Survey of medications for myelomeningocele patients over their lifetime in Japan.

BACKGROUND: This study aimed to investigate medication prescriptions for patients with myelomeningocele (MMC) across different age groups, particularly in adulthood and after middle age. METHODS: The Japan Medical Data Center (JMDC) database, based on medical claims data, was utilized for this analysis. Patients were divided into 10-year age groups, and prescriptions for analgesics, anticonvulsants, psychotropic drugs, lifestyle disease-related drugs, drugs for urinary incontinence, and laxatives were examined. To compare the differences in the utilization of medications unrelated to lifestyle-related diseases across different age groups, the data was categorized into three age groups: 19 or under, 20-39, and 40 or older. RESULTS: Among the 556 MMC patients, the percentage of those regularly prescribed analgesics increased from 2.8% in patients ≤ 19 to 31.7% in patients 40 or older (p < 0.01). Psychotropic medication use also increased with age, rising significantly from 6.3% in patients ≤ 19 to 34.6% in patients 40 or older (p < 0.01). Patients with MMC showed an increasing trend in prescriptions for lifestyle-related disease medications compared to the normal control group. Notably, the percentage of patients in their 30 s taking hypertension medication was 4.9%, significantly higher than the 0.86% in the control group (p = 0.029). In their 40 s, 22.9% of MMC patients were prescribed hyperlipidemia medication, significantly higher than the 3.9% in the control group (p < 0.01). CONCLUSION: Comprehensive multidisciplinary support and follow-up are crucial to enhance the quality of life for MMC patients, with particular attention to pain management, psychological care, and treatment of lifestyle-related diseases.

In-hospital grouping rehabilitation of hybrid closed-wedge high tibial osteotomy results in shorter hospital stays and better clinical outcomes.

[Purpose] This study aimed to examine the impact of physiotherapy on various patients who underwent hybrid closed-wedge high tibial osteotomy. [Participants and Methods] Eighty-four patients were divided into three groups: non-weight-bearing, enhanced recovery after surgery, and grouping exercise. The number of hospital days, distinctions in the duration of cane-walking independence, and postoperative complications were compared among the three groups. Furthermore, the Japanese Orthopaedic Association score and physical function were assessed preoperatively and at 3 and 12 months postoperatively. [Results] There were no considerable differences in postoperative complications among the three groups, and the mean hospital stay was the shortest for the grouping exercise group. At 3 months postoperatively, the grouping exercise group reported less walking pain than the enhanced recovery after surgery group. At 3 and 12 months postoperatively, the grouping exercise group showed greater mean knee extensor strength and a higher mean Japanese Orthopaedic Association score than the non-weight-bearing group. [Conclusion] Grouping exercise therapy, in addition to enhanced recovery after surgery protocol, results in shorter hospital stays, no difference in postoperative complications, and good clinical outcomes.

Association of clinical symptoms and metabolic syndrome-related factors in patients undergoing high tibial osteotomy.

[Purpose] This study aimed to investigate the relationship between clinical outcomes after high tibial osteotomy and metabolic syndrome-related factors, such as hypertension, dyslipidemia, diabetes mellitus, and obesity. [Participants and Methods] A total of 73 patients (73 knees) who underwent high tibial osteotomy for knee osteoarthritis between 2018 and 2020 were included. We investigated the correlation between metabolic syndrome-related factors and clinical symptom assessment (Japanese Orthopedic Association Score) and examined knee function and lower alignment. [Results] At three months postoperatively, the Japanese Orthopedic Association score showed no main and synergistic effects on metabolic syndrome-related factors, and the preoperative Japanese Orthopedic Association score only showed a main effect on metabolic syndrome-related factors. At 12 months postoperatively, the Japanese Orthopedic Association score showed main and synergistic effects on diabetes mellitus, obesity, hypertension and dislipidemia. [Conclusion] Metabolic syndrome-related factors are associated with poorer clinical outcomes after high tibial osteotomy.

Comparison of intraoperative neurophysiological monitoring methods for lumbosacral lipoma surgery in infants.

BACKGROUND: Stable intraoperative neurophysiology monitoring of infants, especially very young infants, is challenging. In this study, motor evoked potentials (MEPs), the bulbocavernosus reflex (BCR), and somatosensory evoked potentials (SEPs) were simultaneously monitored in infants with lumbosacral lipomas, and these methods were compared retrospectively. METHODS: A total of 21 surgeries performed for lumbosacral lipoma in patients less than 1 year old were studied. The mean age at surgery was 133.8 days (range 21-287 days; ≤ 120 days: 9 cases, > 120 days: 12 cases). Transcranial MEPs were measured in the anal sphincter and gastrocnemius, and tibialis anterior and other muscles were added as needed. The BCR was measured by the electromyogram of the anal sphincter muscle with stimulation of the pubic region, and SEPs were measured from the waveforms of stimulation of the posterior tibial nerves. RESULTS: For the BCR, stable potentials could be recorded for all 9 cases at ≤ 120 days of age. In contrast, for MEPs, stable potentials could be recorded in only 4 of 9 cases (p < 0.05). For all patients > 120 days of age, MEPs and the BCR were measurable. SEPs were undetectable in some patients regardless of age. CONCLUSION: The BCR could be more consistently measured than MEPs in infant patients with lumbosacral lipoma at ≤ 120 days of age.

A case of acute hydrocephalus due to a giant prolactinoma rescued by transventricular neuroendoscopic tumorectomy.

Background: A giant prolactinoma extending to the suprasellar area and causing hydrocephalus may be life-threatening and should be treated promptly. A case of a giant prolactinoma with acute hydrocephalus that underwent transventricular neuroendoscopic tumor resection followed by cabergoline administration is presented. Case Description: A 21-year-old man had a headache lasting for about a month. He gradually developed nausea and disturbance of consciousness. Magnetic resonance imaging showed a contrast-enhanced lesion that extended from the intrasellar space to the suprasellar space and into the third ventricle. The tumor obstructed the foramen of Monro and caused hydrocephalus. A blood test showed marked elevation of prolactin (16,790 ng/mL). The tumor was diagnosed as a prolactinoma. The tumor in the third ventricle had formed a cyst, and the cyst wall blocked the right foramen of Monro. The cystic component of the tumor was resected using an Olympus VEF-V flexible neuroendoscope. The histological diagnosis was pituitary adenoma. The hydrocephalus improved rapidly and his consciousness became clear. After the operation, he was started on cabergoline. The tumor size subsequently decreased. Conclusion: Prompt partial resection of the giant prolactinoma by transventricular neuroendoscopy resulted in early improvement of hydrocephalus with less invasiveness, allowing subsequent treatment with cabergoline.

The effect of shunt removal on the quality of life in patients with congenital hydrocephalus.

BACKGROUND: Although there have been reports investigating the quality of life of patients who underwent ventriculoperitoneal shunting or endoscopic third ventriculostomy (ETV) for congenital hydrocephalus, there have been no studies of the quality of life of patients after ventriculoperitoneal shunt (VPS) removal. In the present study, a survey of pediatric and congenital hydrocephalus patients was conducted to compare the quality of life of patients with a remaining VPS with that of patients who had the shunt removed. METHODS: Between February 2020 and November 2021, an outpatient survey was administered to patients 8 years of age and older who had undergone VPS due to a diagnosis of congenital hydrocephalus. The Hydrocephalus Outcome Questionnaire (HOQ) was used to assess the quality of life for this study. The HOQ scores (overall health score, physical health score, cognitive health score, and social-emotional health score) were compared among three groups: a VPS-remaining group, VPS-removed with endoscopic third ventriculostomy (ETV) group, and VPS-removed without ETV group. RESULTS: The total number of patients who underwent VPS for hydrocephalus was 71, with 47 in the VPS-remaining group, 14 in the shunt-removed with ETV group, and 10 in the shunt-removed without ETV group. The HOQ overall health score was 0.68 for the VPS-remaining group, 0.74 for the shunt-removed with ETV group, and 0.74 for the shunt-removed without ETV. There were no significant differences between the VPS-remaining group and the VPS-removed with or without ETV groups (p = 0.3255, 0.4178, respectively). CONCLUSION: There was no significant difference in the quality of life between patients with a remaining VPS and those who had their VPS removed with or without ETV.

A ventricular catheter that migrated into the fourth ventricle successfully removed using a neuroendoscope.

BACKGROUND: In order to remove a foreign body in the ventricle, such as a ventricular drainage catheter, craniotomy and corticotomy are required to access the ventricle. A case in which a catheter in the 4th ventricle was safely removed with a flexible neuroendoscope is reported. CASE DESCRIPTION: A 47-year-old man underwent coil embolization and ventricular drainage for subarachnoid hemorrhage. 10 days after the operation, he tore off the ventricular drainage catheter and the catheter remained intracranially. The tip of the catheter was in the 4th ventricle and the operation to remove remaining catheter with a neuroendoscope was performed. Using a neuroendoscope, we could remove the catheter safely and did not detect the complications. CONCLUSION: To date, there have been no reports of cases in which a drainage catheter in the ventricle was removed using a flexible endoscope. This case suggests that a flexible endoscope is useful for removing a foreign body from the ventricle less invasively.

5-ALA fluorescence-guided resection of pediatric low-grade glioma using the ORBEYE 3D digital exoscope: a technical report.

OBJECTIVE: A case of low-grade glioma in which 5-aminolevulinic acid (5-ALA) fluorescence was visualized by a digital exoscope is presented. CASE PRESENTATION: A 14-year-old girl with recurrent paroxysmal episodes of a strange smell and nausea underwent magnetic resonance imaging (MRI) for further investigation. The MRI showed a tumor with an enhanced nodule in the right temporal lobe. The patient received 5-ALA preoperatively, and intraoperative observation using a 4 K-3-dimension digital exoscope (Olympus ORBEYE) showed that the tumor was fluorescent, which was useful in determining the extent of tumor removal. Postoperative MRI showed that the tumor was completely removed. The histopathological diagnosis was pleomorphic xanthoastrocytoma. She was discharged without any complications. CONCLUSIONS: 5-ALA-fluorescence-guided resection of low-grade glioma using the ORBEYE was useful for determining the extent of removal.

Current status and challenges of neurosurgical procedures for patients with myelomeningocele in real-world Japan.

BACKGROUND: Little is known about the real-world status of neurosurgical treatment of myelomeningocele patients. OBJECTIVE: To investigate the real-world status of neurosurgical treatment of myelomeningocele patients, medical claims data provided by the Japan Medical Data Center (JMDC) were analyzed. METHODS: The health claims data of 556 patients with myelomeningoceles from January 2005 to March 2020 were examined. The number of neurosurgical procedures, including myelomeningocele repair, tethered cord release, cerebrospinal fluid (CSF) shunt, CSF drainage, and endoscopic third ventriculostomy (ETV), was determined. RESULTS: A total of 313 neurosurgical procedures were performed for 135 patients in 74 institutions during the study period. The shunt survival rate was most affected by shunts that were revised when the patient was less than 1 year old, which had a significantly lower survival rate than all of the initial shunts performed when the patient was less than on1 year old; the 1-year shunt survival rate was 35 vs 64% (P = 0.0102). The survival rate was significantly lower in patients younger than 1 year who had CSF drainage before shunting compared to those younger than 1 year who did not have CSF drainage before shunting; the 1-year shunt survival rate was 27 vs 59% (P = 0.0196), and 81% of patients remained free of tethered cord release 10 years later. CONCLUSIONS: In this study, a revised shunt of less than 1 year of age and CSF drainage before shunting were the factors that lowered the shunt survival rate in the real world for CSF shunts for hydrocephalus associated with myelomeningocele.

Primary central nervous system lymphoma of the tectal plate in adult.

Background: Primary central nervous system lymphoma (PCNSL) originating in the brainstem is uncommon. In particular, PCNSL confined to the tectal plate in adults has never been reported in the past. The case of a 53-year-old man who was diagnosed with PCNSL in the tectal plate is reported. Case Description: The patient was referred to our hospital with a 1-month history of disorientation and magnetic resonance imaging showed hydrocephalus with an enhancing lesion in the tectum. Preoperative blood tests showed a high serum soluble interleukin-2 receptor level of 624 U/ml. Through a single burr hole, endoscopic third ventriculostomy and biopsy of the lesion were simultaneously performed with a flexible endoscope. The histological examination confirmed diffuse large B-cell lymphoma. The patient underwent chemotherapy and radiotherapy. Conclusion: Malignant lymphoma of the tectum may occur in adults. By measuring the soluble interleukin-2 level preoperatively, it was possible to include malignant lymphoma in the differential diagnosis. In addition, the use of a neuroendoscope permits biopsy and hydrocephalus treatment to be performed simultaneously.

Treatment of refractory giant suprasellar arachnoid cyst by endoscopic expansion of a fenestrated stoma.

Background: Although endoscopic ventriculo-cysto-cisternostomy is considered to be effective for suprasellar arachnoid cysts, we encountered a giant suprasellar arachnoid cyst that recurred despite surgery using this technique. Case Description: The patient was a 9-month-old boy. Magnetic resonance imaging revealed a huge suprasellar arachnoid cyst extending from the suprasellar region to the anterior skull base and both middle cranial fossa. First, an endoscopic procedure was performed to open the cyst wall between the right ventricle and the cyst and between the cyst and the prepontine cistern. Although the cyst initially shrank, it recurred over the next 2 months, and hence, we performed another endoscopic surgery. At the second surgery, both the previously opened stomas were found to be occluded. To reopen the cyst wall between the ventricle and the cyst, multiple holes were made with monopolar electrodes, and forceps were used to connect the holes by grasping and twisting the cyst wall so that the stoma was much larger than at the previous surgery. Postoperatively, the cyst shrank and the patient's head circumference stopped expanding. Conclusion: Following the treatment of large cysts, the stoma might become narrower as the cyst shrinks, resulting in obstruction. Using the technique reported here might prevent occlusion of large arachnoid cysts.

Familial tendency in patients with lipoma of the filum terminale.

PURPOSE: Lipoma of the filum terminale (FL) is an abnormality in which fat is deposited in the filum terminale. This lipoma is often detected by skin abnormalities in the lumbosacral area such as a sacrococcygeal dimple. Some cases may develop tethered cord and become symptomatic. However, the genetic basis of FL is still unclear. METHODS: This study aimed to determine whether there was a family history of FL or other forms of spina bifida among 54 families of 56 patients with FL and to examine whether there is a familial predisposition in FL. In addition, sex, age at diagnosis, presence of symptoms, presence of sacrococcygeal dimple, and the level of conus medullaris between familial and spontaneous cases were evaluated. RESULTS: Of the 54 families of FL patients, there were 48 siblings. Among the 48 siblings, 2 had "occult" FL. The frequency of FL among siblings was estimated to be 4.2% (2/48), which was significantly higher than the sum of previously reported cases of spontaneous FL (0.91%; p = 0.017). However, there was no significant difference in sex, age at diagnosis, presence of symptoms, presence of sacrococcygeal dimple, diameter of filum terminale, or level of conus medullaris between familial and spontaneous cases. CONCLUSION: To our knowledge, this is the first report on familial FL and examination of the frequency of FL among siblings. The high probability of FL among siblings of FL patients suggests that genetic factors may play a role in FL development.

Stent-Assisted Coil Embolization of Ruptured Blood Blister-Like Aneurysm of the Basilar Artery: A Case Report and Literature Review.

Objective: Blood blister-like aneurysms (BBA) often develop on the anterior wall of the internal carotid artery, and few cases have been reported at other sites. We report a case of stent-assisted coil embolization in the acute phase for a ruptured BBA of the basilar artery. Case Presentation: A 53-year-old woman underwent emergency stent-assisted coil embolization for subarachnoid hemorrhage due to a ruptured BBA in the main trunk of the basilar artery. Seven months after the operation, cerebral angiography confirmed no recurrence and a good clinical course. Conclusion: Stent-assisted coil embolization for BBA may be one treatment option.

Resection of a recurrent medulloblastoma in the anterior middle part of the aqueduct with a flexible endoscope: a case report.

Resection or biopsy of intraventricular brain tumors using an endoscope has become common, but the limitations of these procedures are not clear. Manipulation to access a tumor that arises from the blind angle of the rigid endoscope, such as the anterior part of the aqueduct, is limited. We report here that we successfully resected a recurrent medulloblastoma in the anterior part of the aqueduct using only a flexible endoscope. This method appears to be suitable for poorly vascularized and suctionable tumors that arise in the blind angle of a rigid endoscope.

GSK3ß regulates gluconeogenic gene expression through HNF4α and FOXO1.

Hepatic gluconeogenesis is important for the maintenance of blood glucose homeostasis under fasting condition. Hepatocyte nuclear factor 4α (HNF4α) and FOXO1 transcription factors have implicated in this process through transcriptional regulation of glucose-6-phosphatase (G6Pase) and phosphoenolpyruvate carboxykinase (PEPCK), which are rate-limiting enzymes in gluconeogenesis. In this study, we demonstrate that glycogen synthase kinase 3ß (GSK3ß) regulates the expression of gluconeogenic genes through HNF4α and FOXO1. Silencing of GSK3ß leads to reduction in the expression of gluconeogenic genes, including G6Pase, PEPCK, and peroxisome proliferator-activated receptor γ coactivator-1α. We show that GSK3ß directly binds to both HNF4α and FOXO1. Inhibition of GSK3 by SB-216763 abolishes HNF4α-mediated activation of G6Pase promoter. We also found that overexpression of GSK3ß potentiates G6Pase promoter activation by FOXO1 in a manner dependent on its kinase activity. Treatment of SB-216763 diminishes FOXO1-mediated activation of G6Pase promoter. Taken together, these results reveal a previously unrecognized mechanism for the regulation of gluconeogenic gene expression.

Arginine methylation of BCL-2 antagonist of cell death (BAD) counteracts its phosphorylation and inactivation by Akt.

Protein arginine methylation is a common posttranslational modification catalyzed by a family of the protein arginine methyltransferases (PRMTs). We have previously reported that PRMT1 methylates Forkhead box O transcription factors at two arginine residues within an Akt consensus phosphorylation motif (RxRxxS/T), and that this methylation blocks Akt-mediated phosphorylation of the transcription factors. These findings led us to hypothesize that the functional crosstalk between arginine methylation and phosphorylation could be extended to other Akt target proteins as well as Forkhead box O proteins. Here we identify BCL-2 antagonist of cell death (BAD) as an additional substrate for PRMT1 among several Akt target proteins. We show that PRMT1 specifically binds and methylates BAD at Arg-94 and Arg-96, both of which comprise the Akt consensus phosphorylation motif. Consistent with the hypothesis, PRMT1-mediated methylation of these two arginine residues inhibits Akt-mediated phosphorylation of BAD at Ser-99 in vitro and in vivo. We also demonstrate that the complex formation of BAD with 14-3-3 proteins, which occurs subsequent to Akt-mediated phosphorylation, is negatively regulated by PRMT1. Furthermore, PRMT1 knockdown prevents mitochondrial localization of BAD and its binding to the antiapoptotic BCL-X(L) protein. BAD overexpression causes an increase in apoptosis with concomitant activation of caspase-3, whereas PRMT1 knockdown significantly suppresses these apoptotic processes. Taken together, our results add a new dimension to the complexity of posttranslational BAD regulation and provide evidence that arginine methylation within an Akt consensus phosphorylation motif functions as an inhibitory modification against Akt-dependent survival signaling.

Successful repair of thoracoabdominal aortic aneurysm secondary to chronic dissection by hybrid therapy comprising surgical visceral reconstruction and dual Inoue stent graft implantation.

Crawford type II thoracoabdominal aortic aneurysm (TAAA) secondary to chronic dissection was successfully treated with hybrid therapy comprising surgical visceral reconstruction and dual Inoue stent graft implantation. The proximal single-branched Inoue stent graft effectively sealed the entry located near the left subclavian artery and simultaneously excluded the TAAA, while the distal tubular Inoue stent graft sealed the reentry; thus, the artery of Adamkiewicz was left unexcluded and intact between the two Inoue stent grafts. The visceral arteries were reconstructed using a quadrifurcated retrograde bypass with posterior aortic tunneling and end-to-end distal anastomosis to all four vessels to achieve a curved and smooth configuration.

Multislice computed tomography accurately quantifies left atrial size and function after the MAZE procedure.

BACKGROUND: Although the MAZE procedure allows for the recovery of sinus rhythm and left atrial (LA) mechanical function in the great majority of patients with chronic atrial fibrillation (AF), the effects of MAZE on the precise LA geometry and wall motion remain to be elucidated. We hypothesized that LA size and mechanical function in patients with chronic AF and mitral valvular disease are well restored after MAZE. METHODS AND RESULTS: We studied 14 patients (MAZE group: mean+/-SD age, 63.9+/-8.6 years; 8 men and 6 women) who underwent MAZE for chronic AF and mitral valve surgery and 10 patients with sinus rhythm (coronary artery bypass graft [CABG] group: age, 70.0+/-7.9 years; 5 men and 5 women) who underwent CABG at Takeda Hospital between February 2002 and September 2005. MAZE was conducted by the endocardial application of radiofrequency ablation with a temperature-controlled multipolar radiofrequency catheter. LA volume and booster function were quantitatively evaluated by multislice computed tomography at 17.9+/-10.0 months (MAZE group) and 15.3+/-13.6 months (CABG group) postoperatively. All patients with MAZE were free of AF and other atrial arrhythmias during the follow-up period. In the CABG group, LA maximal and minimal volumes and ejection fraction were 109+/-12 mL, 82+/-11 mL, and 26+/-10%, respectively. In the MAZE group, LA maximal volume was 139+/-17 mL (P=0.187 versus CABG), and LA minimal volume was 121+/-16 mL (P=0.082 versus CABG), with an ejection fraction of 15+/-7% (P=0.004 versus CABG). In both groups, all parts of the LA wall contracted toward the geometric center of the LA. The extent of wall motion was significantly worse in the MAZE group compared with the CABG group. In both groups, LA booster function was inversely correlated with LA maximal volume. CONCLUSIONS: MAZE with radiofrequency ablation is safe and effective for the restoration of sinus rhythm in patients with chronic AF and mitral valve disease. However, chronic AF associated with mitral valve disease deteriorates LA mechanical function diffusely throughout the LA wall. Further studies with the use of multislice computed tomography are needed to sequentially evaluate LA function after MAZE in patients with and without mitral valve surgery.

Feasibility of the Inoue single-branched stent-graft implantation for thoracic aortic aneurysm or dissection involving the left subclavian artery: short- to medium-term results in 17 patients.

OBJECTIVE: This study assessed the short- to medium-term clinical results of the Inoue single-branched stent graft for repair of thoracic aortic aneurysms or dissections involving the left subclavian artery. METHODS: A retrospective review of experiences at two institutions was performed. We analyzed the data of consecutive 17 patients with thoracic aortic aneurysms or dissections who underwent endovascular repairs with the Inoue single-branched stent graft between July 1999 and April 2004. Complete baseline and follow-up data were available on all patients. The mean age was 71 +/- 9 years, and 13 of the patients (76%) were men. Eight patients (47%) were considered unfit for open surgery because of advanced age or the presence of comorbid diseases. RESULTS: The stent grafts were successfully delivered and deployed in all 17 patients. Periprocedural major complications, defined as those that caused any persistent disorder, occurred in one patient who developed spinal ischemia. A postoperative computed tomographic scan revealed three attachment site endoleaks; two endoleaks were from the proximal attachment sites and one endoleak was from the distal attachment site. The mean follow-up period was 26 months (range, 7 to 65 months). Two deaths occurred in the follow-up period from cerebral bleeding and pneumonia, both considered unrelated to the stent grafting. Two patients with attachment site endoleaks needed secondary stent-grafting; one patient required the implantation of a straight stent-graft in the distal attachment site and the other, the implantation of a double-branched stent-graft. Another patient with attachment site endoleak was considered very high-risk for open surgery or secondary stent grafting and did not undergo secondary intervention. The aneurysmal sac size of the patient has been stable for 28 months. The branched section of the stent graft was patent in all patients in the follow-up period. CONCLUSION: The results demonstrate the feasibility of the Inoue single-branched stent graft for thoracic aortic aneurysms or dissections involving the left subclavian artery.