Case report: Neuronal intranuclear inclusion disease initially mimicking reversible cerebral vasoconstriction syndrome: serial neuroimaging findings during an 11-year follow-up.

Neuronal intranuclear inclusion disease (NIID) is a rare, progressive neurodegenerative disorder known for its diverse clinical manifestations. Although episodic neurogenic events can be associated with NIID, no reported cases have demonstrated concurrent clinical features or MRI findings resembling reversible cerebral vasoconstriction syndrome (RCVS). Here, we present the inaugural case of an adult-onset NIID patient who initially displayed symptoms reminiscent of RCVS. The 59-year-old male patient's initial presentation included a thunderclap headache, right visual field deficit, and confusion. Although his brain MRI appeared normal, MR angiography unveiled left posterior cerebral artery occlusion, subsequently followed by recanalization, culminating in an RCVS diagnosis. Over an 11-year period, the patient encountered 10 additional episodes, each escalating in duration and intensity, accompanied by seizures. Simultaneously, cognitive impairment progressed. Genetic testing for NIID revealed an abnormal expansion of GGC repeats in NOTCH2NLC, with a count of 115 (normal range, <60), and this patient was diagnosed with NIID. Our report highlights that NIID can clinically and radiologically mimic RCVS. Therefore, in the differential diagnosis of RCVS, particularly in cases with atypical features or recurrent episodes, consideration of NIID is warranted. Additionally, the longitudinal neuroimaging findings provided the course of NIID over an 11-year follow-up period.

Head-neck movement may predispose to the development of arytenoid dislocation in the intubated patient: a 5-year retrospective single-center study.

BACKGROUND: Arytenoid dislocation is a rare laryngeal injury that may follow endotracheal intubation. We aimed to determine the incidence and risk factors for arytenoid dislocation after surgery under general anaesthesia. METHODS: We reviewed the medical records of patients who underwent operation under general anaesthesia with endotracheal intubation from January 2014 to December 2018. Patients were divided into the non-dislocation and dislocation groups depending on the presence or absence of arytenoid dislocation. Patient, anaesthetic, and surgical factors associated with arytenoid dislocation were determined using Poisson regression analysis. RESULTS: Among the 25,538 patients enrolled, 33 (0.13%) had arytenoid dislocation, with higher incidence after anterior neck and brain surgery. Patients in the dislocation group were younger (52.6 ± 14.4 vs 58.2 ± 14.2 yrs, P = 0.025), more likely to be female (78.8 vs 56.5%, P = 0.014), and more likely to be intubated by a first-year anaesthesia resident (33.3 vs 18.5%, P = 0.048) compared to those in the non-dislocation group. Patient positions during surgery were significantly different between the groups (P = 0.000). Multivariable Poisson regression identified head-neck positioning (incidence rate ratio [IRR], 3.10; 95% confidence interval [CI], 1.50-6.25, P = 0.002), endotracheal intubation by a first-year anaesthesia resident (IRR, 2.30; 95% CI, 1.07-4.64, P = 0.024), and female (IRR, 3.05; 95% CI, 1.38-7.73, P = 0.010) as risk factors for arytenoid dislocation. CONCLUSION: This study showed that the incidence of arytenoid dislocation was 0.13%, and that head-neck positioning during surgery, less anaesthetist experience, and female were significantly associated with arytenoid dislocation in patients who underwent surgeries under general anaesthesia with endotracheal intubation.

Donepezil-related inadequate neuromuscular blockade during laparoscopic surgery: A case report.

BACKGROUND: Donepezil is an acetylcholinesterase inhibitor used to improve cognition and delay disease progression in dementia patients by increasing acetylcholine levels. This drug may potentially interact with neuromuscular blocking agents (NMBAs) that act on muscular acetylcholine receptors during general anesthesia. Herein, we present a case of inadequate neuromuscular blockade with rocuronium, a nondepolarizing NMBA, in a dementia patient who had taken donepezil. CASE SUMMARY: A 71-year-old man was scheduled for laparoscopic gastrectomy. He had been taking donepezil 5 mg for dementia. General anesthesia was induced with propofol and remifentanil. The depth of neuromuscular blockade was monitored by train-of-four (TOF) stimulation. After the administration of rocuronium, the TOF ratio decreased at an unusually slow rate, and a TOF count of 0 was detected 7 min later. After intubation, a TOF count of 1 was detected within 1 min, and a TOF ratio of 12% was detected within 2 min. The TOF count remained at 4 even with an additional bolus and continuous infusion of rocuronium, suggesting resistance to this NMBA. Instead of propofol, an inhalation anesthetic was administered alongside another NMBA (cisatracurium). Then, the quality of neuromuscular blockade improved, and the TOF count remained at 0-1 for the next 70 min. No further problems were encountered with respect to surgery or anesthesia. CONCLUSION: Donepezil may be responsible for inadequate neuromuscular blockade during anesthesia, especially when total intravenous anesthesia is used.

Similarity Analysis of Korean Medical Literature and Its Association with Efforts to Improve Research and Publication Ethics.

In the present study, the frequency of research misconduct in Korean medical papers was analyzed using the similarity check software iThenticate®. All Korean papers written in English that were published in 2009 and 2014 in KoreaMed Synapse were identified. In total, 23,848 papers were extracted. 4,050 Journal Articles of them were randomly selected for similarity analysis. The average Similarity Index of the 4,050 papers decreased over time, particularly in 2013: in 2009 and 2014, it was 10.15% and 5.62%, respectively. And 357 (8.8%) had a Similarity Index of ≥ 20%. Authors considered a Similarity Index of ≥ 20% as suspected research misconduct. It was found that iThenticate® cannot functionally process citations without double quotation marks. Papers with a Similarity Index of ≥ 20% were thus individually checked for detecting such text-matching errors to accurately identify papers with suspected research misconduct. After correcting text-matching errors, 142 (3.5% of the 4,050 papers) were suspected of research misconduct. The annual frequency of these papers decreased over time, particularly in 2013: in 2009 and 2014, it was 5.2% and 1.7%, respectively. The decrease was associated with the introduction of CrossCheck by KoreaMed and the frequent use of similarity check software. The majority (81%) had Similarity Indices between 20% and 40%. The fact suggested that low Similarity index does not necessarily mean low possibility of research misconduct. It should be noted that, although iThenticate® provides a fundamental basis for detecting research misconduct, the final judgment should be made by experts.

Effect of nefopam- versus fentanyl-based patient-controlled analgesia on postoperative nausea and vomiting in patients undergoing gynecological laparoscopic surgery: a prospective double-blind randomized controlled trial.

OBJECTIVE: This study comparatively evaluated the effect of patient-controlled analgesia (PCA) regimens using equipotent doses of nefopam or fentanyl during laparoscopic gynecological surgery on postoperative nausea and vomiting (PONV). RESEARCH DESIGN AND METHODS: Patients undergoing gynecological laparoscopic surgery were randomly allocated to receive either nefopam- (non-opioid; N group) or fentanyl-based (F group) PCA. PONV and postoperative pain were assessed during the 72 hours following discharge from the post-anesthetic care unit (PACU). The adverse effects of nefopam were also evaluated. CLINICAL TRIAL REGISTRATION: Cris.nih.go.kr ID KCT0000783. RESULTS: In total, 94 patients were included in the final analysis. The PONV incidence and scale and the Rhodes index scores were significantly lower in the N group than the F group at all measured times. The N group exhibited a significantly lower incidence of PONV (15/47 [31.9%] vs. 27/47 [57.4%], respectively; P = 0.022) and severity of PONV (0 [1] vs. 1 [2], respectively; P = 0.005) 24 hours after PACU discharge and a significantly lower Rhodes index score (0 [3] vs. 5 [9], respectively; P = 0.002) from 30 minutes after PACU arrival to 24 hours after PACU discharge than did the F group. There was no significant difference in postoperative pain at any time between the two groups. Dry mouth on PACU arrival was significantly more frequent in the N group. However, the frequency of dry mouth decreased after PACU arrival in the N group, resulting in a significantly lower incidence 24 hours after PACU discharge. CONCLUSIONS: Use of a PCA regimen with nefopam for analgesia was associated with a similar degree of pain control and superior PONV outcomes 24 hours after PACU discharge and no adverse events compared with a PCA regimen using an equipotent dose of fentanyl.

Effects of percutaneous stimulation of both sympathetic and parasympathetic cardiac autonomic nerves on cardiac function in dogs.

OBJECTIVE: Augmentation of left ventricular (LV) contractility and heart rate (HR) by sympathetic nerve stimulation and amelioration of heart failure by vagal nerve stimulation has been reported. However, the effects of concomitant electrical stimulation of both sympathetic and parasympathetic cardiac nerves in tissues such as those of the cardiac plexus remain unclear. This study sought to assess acute changes in cardiac function and hemodynamics in response to endovascular cardiac plexus stimulation (CPS). METHODS: Twelve dogs received endovascular CPS via a bipolar catheter within the right pulmonary artery. Stimulation frequency (20 Hz) and pulse width (4 milliseconds) were fixed; voltage varied (range, 15-60 V). RESULTS: Results fell into three categories: 1, no response (n = 4); 2, an increase in systemic arterial pressure that was dependent on electrode placement (n = 4); and 3, a very reproducible and stable increase in aortic pressure (n = 4). In the third group, mean systolic aortic pressures, maximum value of the first derivative of LV pressure, and LV stroke work increased with stimulation (P < 0.02 for all parameters) as did cardiac output, end-systolic elastance, and preload recruitable stroke work (P = 0.03). Systemic and pulmonary vascular resistance, central venous pressure, pulmonary arterial pressure, and HR remained unchanged (P > 0.05). CONCLUSIONS: In contrast to conventional inotropic agents, endovascular CPS induced significant and selective increases in LV contractility without increasing HR. Efforts to optimize electrode placement and fixation will improve the reproducibility of endovascular CPS treatment.

Effect of epivascular cardiac autonomic nerve stimulation on cardiac function.

BACKGROUND: The cardiac plexus contains sympathetic and parasympathetic cardiac nerves. Our goal was to assess the hemodynamic and functional effects of stimulating the cardiac autonomic nervous system (CANS) at the epivascular surfaces of the cardiac plexus. Although CANS therapy to modulate cardiovascular function has drawn widespread interest, research has focused only on stimulating parasympathetic or sympathetic nerves, not both at once. METHODS: Using general anesthesia and an open-chest surgical procedure, 12 dogs received epivascular stimulation of the cardiac plexus. A bipolar electrode was placed between the right pulmonary artery (PA) and the ascending aorta, with the stimulation frequency/pulse width held constant (20 Hz/4 ms) and the voltage varied (10-50 V). Left ventricular (LV) pressure-volume loops and hemodynamic data were recorded with and without stimulation. RESULTS: In all dogs, aortic and LV systolic pressures, maximum rate of change of LV pressure, and LV stroke work increased (p<0.0001), as did cardiac output (2.9±1.0-0.4±1.0 L/min; p=0.001), end-systolic elastance (1.2±0.4-1.5±0.5 mm Hg/mL; p=0.0001), preload recruitable stroke work (30.1±11.0-39.3±7.8 mm Hg; p=0.003), and LV ejection fraction (p=0.012). Systemic vascular resistance increased slightly (p=0.04), and pulmonary vascular resistance decreased (p=0.01). Mean heart rate and pulmonary arterial, central venous, and left atrial pressures remained unchanged (p>0.1). CONCLUSIONS: In contrast to inotropic drugs, epivascular CANS stimulation induced a significant and selective increase in LV contractility with no increase in heart rate.

Surgical outcomes of hemorrhagic metastatic brain tumors.

PURPOSE: Hemorrhagic metastatic brain tumors are not rare, but little is known about the surgical outcome following treatment. We conducted this study to determine the result of the surgical outcome of hemorrhagic metastatic brain tumors. MATERIALS AND METHODS: From July 2001 to December 2008, 21 patients underwent surgery for hemorrhagic metastatic brain tumors at our institution. 15 patients had lung cancer, 3 had hepatocellular carcinoma, and the rest had rectal cancer, renal cell carcinoma, and sarcoma. 20 patients had macroscopic hemorrhage in the tumors, and one patient had intracerebral hemorrhage surrounding the tumor. A retrospective clinical review was conducted focusing on the patterns of presenting symptoms and signs, as well as local recurrence following surgery. RESULTS: Among 21 hemorrhagic brain metastases, local recurrence developed in two patients. The 12 month progression free survival rate was 86.1%. Mean time to progression was 20.8 months and median survival time after surgery was 11.7 months. CONCLUSION: The results of our study showed that hemorrhagic metastatic brain tumors rarely recurred after surgery. Surgery should be considered as a good treatment option for hemorrhagic brain metastasis, especially in cases with increased intracranial pressure or severe neurologic deficits.

Growth rate of newly developed metastatic brain tumors after thoracotomy in patients with non-small cell lung cancer.

Among 1372 lung cancer patients without brain metastasis that underwent resection of lung cancer at our center from 2001 to 2007, brain metastases developed in 72 patients (5.2%) during their hospital course. We hypothesized that there were micro-metastases in the brain at the time of lung surgery in these patients, even though there were no detectable brain metastases on the MRI. The purpose of this study was to evaluate the growth rates of metastatic brain tumors in this unique subset of patients, and to compare the findings with our previous study that calculated the growth rate of brain metastases during chemotherapy. Among 72 patients, 23 with cystic or hemorrhagic metastases were excluded. Seventy-six metastatic brain tumors in 49 patients were reviewed. Twenty-five patients underwent adjuvant or neoadjuvant chemotherapy; however, for the rest of the patients, chemotherapy was not added after lung cancer surgery. The tumor volume was determined using V-works software (v. 4.0) (Cybermed, Seoul, Korea) and T1 gadolinium enhanced MR images. The overall median tumor growth rate was 11.7 mm³/day (interquartile range, 4.9-26.8). There were no statistically significant differences in the tumor growth among the lung cancer stages and the growth rate was similar regardless of the use of chemotherapy. The growth rate reported in this study shows consistency with that of our previous report (12.1 mm³/day). These findings may help optimize patient management during follow up.

The expressions of carbonic anhydrase 9 and vascular endothelial growth factor in astrocytic tumors predict a poor prognosis.

Hypoxia in the tumor microenvironment triggers a variety of genetic and adoptive responses that regulate tumor growth. Tumor hypoxia is often associated with a malignant phenotype, resistance to therapy, and poor survival. The objectives of this study were to evaluate the expressions of carbonic anhydrase 9 (CA9) and vascular endothelial growth factor (VEGF) in astrocytic gliomas and to relate patterns of expression with prognosis, that is with histological grade and survival. We investigated 78 World Health Organization (WHO) grade II, III, and IV astrocytic gliomas. CA9 expression was examined in paraffin-embedded sections by immunohistochemistry. Fourteen tumors were grade II, 30 were grade III, and 34 were grade IV. It was found that CA9 expression was significantly associated with a higher-grade histology (p<0.001). There were 3 CA9 positive tumors in grade II (21.4%), 10 in grade III (33.3%), and 27 in grade IV (79.4%). For all tumors and WHO grade II, overall survival was found to be significantly dependent on CA9 expression (p=0.004, p=0.01). Furthermore, VEGF expression was found to be significantly related to tumor grade (p=0.02) and tended to be related to overall survival (p=0.1). However, no relation was found between the expression of CA9 and VEGF (p=0.17). Nevertheless, the expressions of CA9 and VEGF were found to be associated with tumor grade and possibly with survival. Further studies on a larger patient population are needed to determine the correlation between the expressions of CA9, and VEGF in astrocytic gliomas and clinical outcome.

Canine model of esophageal injury and atrial-esophageal fistula after applications of forward-firing high-intensity focused ultrasound and side-firing unfocused ultrasound in the left atrium and inside the pulmonary vein.

BACKGROUND: Left atrial-esophageal fistula is a serious and poorly understood complication of catheter ablation of atrial fibrillation. The purpose of this study was to (1) develop a canine model of esophageal injury and left atrial-esophageal fistula after applications of forward-firing high-intensity focused ultrasound (HIFU) and side-firing unfocused ultrasound (SFU); (2) examine the relationship to esophageal temperature (Eso-temp); and (3) study the evolution of injury/healing. METHODS AND RESULTS: Twenty dogs were studied. After transeptal puncture, HIFU catheter (ProRhythm Inc; 13 dogs) was positioned close to the esophagus, either outside (n=6) or inside (n=7) the inferior pulmonary vein (PV). In 7 other dogs, an SFU catheter was placed deep inside the PV, close to the esophagus. A balloon (20- to 25-mm diameter) with 7 thermocouples (2-mm separation) was positioned in the esophagus (Eso-balloon). Variable air filling of the Eso-balloon controlled the distance from the esophagus to the sonication source, pressing the esophagus against left atrium/PV. One to 9 (median, 5) HIFU (35 W) and 5 to 7 (median, 5) SFU (40 W) sonications were delivered for 40 seconds. Maximum luminal Eso-temp was closely related to HIFU Eso-balloon distance. For HIFU outside PV, Eso-temp >or=50 degrees C occurred only for HIFU Eso-balloon distance or=50 degrees C, with HIFU Eso-balloon distance up to 6.8 mm. Endoscopy identified esophageal ulcer immediately after ablation in 11 of 13 HIFU dogs and 7 of 7 SFU dogs, all with Eso-temp >or=50 degrees C. Endoscopy at 2 weeks showed ulcer healing in 5 of 11 chronic dogs and ulcer size progression with relaxation of the lower esophageal sphincter and esophagitis in 6 dogs. Two dogs developed left atrial-esophageal fistula and died at 2 weeks. CONCLUSIONS: This model produces esophageal ulcer when Eso-temp is >or=50 degrees C. Eso-temp is higher with HIFU/SFU applications closer to the esophagus and with HIFU/SFU applications inside the PV. Ulcer progression and left atrial-esophageal fistula were associated with reflux esophagitis.