiated, and its dose was tapered following the decrease in serum calcium and creatinine levels. However, the levels of these parameters increased again when the prednisone dose was reduced to ≤ 4 mg/day. We were concerned about glucocorticoid-induced osteoporosis in the patient but hesitated to use first-line bisphosphonates because of renal dysfunction. Therefore, denosumab was initiated to reduce the risk of hypercalcemia, renal dysfunction, and glucocorticoid-induced osteoporosis. Serum creatinine and corrected serum calcium levels subsequently decreased. The prednisone dose could be reduced following repeated denosumab administration.Thus, denosumab can be a multifaceted, beneficial option for sarcoidosis-induced hypercalcemia, as it alleviates renal dysfunction indirectly by normalizing serum calcium levels, facilitates reduction of the glucocorticoid dose, and ameliorates glucocorticoid-induced osteoporosis.

Proximal Renal Tubular Acidosis Complicated by Severe Hypocalcemia Caused by Malnutrition and Inappropriate Long-term Use of Zoledronate: A Case Report and Review of the Literature.

An 80-year-old man presented with electrolyte abnormalities, particularly hypocalcemia (3.6 mg/dL). He was diagnosed with bone and lymph node metastases from prostate cancer seven years earlier and continuously received goserelin, bicalutamide, and zoledronate. He later developed gradually worsening hypocalcemia, hypokalemia, hypophosphatemia, hypouricemia, renal dysfunction, and weight loss. Urinary potassium and phosphate loss, renal glucosuria, metabolic acidosis, and a low urine pH (5.0) were observed. Given the acquired onset and clinical course, we diagnosed the patient with zoledronate-induced proximal renal tubular acidosis. In the present case, severe hypocalcemia may have been caused by malnutrition and inappropriate long-term use of zoledronate.

Increasing averaging beats improves the test accuracy on Holter-based late potentials in patients with myocardial infarction.

BACKGROUND: The prevalence of Holter-based late potentials (H-LPs) in cases of fatal cardiac events has increased. Although the noise level of H-LP is higher than that of conventional real-time late potential (LP) recording, a procedure to reduce the noise severity in H-LP by increasing the averaging beats has not been investigated. METHODS: We enrolled 104 patients with post-myocardial infarction (MI) and 86 control participants. Among the patients, 30 reported sustained ventricular tachycardia (VT), and the remaining 74 had unrecorded VT. H-LPs were measured twice in all groups to evaluate the efficacy of increasing the averaging beats for H-LPs. Thereafter, the average of LP was calculated at 250 (default setting), 300, 400, 500, 600, 700, and 800 beats. RESULTS: Across all three groups (MI-VT group, MI non-VT group, and control group), the noise levels significantly decreased in consonance with the increase in averaging beats. In the MI-VT group, the H-LP positive rate considerably increased with the increase in the averaging beats from 250 to 800 both at night and daytime. In the MI-VT group, the LP parameters significantly deteriorated, which led to a positive judgment corresponding to the increment of the averaged night and day beats. The H-LP positive rates were unchanged in the MI non-VT and control groups, while the LP parameters remained consistent, despite the increased averaging beats in the MI non-VT and control groups. CONCLUSION: Increasing the calculated averaging beats in H-LPs can improve the sensitivity of predicting fatal cardiac events in patients with MI.

Diurnal Variation in and Optimal Time to Measure Holter-Based Late Potentials to Predict Lethal Arrhythmia after Myocardial Infarction.

Background and Objectives: Holter-based late potentials (LPs) are useful for predicting lethal arrhythmias in organic cardiac diseases. Although Holter-based LPs exhibit diurnal variation, no studies have evaluated the optimal timing of LP measurement over 24 h for predicting lethal arrhythmia that leads to sudden cardiac death. Thus, this study aimed to validate the most effective timing for Holter-based LP testing and to explore factors influencing the diurnal variability in LP parameters. Materials and Methods: We retrospectively analyzed 126 patients with post-myocardial infarction (MI) status and 60 control participants who underwent high-resolution Holter electrocardiography. Among the 126 post-MI patients, 23 developed sustained ventricular tachycardia (VT) (the MI-VT group), while 103 did not (the MI-non-VT group) during the observation period. Holter-based LPs were measured at 0:00, 4:00, 8:00, 12:00, 16:00, and 20:00, and heart rate variability analysis was simultaneously performed to investigate factors influencing the diurnal variability in LP parameters. Results: Holter-based LP parameters showed diurnal variation with significant deterioration at night and improvement during the day. Assessment at the time with the longest duration of low-amplitude signals < 40 µV in the filtered QRS complex terminus (LAS40) gave the highest receiver operating characteristics curve (area under the curve, 0.659) and the highest odds ratio (3.75; 95% confidence interval, 1.45-9.71; p = 0.006) for predicting VT. In the multiple regression analysis, heart rate and noise were significant factors affecting the LP parameters in the MI-VT and control groups. In the non-VT group, the LP parameters were significantly influenced by noise and parasympathetic heart rate variability parameters, such as logpNN50. Conclusions: For Holter-based LP measurements, the test accuracy was higher when the LP was measured at the time of the highest or worst value of LAS40. Changes in autonomic nervous system activity, including heart rate, were factors influencing diurnal variability. Increased parasympathetic activity or bradycardia may exacerbate Holter-based LP parameters.

Age-related reference intervals for ambulatory electrocardiographic parameters in healthy individuals.

Background: The advent of novel monitoring technologies has dramatically increased the use of ambulatory electrocardiography (AECG) devices. However, few studies have conducted detailed large-scale investigations on the incidence of arrhythmias over 24 h, especially ectopy, in healthy individuals over a wide age range. Objectives: This study aimed to investigate the incidence of arrhythmias detected using AECG and associated factors, in healthy individuals, over a wide age range. Methods: In this cross-sectional study, we performed AECG on 365 healthy volunteers (median [interquartile range]: 48 [36, 67], 20-89 years, 165 men) under free-running conditions for 24 h. Ultrasonic echocardiography and heart rate variability analysis were performed to explore the factors associated with the incidence of arrhythmias. Results: The 97.5th percentile of single ventricular ectopy (VE) was 149/day, 254/day, and 1,682/day in the 20-39-, 40-59- and 60-89-year age groups, respectively; that of single supraventricular ectopy (SVE) was 131/day, 232/day, and 1,063/day, respectively. Multivariate analysis revealed that aging was the only independent significant factor influencing the frequency of VE (ß = 0.207, P = 0.001). Age (ß = 0.642, P < 0.001), body mass index (BMI) (ß = -0.112, P = 0.009), and the root mean square of successive differences in RR intervals (ß = 0.097, P = 0.035) were factors significantly associated with SVE frequency. Conclusions: Age-specific reference intervals of VE and SVE in a large population of healthy participants over a wide age range were generated. VE and SVE increased with age; SVE was influenced by BMI and the aging-induced decrease in parasympathetic tone activity.

Iodine-containing ointment-induced hypothyroidism in a patient with anorexia nervosa and sacral decubitus ulcer: a case report with literature review.

We present a case of a 58-year-old woman with anorexia nervosa and a sacral decubitus ulcer who developed hypothyroidism because of an iodine-containing ointment. Considering the absence of autoimmune thyroid diseases, the development of hypothyroidism after the use of an iodine-containing ointment, and the recovery of thyroid function after the discontinuation of the ointment, we presumed that her hypothyroidism was induced by the iodine-containing ointment. Although the hypothyroidism improved after discontinuing the iodine-containing ointment, she developed aspiration pneumonia and required long-term hospitalization. Many patients with autoimmune thyroid diseases develop hypothyroidism after excessive iodine intake. However, anorexia nervosa may have exacerbated the iodine-induced hypothyroidism in our patient. To the best of our knowledge, no previous study has reported a case of hypothyroidism caused by iodine-containing ointment in a patient with anorexia nervosa. Hence, physicians must pay careful attention to a patient's background factors to ensure the early diagnosis of hypothyroidism due to iodine-containing ointments.

Rapid progression of marginal zone B-cell lymphoma after COVID-19 vaccination (BNT162b2): A case report.

B-cell lymphomas are neoplastic diseases occasionally associated with chronic inflammation. mRNA vaccines for coronavirus disease 2019 (COVID-19) induce inflammatory responses, which often lead to fever and lymphadenopathies indistinguishable from lymphomas. Although both lymphadenopathies and lymphomas can be influential, the correlation between them is unclear. Herein, we present the first case of marginal zone B-cell lymphoma following mRNA COVID-19 vaccination. An 80-year-old Japanese woman presented with a right temporal mass that appeared the morning after she was administered her first mRNA COVID-19 vaccination (BNT162b2). The mass gradually decreased in size but persisted over 6 weeks after her first vaccination (3 weeks after her second vaccination). At her first visit to our hospital, ultrasound revealed the size of the mass to be 28.5 × 5.7 mm, and computed tomography revealed multiple lymphadenopathies in the right parotid, submandibular, jugular, and supraclavicular regions. Initially, we suspected head-and-neck benign lymphadenopathy as a side effect of vaccination. Nine weeks later, the number of swollen submandibular and parotid glands increased, and the lymph nodes further enlarged. Finally, the right temporal mass was diagnosed as marginal zone B-cell lymphoma based on immunohistochemical and flow cytometry findings of biopsy specimens. Our findings suggest that although 4-6 weeks of observation for lymph node inflammation after the second vaccination is recommended, malignancy should also be considered in the differential diagnosis of lymphadenopathy following vaccination.

Fulminant elderly adult-onset Still disease effectively treated with tocilizumab and methotrexate: A case report.

RATIONALE: Adult-onset Still disease (AOSD) is a rare inflammatory disease of unknown etiology. AOSD is common in young or middle-aged adults; however, in recent years, there have been increasing reports of elderly AOSD. Differentiating AOSD from diseases such as infections and malignancies is difficult. Moreover, rare fulminant AOSD cases with resistance to corticosteroids and immunosuppressive drugs have been reported. PATIENT CONCERNS: An 80-year-old woman presented with flaccid fever, generalized arthralgia, and erythema of the anterior chest for 2 weeks. On day 5 of hospitalization, the patient developed pleural effusion with hypoxemia and her vital signs indicated rapid progression to shock. During the clinical course, the levels of inflammatory markers, including maximum level of ferritin and white blood cells (WBCs) were elevated (252,796 ng/mL and 86,500/µV, respectively) with disseminated intravascular coagulation syndrome (DIC) and macrophage activation syndrome (MAS). DIAGNOSIS: The patient was diagnosed with elderly AOSD as per the Yamaguchi criteria for AOSD. The state of disease was extremely severe with rapid progression and was, thus, categorized as a fulminant form of elderly AOSD. INTERVENTIONS: The patient was treated with prednisolone (PSL) pulse therapy (1000 mg/d) twice and plasma exchange in the intensive care unit for the primary disease and shock. Although she recovered from shock, she developed DIC and MAS. Methotrexate (MTX; 10 mg/d) improved the DIC and MAS. However, severe pleuritis recurred and the patient developed pericarditis; her primary disease was poorly controlled. Finally, tocilizumab (TCZ) was introduced using interleukin-18 (IL-18) as a surrogate marker. The IL-18 level was measured repeatedly following admission, with the peak level (170,000 pg/mL) recorded on the 75th day of hospitalization, immediately prior to introducing TCZ. OUTCOMES: The combined use of MTX, TCZ, and PSL was effective in suppressing elderly AOSD, which was unsuccessfully controlled with MTX and PSL. Frequent monitoring of IL-18 levels proved useful for differentiating elderly AOSD from other diseases. LESSONS: A fulminant form of elderly AOSD was treated with a combination of MTX, TCZ, and PSL. Repeated monitoring of IL-18 levels can be useful for decision-making in treating elderly AOSD.

Bilateral inguinal bladder hernias.

Inguinal hernia may contain the bladder as one of its contents, while bilateral inguinal bladder herniation is rare. Urinary obstruction and obesity are associated with increased abdominal pressure and are risk factors of bladder herniation. Clinicians should be aware of the bladder hernia in elderly with chronic dysuria and obesity.

Frequent occurrence of postbreakfast syncope due to carotid sinus syndrome after surgery for hypopharyngeal cancer: A case report.

RATIONALE: Syncope often occurs in patients with advanced head and neck cancers due to the stimulation of the autonomic nervous system by the tumor. Here, we describe a case of frequent syncopal episodes after laryngopharyngectomy for hypopharyngeal cancer. As all syncopal episodes were observed during the forenoon, we also evaluated the heart rate variability using ambulatory electrocardiography to determine why the syncopal episodes occurred during a specified period of the day. PATIENT CONCERNS: A 73-year-old Japanese man who underwent laryngopharyngectomy for recurrent hypopharyngeal cancer started experiencing frequent episodes of loss of consciousness that occurred during the same time period (10:00-12:00). He had never experienced syncopal episodes before the operation. From 23 to 41 days postoperatively, he experienced 9 syncopal episodes that occurred regardless of his posture. DIAGNOSES: Pharyngo-esophagoscopy revealed an anastomotic stricture between the free jejunum graft and the upper esophagus. Swallowing videofluoroscopy confirmed the dilatation of the jejunal autograft and a foreign body stuck on the oral side of the anastomosis. Contrast-enhanced computed tomography revealed that the carotid artery was slightly compressed by the edematous free jejunum. The patient was diagnosed with carotid sinus syndrome (CSS) as the free jejunum was dilated when consuming breakfast, which may have caused carotid sinus hypersensitivity and induced a medullary reflex. INTERVENTIONS: Administration of disopyramide was effective in preventing syncope. Heart rate variability analysis using ambulatory electrocardiography showed that parasympathetic dominancy shifted to sympathetic dominancy during 10:00 to 12:00. The significant time regularity of the syncopal episodes may have been affected by modified diurnal variation in autonomic tone activity. OUTCOMES: After the surgical release and re-anastomosis of the pharyngoesophageal stenosis via an open-neck approach, no recurrent episodes of syncope were reported. LESSONS: We reported a case of frequent syncopal episodes limited to the forenoon due to CSS after surgery for hypopharyngeal carcinoma. The patient was treated with anticholinergics followed by the release and re-anastomosis of the pharyngoesophageal stenosis. When syncope occurs after surgery for head and neck lesions, CSS due to postoperative structural changes should be considered as a differential diagnosis of syncope.

Successful surgical drainage with intraoperative ultrasonography for amebic liver abscess refractory to metronidazole and percutaneous drainage: a case report.

BACKGROUND: Metronidazole (MNZ) has been clearly established as a medication for amebic liver abscess. In uncomplicated cases, surgical drainage should be avoided. We report a case of amebic liver abscess refractory to MNZ that was successfully treated using preoperative computed tomography (CT) and percutaneous and surgical drainage with intraoperative ultrasonography (IOUS). CASE PRESENTATION: A 53-year-old man with high-grade fever was diagnosed with a cystic lesion on his right hepatic lobe using CT. Percutaneous drainage was performed, and antibacterial drugs were administered. However, the infection and condition of the patient worsened. Entamoeba histolytica was detected from pus within the mediastinal cavity. Hence, the patient was diagnosed with amebic liver abscess. After the diagnosis was established, we administered MNZ for 10 days. Despite this, the patient's physical condition did not improve. Blood tests suggested impending disseminated intravascular coagulation (DIC). We performed surgical intervention to drain the amebic liver abscess refractory to conservative treatment. During surgery, imaging information from preoperative CT and IOUS enabled us to recognize the anatomical structures and determine the incision lines of the hepatic capsule and hepatic tissue. The patient's DIC immediately regressed after surgery. Unfortunately, malnutrition and disuse syndrome contributed to the patient's long recovery period. He was discharged 137 days post-surgery. CONCLUSIONS: We reported a case of amebic liver abscess refractory to conservative treatment. Surgical drainage with preoperative CT and IOUS allowed us to safely and effectively perform complex abscess decompression.

A high concentration of triiodothyronine attenuates the stimulatory effect on hemin-induced erythroid differentiation of human erythroleukemia K562 cells.

Although thyroid hormone is a known stimulator of erythropoietic differentiation, severe anemia is sometimes observed in patients with hyperthyroidism and this mechanism is not fully understood. The aim of this study was to investigate the effect of triiodothyronine (T3) on hemin-induced erythropoiesis. Human erythroleukemia K562 cells were used as an erythroid differentiation model. Cell differentiation was induced by hemin and the effect of pre-incubation with T3 (0.1 to 100 nM) was analyzed by measuring the benzidine-positive rate, hemoglobin content, CD71 expression (transferrin receptor), and mRNA expression for transcription factors related to erythropoiesis and thyroid hormone receptors (TRs). Hemin, a promoter of erythroid differentiation, increased the levels of mRNAs for TRα, TRß, and retinoid X receptor α (RXRα), as well as those for nuclear factor-erythroid 2 (NFE2), GATA-binding protein 1 (GATA1) and GATA-binding protein 2 (GATA2). Lower concentrations of T3 had a stimulatory effect on hemin-induced hemoglobin production (1 and 10 nM), CD71 expression (0.1 nM), and α-globin mRNA expression (1 nM), while a higher concentration of T3 (100 nM) abrogated the stimulatory effect on these parameters. T3 at 100 nM did not affect cell viability and proliferation, suggesting that the abrogation of erythropoiesis enhancement was not due to toxicity. T3 at 100 nM also significantly inhibited expression of GATA2 and RXRα mRNA, compared to 1 nM T3. We conclude that a high concentration of T3 attenuates the classical stimulatory effect on erythropoiesis exerted by a low concentration of T3 in hemin-induced K562 cells.

Management of primary iliopsoas abscess in an immunocompetent patient followed by streptococcal toxic shock syndrome: A case report.

OBJECTIVES: Clinical diagnosis of iliopsoas abscess can be challenging, as this pathology often presents without obvious focal findings. Iliopsoas abscess should thus be a differential diagnosis for patients presenting with fever of unknown origin. PATIENT AND METHODS: A 62-year-old healthy Japanese man showed primary iliopsoas abscess of Klebsiella pneumoniae complicated by shock after a complete course of treatment for streptococcal toxic shock syndrome. Successful treatment was achieved with culture-driven antibiotic selection and delayed drainage. RESULTS: This case demonstrates the importance of identifying the causative microorganisms in iliopsoas abscess to guide therapy. The standard treatments for iliopsoas abscess are antibiotics and drainage of the abscess. Management of this case included successful antibiotic use along with delayed drainage. CONCLUSION: This case report advances the knowledge on the etiology of iliopsoas abscess and sheds light on the need for scientific development of a treatment strategy.

[A case of small intestinal cancer in the efferent loop of roux-en Y reconstruction after total gastrectomy and liver metastases].

A 61-year-old woman, who had undergone total gastrectomy and distal splenopancreatectomy with Roux-en Y reconstruction for a gastric cancer 16 years earlier, was found primary small intestinal cancer located in intestinal loop of Roux- en Y in gastrointestinal endoscopy for abdominal pain. Computed tomography showed liver metastases which were 8 cm in diameter at lateral segment and 1 cm in diameter at segment 8 of the liver. In the operation, the small intestinal cancer was located in the ρ-anastomosis in the loop of Roux-en Y with the other jejunum fistula. We performed lateral segment hepatectomy, partial hepatectomy of segment 8, partial resection of small intestine including loop of ρ-Roux-en Y, partial resection of transverse colon, and restoration Roux-en Y again. We succeeded in preserving double tract anastomosis at duodenum. Histological examination revealed a moderately differentiated adenocarcinoma of the small intestine and segment 8 of the liver, and angiomyolipoma of lateral segment of the liver. It is extremely rare for small intestinal cancer to arise in a loop of Roux-en Y reconstruction caused by total gastrectomy.