Midterm outcomes of side branch embolization and endovascular abdominal aortic aneurysm repair.

OBJECTIVE: To analyze the effects of total side branch embolization at endovascular aneurysm repair (EVAR) for abdominal aortic aneurysms on the incidences of persistent type 2 endoleak (pT2EL), changes in sac diameter, and reintervention. METHODS: Between 2013 and 2021, all patients who underwent primary EVAR with a few exceptions were included. Side branch embolization was considered during EVAR for inferior mesenteric artery (IMA) or IMA plus lumbar artery (LA) when feasible for contrast agent use. Outcomes measured were pT2EL, sac diameters, reintervention, ruptures, and aneurysm-related mortality. Radiation exposure and safety outcomes were also reported. RESULTS: Among 732 patients who underwent EVAR, 616 (84.2%) were included. Of the 616 patients, 223 (36.2%) did not undergo side branch embolization (NO-E), whereas 228 (37.0%) underwent IMA only (IMA-E) and 165 (26.8%) underwent IMA+LA including median sacral artery (IMA+LA-E). The technical success rate of IMA and LA embolization was 97.0% and 74.7%, respectively. Crude incidences of pT2EL were significantly different from 6 months through 3 years (NO-E, 27.8%; IMA-E, 31.7%; IMA+LA-E, 9.4% at 3 years; P = .007). In the multivariate analysis adjusted for background differences, the incidences of pT2EL were significantly higher in the NO-E (odds ratio [OR], 3.21; 95% confidence intervals [CIs], 1.08-9.57; P = .004) and IMA-E (OR, 4.86; 95% CIs, 1.68-14.11; P = .004) compared with the IMA+LA-E group. Similarly, any reintervention until 3 years was significantly frequent in the NO-E (OR, 5.26; 95% CIs, 1.76-15.70; P = .003) and IMA-E group (OR, 4.19; 95% CIs, 1.38-12.67; P = .01). Surgical conversion and secondary rupture were seen only in 1 patient without any aneurysm-related mortality. Percent sac shrinkage from the baseline was significantly promoted in the IMA+LA group (NO-E, 12.1% ± 16.6%; IMA-E, 11.4% ± 16.7%; IMA+LA-E, 18.0% ± 18.8%; P = .047). Fluoroscopy time was significantly longer in the IMA+LA-E group (NO-E, 60.2 ± 47.4 minutes; IMA-E, 59.3 ± 39.5 minutes; IMA+LA-E, 75.5 ± 42.8 minutes; P < .0001), and so do the dose-area product (NO-E, 424.6 ± 333.4 Gy cm2; IMA-E, 477.7 ± 342.4 Gy cm2; IMA+LA-E, 631.8 ± 449.1 Gy cm2; P < .0001). No embolization-related complications or radiation-related adverse events were recorded. CONCLUSIONS: Pre-emptive embolization of IMA, LAs, and median sacral artery at the time of EVAR reduced the incidences of pT2EL and any reintervention and promoted sac shrinkage during the follow-up period of 3 years.

Amyotrophic Lateral Sclerosis with a Priority Request for a Postmortem Kidney Donation to a Relative.

The patient was 57 years old when he was diagnosed with amyotrophic lateral sclerosis (ALS) at 1 year after developing bulbar symptoms. At 58 years old, he stated that he was considering donating his kidney to his son suffering from diabetic nephropathy. We confirmed the patient's intentions through repeated interviews before his death at 61 years old. Nephrectomy was performed 30 min after his cardiac death. Organ donation spontaneously proposed by an ALS patient should be considered in order to meet the requests of patients who want their families and other patients to live longer, thereby imparting a beneficial legacy through their deaths.

Open Versus Zone 0/1 Endovascular Aortic Repair for Arch Aneurysm: A Propensity Score-Matched Study from the National Clinical Database in Japan.

BACKGROUND: Although open surgical repair (OSR) is the gold standard for treating arch aneurysms, thoracic endovascular aortic repair (TEVAR) may be a less invasive alternative. However, it remains unclear which of the 2 methods yields better outcomes. In this study, we compared the perioperative outcomes of both procedures for arch aneurysms using a nationwide surgical database. METHODS: Data of patients who underwent elective aortic repair for true arch aneurysms were extracted from the National Clinical Database of Japan. Patients who underwent OSR and Zone 0/1 TEVAR were matched in a 1:1 ratio using propensity scores and their mortality and morbidity rates were compared. RESULTS: A total of 2,815 and 1,125 patients underwent OSR and Zone 0/1 TEVAR, respectively. After propensity score matching, 1,058 patients were included in both groups. Compared with OSR, Zone 0/1 TEVAR was associated with a significantly higher incidence of stroke (5.8 vs. 10.0%, P < 0.001) and paraplegia/paraparesis (1.6 vs. 4.4%, P < 0.001). However, there were no significant differences in the 30-day and operative mortality rates between the 2 groups (2.2 vs. 2.7% and 4.5 vs. 5.4%, respectively). In the Zone 0/1 TEVAR group, postoperative computed tomography was performed in 92.4% of patients, and types I and III endoleaks were identified in 6.4% and 1.1% of patients, respectively. CONCLUSIONS: Zone 0/1 TEVAR has higher incidences of stroke and paraplegia/paraparesis than OSR, with a risk of postoperative endoleaks. Resolving these problems is the key for expanding the application of Zone 0/1 TEVAR and in the meantime OSR remains the gold standard for surgically fit patients.

Outcomes of Clampless Real Chimney Technique with Dacron Graft and Sleeve Banding of the Ascending Aorta for Total Debranching Endovascular Arch Repair.

BACKGROUND: Hybrid aortic arch repair with debranching of the supra-aortic vessels carries a high risk of cerebral infarction and fatal complications associated with side clamping of the ascending aorta. A previous study had reported the "real chimney" technique, a novel method for clamp-free total debranching thoracic endovascular aortic repair with the ascending aortic sleeve banding with dacron. In this study, we aim to build upon this foundation by presenting our experience with the early and midterm outcomes of this technique. METHODS: We retrospectively reviewed the medical records of 61 consecutive older adult patients with aortic arch pathologies and a high risk of open repair who underwent total debranching thoracic endovascular aortic repair using the real chimney technique at our institution between January 2014 and June 2022. This technique was used to anastomose the ascending aorta with the main trunk of the triple-branched prosthetic graft. RESULTS: The mean patient age was 75.1 years, and 54% of patients were transferred from medical facilities with cardiothoracic surgery departments. The comorbidities included chronic obstructive pulmonary disease (49.2%), chronic kidney disease (63.9%), coronary artery disease (27.9%), and history of stroke (31.1%). No 30-day mortality was observed. Complications included postoperative renal failure requiring permanent hemodialysis (4; 6.6%), stroke (modified Rankin scale score: ≥2 in 5; 8.2%), permanent paraplegia (1; 1.6%), and permanent paraparesis (4; 6.6%). The median follow-up period was 40.5±28.5 months. The postoperative survival rates at 5 years were 66.8%. No patients experienced complications associated with the aorta or anastomosis site; only 1 patient required an additional procedure for stenosis of the anastomosis site (midterm). An aneurysmal diameter reduction of ≥3 mm was observed in 37 cases (60.7%), and the mean aneurysmal diameter reduction was 5.3 mm. CONCLUSIONS: The postoperative outcome of total debranching thoracic endovascular aortic arch repair is not only acceptable but also promising, particularly in patients at high risk of open repair. CLINICAL IMPACT: Our novel real chimney technique for total debranching thoracic endovascular aortic repair of aortic arch pathologies in patients who were at a high-risk for open repair resulted in no 30-day mortality and no complications related to the aorta or the real chimney anastomosis site.The real chimney technique is effective for managing aortic arch pathologies and enables less invasive total debranching thoracic endovascular aortic arch repair without the need for extracorporeal circulation and clamping of the ascending aorta.

Efficacy confirmation study of aceneuramic acid administration for GNE myopathy in Japan.

BACKGROUND: A rare muscle disease, GNE myopathy is caused by mutations in the GNE gene involved in sialic acid biosynthesis. Our recent phase II/III study has indicated that oral administration of aceneuramic acid to patients slows disease progression. METHODS: We conducted a phase III, randomized, placebo-controlled, double-blind, parallel-group, multicenter study. Participants were assigned to receive an extended-release formulation of aceneuramic acid (SA-ER) or placebo. Changes in muscle strength and function over 48 weeks were compared between treatment groups using change in the upper extremity composite (UEC) score from baseline to Week 48 as the primary endpoint and the investigator-assessed efficacy rate as the key secondary endpoint. For safety, adverse events, vital signs, body weight, electrocardiogram, and clinical laboratory results were monitored. RESULTS: A total of 14 patients were enrolled and given SA-ER (n = 10) or placebo (n = 4) tablets orally. Decrease in least square mean (LSM) change in UEC score at Week 48 with SA-ER (- 0.115 kg) was numerically smaller as compared with placebo (- 2.625 kg), with LSM difference (95% confidence interval) of 2.510 (- 1.720 to 6.740) kg. In addition, efficacy was higher with SA-ER as compared with placebo. No clinically significant adverse events or other safety concerns were observed. CONCLUSIONS: The present study reproducibly showed a trend towards slowing of loss of muscle strength and function with orally administered SA-ER, indicating supplementation with sialic acid might be a promising replacement therapy for GNE myopathy. TRIAL REGISTRATION NUMBER: ClinicalTrials.gov (NCT04671472).

Cognitive dysfunction in drug-naïve late-onset temporal lobe epilepsy.

OBJECTIVES: To evaluate cognitive functions including memory in middle-aged and elderly patients with antiseizure drug-naïve late-onset temporal lobe epilepsy (TLE). METHODS: We performed assessments with the Wechsler Adult Intelligence Scale-III (WAIS-III) and Wechsler Memory Scale-Revised (WMS-R) in 26 antiseizure drug-naïve patients with late-onset TLE, in comparison to 30 healthy subjects. We investigated the relationships between these cognitive function scores and clinical characteristics, seizure frequency, and frequency of interictal epileptic discharges (IEDs). RESULTS: Patients with epilepsy had a significantly lower score than healthy controls in the verbal intelligence quotient (IQ), the performance IQ, and full-scale IQ in intelligence testing. Patients showed significantly decrease in the verbal memory scores, visual memory scores, general memory scores, and delayed recall scores compared with those in the control subjects. Delayed recall scores were significantly negatively correlated with recent seizure frequency and the total IEDs count per minute, but not with age of onset or duration of illness. SIGNIFICANCE: Patients with antiseizure drug-naïve late-onset TLE displayed cognitive deficits including the domains of memory by using standard clinical neuropsychological test. Patients with late-onset epilepsy need to be considered for cognitive dysfunction at the time of diagnosis of TLE because they may have their daily life and work affected not only by epileptic seizures but also by cognitive deficits. Appearance of seizures and EEG abnormalities may affect the memory function in patients with late-onset TLE.

Phase II/III Study of Aceneuramic Acid Administration for GNE Myopathy in Japan.

BACKGROUND: GNE myopathy is an ultra-rare muscle disease characterized by a reduction in the synthesis of sialic acid derived from pathogenic variants in the GNE gene. No treatment has been established so far. OBJECTIVE: We evaluated the safety and efficacy of oral supplementation of aceneuramic acid in patients with GNE myopathy. METHODS: This multicenter, placebo-controlled, double-blind study comprised genetically confirmed GNE myopathy patients in Japan who were randomly assigned into treatment groups of sialic acid-extended release (SA-ER) tablets (6 g/day for 48 weeks) or placebo groups (4:1). The primary endpoint of effectiveness was set as the change in total upper limb muscle strength (upper extremity composite [UEC] score) from the start of administration to the final evaluation time point. RESULTS: Among the 20 enrolled patients (SA-ER group, 16; placebo group, 4), 19 completed this 48-week study. The mean value of change in UEC score (95% confidence interval [CI]) at 48 weeks was -0.1 kg (-2.1 to 2.0) in the SA-ER group and -5.1 kg (-10.4 to 0.3) in the placebo group. The least squares mean difference (95% CI) between the groups in the covariance analysis was 4.8 kg (-0.3 to 9.9; P = 0.0635). The change in UEC score at 48 weeks was significantly higher in the SA-ER group compared with the placebo group (P = 0.0013) in the generalized estimating equation test repeated measurement analysis. In one patient in the SA-ER group, who was found to be pregnant 2 weeks after drug administration fetal death with tangled umbilical cord occurred at 13 weeks after the discontinuation of treatment. No other serious adverse effects were observed. CONCLUSIONS: The present study indicates that oral administration of SA-ER tablets is effective and safe in patients with GNE myopathy in Japan.

Modified adaption of iliac branch endografts in rare congenital anomaly of proximal origin of bilateral internal iliac arteries.

We report a case of 55 mm abdominal aortic aneurysm coinciding with a rare congenital anomaly of proximal origin of bilateral internal iliac arteries (IIAs). Because renal to iliac bifurcation lengths were bilaterally short (129 mm and 125 mm), a trunk-ipsilateral leg and an iliac leg were deployed before iliac branch component insertion into the iliac leg. With help of a pull-through wire, internal iliac component was delivered without migration of the main body. The left IIA was embolized, but the right IIA was successfully preserved with commercially available iliac branch endoprosthesis only from femoral approaches, and the patient fully recovered without any complication.

Psychoses after an antiepileptic drug administration: Frequency, timing, and duration.

OBJECTIVES: To clarify the pathophysiology of psychoses after the new administration of antiepileptic drugs (AED), we analyzed the annual incidence, timing of development, and duration of episodes. METHODS: Psychotic outcomes in the first 6-month period after an AED or non-AED administration in patients with focal epilepsy were exhaustively reviewed in eight Japanese neuropsychiatry institutions. In cases with psychotic episodes, the subtype of psychosis, timing of development, previous history of psychosis, and duration of the episode were evaluated. RESULTS: Between 1981 and 2015, 5018 new drugs (4402 AED and 616 non-AED) were administered to 2067 patients with focal epilepsy. In the first 6-month period, 105 psychotic episodes occurred (81 interictal psychosis [IIP] and 24 postictal psychosis). Furthermore, 55 cases were first episodes and 50 were recurrent episodes. The frequency of psychoses is significantly higher after AED administration (n = 102) compared with non-AED administration (n = 3). Psychosis occurred most frequently in the initial 1-month period after new-AED administration and tended to decrease with increasing time. The estimated annual incidence of all psychoses after a new AED administration was 3.5% (2.0% for first-episode psychosis and 1.8% for first-episode IIP). Duration of psychoses (mean, 38.5 weeks) was equivalent to overall IIP. Duration of IIP did not shorten with discontinuation of newly administered AED. SIGNIFICANCE: Patients with epilepsy exhibit psychosis more frequently after new AED administration than after non-AED administration. This study shows the pathophysiology of psychoses after AED administration with annual incidence, the timing of development, and the duration of PAP, which have rarely been reported.

Videofluoroscopic Dysphagia Scale as an Additional Indicator of Gastrostomy in Patients with Amyotrophic Lateral Sclerosis with Dysphagia.

Pseudobulbar palsy and bulbar palsy cause dysphagia in patients with amyotrophic lateral sclerosis (ALS). Dysphagia in patients with ALS not only increases the risk of aspiration and pneumonia but also leads to malnutrition and weight loss, which are poor prognostic factors. Gastrostomy is the preferred route of feeding and nutritional support in patients with dysphagia. However, there are no established standards to determine the ideal timing of gastrostomy for patients with ALS. Therefore, we used the videofluoroscopic dysphagia scale (VDS), which objectively quantifies swallowing function, in videofluoroscopic swallowing study (VFSS) to investigate whether this scale at diagnosis can be a useful predictor for the timing of gastrostomy. We retrospectively evaluated 22 patients with ALS who were diagnosed at our hospital. We assessed the VDS scores in all patients within 3 months of diagnosis. A decline in the ALS functional rating scale revised (ALSFRS-R) scores was used as an indicator of disease progression. As a result, we found that the VDS score of the pharyngeal phase and the total VDS score were significantly correlated with the ΔALSFRS-R scores. These scores were also associated with the existing indicators for the timing of gastrostomy, i.e., decreased body weight and percent-predicted forced vital capacity. We demonstrated the noninferiority of the VDS scores relative to the existing indicators. In addition, the VDS score of the pharyngeal phase was significantly correlated with the time from diagnosis to gastrostomy. The VDS score could estimate the timing of gastrostomy in patients with ALS with dysphagia at diagnosis.

Clampless In-Situ Immobilized Branching (CLIMB) to Reconstruct the Internal Iliac Artery.

BACKGROUND: Surgical reconstruction of the internal iliac artery (IIA) or its branches is sometimes demanding because of difficulty in distal clamping and suturing in the narrow pelvic space. Here we present a hybrid technique of ClampLess In-situ imMobilized Branching (CLIMB) to reconstruct IIA. METHODS: in the CLIMB technique, an 8 mm artificial graft is sutured onto the surface of the common iliac artery (CIA) without clamping. Following puncture of the CIA wall, stent grafts are bridged from IIA to the graft. Finally, the graft is sutured to the ipsilateral external iliac artery (EIA). Concomitant endovascular aneurysm repair or IIA branch embolization can also be performed. We applied this technique to the patients unsuited for other IIA reconstruction. RESULTS: eleven patients underwent the current technique. All but one patient underwent contralateral IIA interruption. Seven patients had a history of aorto-iliac repair before the index surgery. Iliac extender, internal iliac component, Viabahn VBX or Fluency covered stent were used for bridging the graft. Simultaneous IIA branch embolization was performed in 2 patients. Distal landing zones were IIA in 7 grafts, superior gluteal artery in 4 grafts and inferior gluteal artery (IGA) in 1 graft. Technical success was achieved in all cases. No patient complained of buttock claudication or other ischemic symptoms on the treatment side. During a mean follow-up period of 38 months, 11 out of 12 grafts were patent without any related endoleak. One IGA graft occluded at 56 months after surgery. CONCLUSIONS: the CLIMB technique is a viable alternative to preserve IIA with an acceptable mid-term durability.

Impact of COVID-19 pandemic on epilepsy care in Japan: A national-level multicenter retrospective cohort study.

OBJECTIVE: The impact of the coronavirus disease 2019 (COVID-19) pandemic on epilepsy care across Japan was investigated by conducting a multicenter retrospective cohort study. METHODS: This study included monthly data on the frequency of (1) visits by outpatients with epilepsy, (2) outpatient electroencephalography (EEG) studies, (3) telemedicine for epilepsy, (4) admissions for epilepsy, (5) EEG monitoring, and (6) epilepsy surgery in epilepsy centers and clinics across Japan between January 2019 and December 2020. We defined the primary outcome as epilepsy-center-specific monthly data divided by the 12-month average in 2019 for each facility. We determined whether the COVID-19 pandemic-related factors (such as year [2019 or 2020], COVID-19 cases in each prefecture in the previous month, and the state of emergency) were independently associated with these outcomes. RESULTS: In 2020, the frequency of outpatient EEG studies (-10.7%, p<0.001) and cases with telemedicine (+2,608%, p=0.031) were affected. The number of COVID-19 cases was an independent associated factor for epilepsy admission (-3.75*10-3 % per case, p<0.001) and EEG monitoring (-3.81*10-3 % per case, p = 0.004). Further, the state of emergency was an independent factor associated with outpatient with epilepsy (-11.9%, p<0.001), outpatient EEG (-32.3%, p<0.001), telemedicine for epilepsy (+12,915%, p<0.001), epilepsy admissions (-35.3%; p<0.001), EEG monitoring (-24.7%: p<0.001), and epilepsy surgery (-50.3%, p<0.001). SIGNIFICANCE: We demonstrated the significant impact that the COVID-19 pandemic had on epilepsy care. These results support those of previous studies and clarify the effect size of each pandemic-related factor on epilepsy care.

Long-term outcomes after surgery to prevent aspiration for patients with amyotrophic lateral sclerosis.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects motor neurons selectively. In particular, weakness in respiratory and swallowing muscles occasionally causes aspiration pneumonia and choking, which can be lethal. Surgery to prevent aspiration, which separates the trachea and esophagus, can reduce the associated risks. Central-part laryngectomy (CPL) is a relatively minimally invasive surgery to prevent aspiration. No studies have been conducted on the long-term outcomes of surgery to prevent aspiration in patients with ALS. This case series aimed to determine the long-term outcomes of surgery to prevent aspiration and the use of a continuous low-pressure aspirator in patients with ALS by evaluating the frequency of intratracheal sputum suctions performed per day, intra- and postoperative complications, oral intake data, and satisfaction of patients and their primary caregiver to predict improvement in patients' quality of life (QOL). METHODS: We report a case series of six patients with ALS who underwent CPL along with tracheostomy to prevent aspiration between January 2015 and November 2018. We evaluated their pre- and postoperative status and administered questionnaires at the time of last admission to the patients and their primary caregivers. RESULTS: The mean follow-up period after CPL was 33.5 months. Aerophagia was a common postoperative complication. The use of a continuous low-pressure aspirator resulted in reduced frequency of intratracheal sputum suctions. All cases avoided aspiration pneumonia. Oral intake was continued for 2-4 years after the tracheostomy and CPL. The satisfaction levels of the patient and primary caregiver were high. CONCLUSION: Our case series suggests that the use of a continuous low-pressure aspirator in patients undergoing CPL improves oral intake and reduces the frequency of intratracheal sputum suctions, which improves the QOL of patients with ALS and their families and caregivers. CPL and continuous low-pressure aspiration should be considered as a management option for ALS with significant bulbar and respiratory muscle weakness/dysfunction.

Comparative study of Japanese frozen elephant trunk device for open aortic arch repairs.

OBJECTIVE: We performed a multicenter prospective comparative study to determine the impact of a Japanese frozen elephant trunk device on total arch replacement compared with conventional repair without it. METHODS: Between 2016 and 2019, a total of 684 patients (frozen elephant trunk procedure; n = 369; conventional repair, n = 315) from 41 institutions were enrolled. The 2 procedures were selected according to each center's strategy. RESULTS: The frozen elephant trunk procedure was applied more for aortic dissection, whereas the conventional repairs were predominantly performed for aneurysms. In the former, only hypothermic circulatory arrest time was reduced among the intraoperative parameters. Although there were no differences in the 30-day and in-hospital mortality rates (0.8% and 1.6%, respectively, for the frozen elephant trunk procedure vs 0.3% and 0.6%, respectively, for conventional repair), the neurologic complication rates were significantly higher in stroke (5.7% vs 2.2%; P = .022) and paraplegia (1.6% vs 0%; P = .023). In the propensity score matching analyses using 11 variables, statistical significance disappeared in the differences for mortality and neurologic morbidity (stroke and paraplegia/paraparesis) rates of 194 patients of each group, although they were still higher for the frozen elephant trunk procedure. CONCLUSIONS: The early outcomes of total arch replacement with the frozen elephant trunk procedure were acceptable despite its higher prevalence of emergency or redo surgery, which was comparable to that of the conventional repair. This procedure had higher rates of spinal cord injury than the conventional repair, which is a disadvantage of this approach.

Quantitative psychopathology of interictal psychosis in epilepsy; interaction between epilepsy-related and psychosis-general effects.

OBJECTIVE: There is a historical debate whether psychopathology of epilepsy psychosis is unique to epilepsy or common to other psychoses. However, a large comprehensive studies on this issue are scarce. To clarify the characteristics of interictal psychosis (IIP), we evaluated psychopathology quantitatively. METHODS: This study included 150 patients with IIP (epilepsy+/psychosis+), 187 patients with schizophrenia (SC: epilepsy-/psychosis+), 182 patients with epilepsy (EP: epilepsy+/psychosis-), and 172 non-clinical individuals (NC: epilepsy-/psychosis-). The IIP group comprised 127 chronic and 23 brief psychoses. Age, sex, and years of education, onset and duration of psychosis, and onset and duration of epilepsy were matched among the groups. The psychopathology was evaluated using the 16-item Brief Psychiatric Rating Scale (BPRS), which comprises three symptom factors namely negative symptoms (NS), positive symptoms (PS), and anxiety-depressive symptoms (ADS). RESULTS: For overall 16-BPRS and NS factor scores, there were significant interactions between epilepsy-related (epilepsy+/-) and psychosis-general (psychosis+/-) effects. The EP exhibited higher scores than did the NC, whereas the IIP exhibited lower scores than did the SC. For PS and ADS factor scores, the IIP and SC exhibited a significant psychosis-general effect. Chronic IIP was associated with more serious psychopathologies than was brief IIP. However, limited with chronic IIP, there was a significant interaction between epilepsy-related and psychosis-general effects on the overall 16-BPRS and NS factor scores. CONCLUSION: These findings demonstrate the first large quantitative evidence on the unique psychopathology of IIP which has been only narratively described. The psychopathology is associated with the interaction between epilepsy-related and psychosis-general effects.

Effect of the urgency and landing zone on rates of in-hospital death, stroke, and paraplegia after thoracic endovascular aortic repair in Japan.

OBJECTIVE: The present study used data from the Japanese Committee for Stentgraft Management's national registry, which contains unique surgical data, including surgical timing, anatomic factors, and pathologic factors, to determine the generalized community experience with thoracic endovascular abdominal aortic repair (TEVAR). METHODS: The medical background and short-term outcomes were reviewed for patients who had undergone TEVAR for a thoracic aortic aneurysm (TAA; 14,235 cases) or aortic dissection (AD; 990 type A and 4259 type B) from 2008 to 2015. TEVAR for AD was separated from that for TAAs; only the background and short-term outcomes were evaluated. The technical outcomes of TEVAR for TAA were also evaluated. All the cases were categorized as follows: elective, urgent (within 24 hours after admission), or emergent (immediately after admission). The outcomes included in-hospital mortality and persistent stroke and paraplegia diagnosed at discharge. The number of debranching bypasses, proximal landing zone (0, 1, 2, ≥3), and zone length were included in the logistic regression analysis. RESULTS: The mortality, stroke, and paraplegia rates in the TAA and AD groups were 4.4%, 4.6%, and 3.7% and 4.0%, 2.9%, and 2.8%, respectively. After analyzing the TAA cohort, we found that urgent and emergent cases were associated with all adverse outcomes. The rate of paraplegia increased drastically in the patients with stent graft coverage that extended for six or more zones. Massive atheroma was associated with stroke and paraplegia. The cumulative survival rate of the TAA group was stratified by the urgency (ie, elective, urgent, emergent; P < .001). We found that the more proximal (0, 1, and 2) the landing zone, the greater the risk of stroke. Likewise, the longer (six or more zones) the coverage, the greater the risk of paraplegia. CONCLUSIONS: Urgency was strongly associated with mortality, stroke, and paraplegia, and the classification of urgent and emergent, according to the surgical timing after admission, successfully stratified the population in the long-term overall survival analysis. A proximal landing zone involving the aortic arch and debranching bypasses were associated with the occurrence of stroke, and the length of stent graft coverage for six or more zones was associated with paraplegia. Identifying these risk factors will help operators of TEVAR develop appropriate operative strategies to mitigate patient risk.

Hybrid Assistive Limb® for sporadic inclusion body myositis: A case series.

We evaluated the efficacy of rehabilitation therapy with Hybrid Assistive Limb® (HAL; hereafter HAL therapy) in three patients diagnosed with sporadic inclusion body myositis (sIBM) who were hospitalized to undergo HAL therapy. Among them, one patient participated in eight courses and the other two in two courses of HAL therapy between 2017 and 2020. We determined the mean rate of improvement in two-minute walking distance and 6 m walking speed at the time of hospital discharge. After HAL therapy, we confirmed the patients' desire to continue the use of HAL. In one patient, we observed improvements of 146.0% and 120.0% in two-minute walk and 6 m walking speed, respectively, after the first course of HAL therapy; these values are 133.7% and 130% after the eighth course of HAL therapy. These values exceeded 90% in the other two patients after the second course of HAL therapy. HAL therapy maintained both quantity and quality of ambulation and showed positive psychological effects on patient conditions because it reduces exercise load and facilitates safety. While HAL therapy might be effective in maintaining and improving ambulation in patients with sIBM, we should consider to discontinue HAL therapy as it increased risk of falling.