Case report: Five patients with myocarditis after mRNA COVID-19 vaccination.

Objectives: To describe clinical features and laboratory data of myocarditis after the mRNA COVID-19 vaccine in children. Methods: We reviewed patients younger than 18 years of age, who visited our hospital because of myocarditis within 1 week after BNT162b2 from June 2021 to January 2022. Results: We identified five male patients aged 12-16 years who presented to our hospital with myocarditis within 2-3 days after the second dose of BNT162b2 COVID-19 vaccination between June 2021 and January 2022. All patients experienced chest pain, and fever, pain other than chest pain, and shortness of breath were present in two, three, and two patients, respectively. The serum troponin I level was increased in all patients except one, and electrocardiogram (ECG) showed ST elevation in all patients. Echocardiography revealed pericardial effusion and decreased ejection fraction in three and one patients, respectively. In accordance with the Japanese guidelines for myocarditis, the patients were treated with colchicine and aspirin. Chest pain improved within a few days with no hemodynamic instability. The patients were discharged with no sequelae. Conclusions: ST changes on ECG and elevated troponin I levels may aid the diagnosis of myocarditis after mRNA COVID-19 vaccination.

The clinical outcome of the patients with horizontal semicircular canal variants of benign paroxysmal positional vertigo.

BACKGROUND: Gufoni maneuver is known to be effective for horizontal canal benign positional vertigo (HC-BPPV), but there are some intractable patients that the treatment procedure does not work. OBJECTIVE: The clinical outcomes of patients with HC-BPPV were investigated. We also investigated the characteristics of intractable patients which needed long time to the remission. METHODS: Sixty-six patients with HC-BPPV receiving Gufoni maneuver at Tokyo Medical University Hachioji Medical Center were investigated. The patients were classified into geotropic DCPN group and apogeotropic DCPN group. The clinical outcomes in 2 groups were examined. RESULTS: There were 48 patients with geotropic DCPN and 18 patients with apogeotropic DCPN. There were significant differences between the geotropic HC-BPPV and apogeotropic HC BPPV in the period to remission. There were 7 intractable patients and the average ages of these intractable patients were higher than other patients. CONCLUSION: The patients with geotropic DCPN and the patients with the nystagmus conversion from apogeotropic to geotropic DCPN have tendency to easy to resolve, but in patients with apogeotropic type without nystagmus transformation have long time to resolve. The elderly patients whose nystagmus remains apogeotropic without nystagmus conversion have tendencies to become intractable.

Electronystagmography in 166 patients with acoustic neuroma before and after surgery.

OBJECTIVE: To analyze the preoperative patterns of caloric test, eye tracking test (ETT), and optokinetic pattern (OKP) in patients with acoustic neuroma (AN) and compare them with the postoperative patterns of ETT and OKP results METHODS: A total of 166 patients with AN (102 women; mean age: 41 years, range: 11-79 years) who were being treated at our hospital between 2013 and 2016 were enrolled. Preoperatively, a detailed history was taken regarding the presence of subjective symptoms of equilibrium dysfunction, and the patients underwent caloric test, ETT, and OKP. They were classified into three groups based on the preoperative ETT and OKP results as follows: Group A, normal ETT and OKP; Group B, either ETT or OKP was abnormal; and Group C, both ETT and OKP were abnormal. All patients were evaluated for subjective symptoms of vestibular dysfunction and were also grouped based on the tumor size on imaging. All surgeries were performed by a neurosurgeon using the lateral suboccipital retrosigmoid approach. About one month later after surgery, postoperatively ETT was performed on 150 patients and OKP was performed on 148 patients. The preoperative and postoperative ETT and OKP results were compared. The same two specialists analyzed the postoperative ETT and OKP findings as improved, unchanged, or worse. Student t-test was used for statistical analysis and a P-value of <0.05 was considered to indicate a statistically significant difference. RESULTS: The average canal paresis(CP) % was 65.8%. No correlation was found between tumor size and CP%. The other side, the average tumor size in each group was 26.6 mm, 28.7 mm, and 37.8 mm in the Group A, B, and C, respectively. The average tumor size in Group C was significantly greater than those of Group A and B (P<0.01). The presence of gait disturbance in Group C was significantly higher than the other groups (P<0.01). The other side, abnormal ETT and OKP were seen in 32.5% and 31.9% of all patients, respectively. ETT and OKP results improved postoperatively in 67.4% and 68.9% of these patients. CONCLUSIONS: Abnormal ETT and OKP results showed positive correlations with the tumor size and presence of subjective symptoms. Further, dysfunction of cerebellum and brain stem owing to tumor compression was observed to recover in many cases after surgery.

Overlapping Features in Kawasaki Disease-Related Arthritis and Systemic-Onset Juvenile Idiopathic Arthritis: A Nationwide Study in Japan.

Background: Arthritis may occur after the diagnosis of Kawasaki disease (KD). Most cases are self-limiting; however, some patients require prolonged treatment. Method: To characterize KD-related arthritis, 14 patients who required arthritis treatment within 30 days after the diagnosis of KD were recruited from the 23rd KD survey in Japan. Twenty-six additional patients were included from our tertiary center and literature review cohorts. Results: The estimated prevalence of KD-related arthritis in Japan was 48 per 100,000 KD patients. Patients with KD-related arthritis had an older age at onset (52 vs. 28 months, P = 0.002) and higher rate of intravenous immunoglobulin (IVIG) resistance in comparison to those without arthritis (86 vs. 17%, P < 0.001). Among 40 patients, 18 had arthritis in the acute phase KD (continued fever-onset type) and 22 did in the convalescent phase (interval fever-onset type). Both showed a similar rate of complete KD or IVIG response. Interval-type patients required biologics for arthritis control less frequently (5 vs. 39%, P = 0.02) and had a higher 2-year off-treatment rate (100 vs. 43%, P = 0.009) than continued-type ones. Interval-types showed lower serum ferritin and interleukin-18 levels than continued-types. When continued-types were grouped according to whether or not they required biologics (n = 7 and n = 11, respectively), the former subgroup had higher ferritin and interleukin-18 levels (P = 0.01 and 0.02, respectively). A canonical discriminant analysis differentiated interval-type from continued-type with the combination of age, time to arthritis, and the ferritin and matrix metalloproteinase-3 levels. Conclusion: Arthritis requiring treatment is a rare complication of KD. KD-associated arthritis includes interval-type (KD-reactive) and continued-type (true systemic-onset juvenile idiopathic arthritis [JIA] requiring biologics), and overlapping arthritis, suggesting the pathophysiological continuity of autoinflammation between KD and JIA.

Phosphorylated proteome analysis of a novel germline ABL1 mutation causing an autosomal dominant syndrome with ventricular septal defect.

BACKGROUND: A gain-of-function mutation in germline ABL1 causes a syndrome including congenital heart defects. However, the molecular mechanisms of this syndrome remain unknown. In this study, we found a novel ABL1 mutation in a Japanese family with ventricular septal defect, finger contracture, skin abnormalities and failure to thrive, and the molecular mechanisms of these phenotypes were investigated. METHODS AND RESULTS: Whole-exome sequencing on several family members revealed a novel mutation (c.1522A > C, p.I508L) in the tyrosine kinase domain of ABL1, and complete co-segregation with clinical presentations was confirmed in all members. Wild-type and mutant ABL1 were transfected into human embryonic kidney 293 cells for functional analysis. Western blotting confirmed that tyrosine phosphorylation in STAT5, a substrate of ABL1, was enhanced, and the novel mutation was proved to be a gain-of-function mutation. Since this novel mutation in ABL1 enhances tyrosine kinase activity, phosphorylated proteome analysis was used to elucidate the molecular pathology. The proteome analysis showed that phosphorylation in proteins such as UFD1, AXIN1, ATRX, which may be involved in the phenotypes, was enhanced in the mutant group. CONCLUSIONS: The onset of congenital heart defects associated with this syndrome appears to involve a mechanism caused by UFD1 common to 22q.11.2 deletion syndrome. On the other hand, AXIN1 and ATRX may be important in elucidating the mechanisms of other phenotypes, such as finger contracture and failure to thrive. Verification of these hypotheses would lead to further understanding of the pathophysiology and the development of treatment methods.

Reactivation of human herpesviruses 6 and 7 in Kawasaki disease.

Objectives: Kawasaki disease (KD) is one of the most common childhood vasculitides. Some serological studies have suggested an etiological relationship between KD and human herpesvirus (HHV)-6 or HHV-7. However, primary or reactivated HHV-6 and -7 has not been fully investigated in patients with KD. Methods: Twenty-three patients with KD were prospectively enrolled in this study. Peripheral blood was collected in the acute and convalescence phases, and HHV-6 and -7 viral loads were measured by real-time PCR. Results: In the acute phase, HHV-6 and -7 DNA was detected in 7 (30%) patients each, compared to 13 (57%) and 9 (39%) patients in the convalescence phase, respectively. HHV-6 and -7 DNA loads were significantly higher in the convalescence phase than in the acute phase. Significant increases in HHV-6 and -7 DNA loads were not observed in disease control patients. Taking into account HHV-6 and -7 serostatus, reactivation of HHV-6 and -7 was observed in 7 and 9 patients, respectively. KD patients with HHV-6 reactivation showed higher C-reactive protein levels and more frequently required steroid therapies than patients without reactivation. Conclusion: HHV-6 and -7 reactivation is frequent in KD patients. HHV-6 reactivation might exacerbate the severity of KD.

Comparison of cervical vestibular evoked potentials evoked by air-conducted sound and bone-conducted vibration in vestibular Schwannoma patients.

OBJECTIVE: The vestibular evoked myogenic potential (VEMP) is associated with otolithic afferents and can be used to evaluate the function of the saccule and utricle. In this study, we compared cervical VEMP evoked by stimulation with Air-conducted sound (ACS) and bone-conducted vibration (BCV) to the forehead and investigated whether BCV can be used as a substitute for ACS. METHODS: Data were obtained from 33 patients with vestibular schwannoma. Vestibular examinations were performed preoperatively. VEMP was obtained upon stimulation with ACS (ACS cVEMP) and BCV to the forehead using a minishaker (BCV cVEMP). Vestibular function was also analyzed using the caloric test and ocular VEMP (oVEMP) testing. oVEMP was measured using bone-conductive vibration to the forehead. The results of BCV cVEMP, ACS cVEMP, and oVEMP were compared by the caloric test. RESULTS: Rates of patients with abnormal ACS cVEMP, BCV cVEMP, oVEMP, and caloric test results were 78.8%, 75.8%, 78.8%, and 69.7%, respectively. BCV cVEMP did not correlate with ACS cVEMP, but correlated with oVEMP and caloric test results. CONCLUSION: BCV cVEMP did not correlate with ACS cVEMP. Therefore, BCV cVEMP cannot be used as a substitute for ACS cVEMP.

Hemostasis with emergently modified application of intra-aortic balloon occlusion in a patient with impending cardiac arrest following blunt proximal thigh amputation.

Case: A 30-year-old woman had her left thigh run over by a train. We tried to compress the left femoral area to control the arterial bleeding, but bleeding continued from the stump and injured soft tissue. The application of a tourniquet bandage also failed because of the limited remaining thigh. She developed impending cardiac arrest. As the left femoral arterial pulsation was still palpable, we inserted an intra-aortic balloon occlusion catheter percutaneously. The hemorrhage from the stump region decreased rapidly. She was transferred to an operating room to carry out surgical hemostasis, and it was confirmed with deflation of the balloon in the common iliac artery. Outcome: There was no complication of the skin or soft tissue at the surgical site caused by impaired circulation, and her consciousness fully recovered. Conclusion: We report the successful control of bleeding by the emergently modified application of intra-aortic balloon occlusion in the left common iliac artery.

Spontaneous inversion of nystagmus without a positional change in the horizontal canal variant of benign paroxysmal positional vertigo.

OBJECTIVE: We investigated the neuro-otological findings, including nystagmus, and the clinical course of patients with the horizontal canal variant of benign paroxysmal positional vertigo (HC-BPPV), who showed spontaneous inversion of nystagmus without a positional change. Furthermore, we speculated on the possible mechanism of spontaneous inversion of nystagmus without a positional change. PATIENTS AND METHODS: The characteristics of spontaneous inversion of positional nystagmus without a positional change were analyzed in 7 patients with HC-BPPV. RESULTS: All patients were diagnosed as having HC-BPPV. During the positional test, the spontaneous inversion of nystagmus was observed in the same head position in all patients. Spontaneous inversion was observed on both sides in 5 patients, and only on 1 side in 2 patients. All patients presented with geotropic nystagmus in the first phase, and ageotropic nystagmus in the second phase. CONCLUSIONS: The coexistence of cupulolithiasis and canalolithiasis appears to be a possible mechanism of the spontaneous inversion of positional nystagmus.

A case of nasal glial heterotopia in an adult.

We report a rare case of nasal glial heterotopia in an adult. After the surgery, frontal lobe cerebral hemorrhage developed. A 58-year-old man had unilateral nasal obstruction that progressed for one year. He had been treated for hypertension, chronic heart failure, and cerebral infarction with aspirin and warfarin. A computed tomography scan showed that the tumor occupied the right nasal cavity and the sinuses with small defect in the cribriform plate. The tumor was removed totally with endoscopy. After the operation, the patient developed convulsions and frontal lobe cerebral hemorrhage. The hemorrhage site was located near a defect in the cribriform plate. Nasal glial heterotopia is a rare developmental abnormality, particularly rare in adult. Only few cases were reported. We could not find any report of adult nasal glial heterotopias that developed cerebral hemorrhage as a complication of the surgery.

Intermittent positional downbeat nystagmus of cervical origin.

Intermittent positional down beat nystagmus (p-DBN) is rare. We describe an unusual case of intermittent p-DBN which was induced by rotation, anteflexion, and lateral flexion of the neck. A 59-year-old man complained of loss of consciousness and lightheadedness. Positional testing revealed the p-DBN. The evoked p-DBN had latency and the patient had a feeling of passing out while the p-DBN was present. There were no abnormal findings in the vestibular functional examinations. Findings of the MRI were negative. MRA revealed no stenosis of the vertebral artery bilaterally, but there was an anatomical difference. The p-DBN characteristics were documented by electronystagmography during the positional test. The p-DBN lasted intermittently while maintaining the provoking position. It was found that p-DBN occurred with not only the rotation of the neck, but also in the anteflexion and lateral flexion of the neck. There was no stenosis of the vertebral artery (VA) on angiography, but we speculated that the cause of the p-DBN was the VA occlusion due to rotation, anteflexion, and lateral flexion of the neck.

Ocular vestibular evoked myogenic potentials induced by bone-conducted vibration in patients with unilateral inner ear disease.

CONCLUSION: Patients with vestibular neuritis (VN) with complete canal paresis (CP) showed a higher rate of abnormal ocular vestibular evoked myogenic potential (oVEMP) than those with partial CP. From these results, it is speculated that the superior vestibular nerve function mainly affects oVEMP. Significant correlation was found between the grades of the hearing outcome and oVEMP in sudden sensorineural hearing loss (SSHL). OBJECTIVE: We attempted to correlate the results of oVEMP with the results of cervical VEMP (cVEMP), results of subjective visual vertical (SVV), and clinical course in patients with various vestibular disorders. METHODS: Twenty-two patients with VN, 65 with SSHL, and 22 with Meniere's disease (MD), were enrolled in this study. We compared the results of oVEMP with those of cVEMP, SVV, and the caloric test. Furthermore, the oVEMP results were compared with the initial hearing threshold, presence of vertigo, and hearing recovery in the patients with SSHL. RESULTS: The patients with VN with complete CP showed a higher rate of abnormal oVEMP than those with partial CP. In the patients with SSHL, the hearing recovery rate was lower in the patients with abnormal oVEMP than in those with normal oVEMP.

Osteoma of the pharynx that developed from the hyoid bone.

This paper reports on apparently the first case of a pharyngeal osteoma that developed from the hyoid bone. An 84-year-old man's, presenting symptom was a slight throat pain. Endoscopic examination revealed a huge mass occluding the pharyngeal space. CT scan of the neck showed a large osseous mass adjacent to the hyoid bone. Transoral resection with tracheostomy was performed. Histopathologically, the tumor consisted of mature lamellar bone without a fibrous component. For two years postoperatively, the patient has been free from throat symptoms and signs of recurrence. Osteomas are benign, slow-growing tumors. They rarely develop symptoms or cause functional disturbance. We performed total resection to avoid further functional disturbance as the osteoma was huge. To the best of our knowledge, this is the first report on an osteoma that occupied the pharyngeal space and developed from the hyoid bone.

Gender differences in the clinical characteristics among Japanese patients with obstructive sleep apnea syndrome.

BACKGROUND: Gender differences in the prevalence of various manifestations of obstructive sleep apnea syndrome (OSAS) is not as great as previously believed. The aim of the present study was to clarify the clinical patient characteristics of Japanese women and men with OSAS. METHODS: A cross-sectional case-match control study was performed on patients from two sleep disorder centers. Two hundred forty-five women with OSAS were classified into premenopausal (n = 70) and postmenopausal (n = 175) groups. As well, 245 men matched for both age and apnea-hypopnea index (AHI) and another 245 men matched for age and body mass index (BMI) were established. We compared descriptive variables between genders in both the premenopausal and the postmenopausal female patient groups. RESULTS: As a whole, female patients had significantly higher BMI than AHI-matched male patients (p < 0.05) and a significantly lower value of AHI than BMI-matched male patients (p < 0.001). Female patients had lower Epworth Sleepiness Scale scores than BMI-matched male patients (p < 0.05). On logistic regression analysis, presence of hypertension was significantly associated with BMI (>or=25 kg/m(2)), AHI (>or= 15 to < 30 events/h; >or= 30 to < 60 events/h; >or= 60 events/h), and presence of both hyperlipidemia and diabetes mellitus. However, gender differences were not associated with the occurrence of hypertension. Female patients had significantly lower optimal levels of continuous positive airway pressure than male patients. CONCLUSIONS: Our results suggest that both the OSAS severity and the strength of pharyngeal closure is less in Japanese female patients than in male patients. Moreover, Japanese female patients are thought to have less daytime sleepiness than male patients but a similar rate of hypertension as male patients.

Upper thoracic extralobar pulmonary sequestration with anomalous blood supply from the subclavian artery.

The authors report on a newborn boy with extralobar pulmonary sequestration in the right upper thoracic region. Preoperative angiography showed an anomalous large vessel from the right subclavian artery, supplying the sequestrated lobe. Right thoracotomy was performed to resect the sequestrated lobe, which was diagnosed as extralobar form. This is an extremely rare case of extralobar pulmonary sequestration in which anomalous blood supply from the subclavian artery was seen preoperatively on radiographs. The authors recommend angiographic examination, particularly in case of diagnostic difficulty.