Association between Sarcopenia and Balance in Patients Undergoing Inpatient Rehabilitation after Hip Fractures: A Retrospective Cohort Study.

Background and Objectives: Sarcopenia is characterized by a decline in skeletal muscle mass, strength, and function and is associated with advancing age. This condition has been suggested as a factor that negatively influences the functional outcomes of patients with hip fractures. However, the association between sarcopenia and balance impairment in patients undergoing inpatient rehabilitation after hip fractures remains unclear. In this retrospective cohort study, we aimed to investigate the impact of sarcopenia on balance outcomes in patients undergoing inpatient rehabilitation following hip fractures. Materials and Methods: Baseline sarcopenia was diagnosed using skeletal muscle mass index and handgrip strength, with cut-off values recommended by the Asian Working Group for Sarcopenia. The primary outcome was balance, which was assessed using the Berg Balance Scale (BBS) at the time of discharge. A multiple linear regression model analyzed the association between sarcopenia and balance. The model was adjusted for age, sex, comorbidities, and cognitive function. Results: Among the 62 patients (mean age: 78.2; sex: 75.8% women), 24.2% had sarcopenia. Patients with sarcopenia had significantly lower BBS scores than did those without sarcopenia (41 vs. 49 points, p = 0.004). Multiple linear regression analysis revealed that baseline sarcopenia was independently associated with BBS scores at discharge (ß = -0.282, p = 0.038). Conclusions: Following inpatient rehabilitation, patients with baseline sarcopenia had inferior balance outcomes than did those without sarcopenia at discharge. Sarcopenia should be assessed on admission to consider and provide additional care for those with a higher risk of poor functional outcomes. More studies are needed to investigate the association between sarcopenia and functional outcomes, examine the impact of sarcopenia treatment on these outcomes, and reduce the risk of recurrent falls and fractures in patients with hip fractures.

Association between Obesity and Short-Term Patient-Reported Outcomes following Total Knee Arthroplasty: A Retrospective Cohort Study in Japan.

Background: This study investigated the association between obesity and short-term patient-reported outcomes after total knee arthroplasty (TKA). Methods: The primary outcomes were the Western Ontario and McMaster Universities Osteoarthritis Index's (WOMAC) pain and function scores. Data were collected preoperatively and 2 and 4 weeks after surgery. Patients were stratified into three groups based on body mass index (BMI): normal weight (BMI < 24.99 kg/m2), overweight (25 ≤ BMI < 29.99 kg/m2), and obese (BMI ≥ 30 kg/m2). The associations between BMI and the WOMAC pain and function scores were assessed using generalized linear mixed models. Results: Among the 102 patients (median age: 75.0, women [85.3%]), 29.4%, 48.0%, and 22.5% were normal weight, overweight, and obese, respectively. The mean pain and function scores at baseline were similar across the BMI-stratified groups (p = 0.727 and 0.277, respectively). The pain score significantly improved 2 weeks post-surgery (p = 0.001). The function score improved significantly 4 weeks post-surgery (p < 0.001). The group and group-by-time interaction effects lacked statistical significance. Conclusions: All patients statistically and clinically showed relevant pain reduction and functional improvement shortly after TKA, irrespective of their obesity status. These data may help healthcare professionals discuss the expectations of pain amelioration and functional improvement with TKA candidates.

Gait training using a wearable robotic hip device for incomplete spinal cord injury: A preliminary study.

CONTEXT/OBJECTIVE: To explore changes in gait functions for patients with chronic spinal cord injury (SCI) before and after standard rehabilitation and rehabilitation with a wearable hip device, explore the utility of robot-assisted gait training (RAGT), and evaluate the safety and dose of RAGT. DESIGN: Single-arm, open-label, observational study. SETTING: A rehabilitation hospital. PARTICIPANTS: Twelve patients with SCI. INTERVENTIONS: Standard rehabilitation after admission in the first phase. RAGT for two weeks in the second phase. OUTCOME MEASURES: Self-selected walking speed (SWS), step length, cadence, and the 6-minute walking distance were the primary outcomes. Walking Index for SCI score, lower extremity motor score, and spasticity were measured. Walking abilities were compared between the two periods using a generalized linear mixed model (GLMM). Correlations between assessments and changes in walking abilities during each period were analyzed. RESULTS: After standard rehabilitation for 66.1 ± 36.9 days, a period of 17.6 ± 3.3 days of RAGT was safely performed. SWS increased during both periods. GLMM showed that the increase in cadence was influenced by standard rehabilitation, whereas the limited step length increase was influenced by RAGT. During RAGT, the increase in step length was related to an increase in hip flexor function. CONCLUSIONS: Gait speed in patients with SCI increased after rehabilitation, including RAGT, in the short-term. This increase was associated with improved muscle function in hip flexion at the start of RAGT.Trial Registration: This study was registered with the UMIN Clinical Trials Registry (UMIN-CTR; UMIN000042025).

Benefits of a Wearable Cyborg HAL (Hybrid Assistive Limb) in Patients with Childhood-Onset Motor Disabilities: A 1-Year Follow-Up Study.

Rehabilitation robots have shown promise in improving the gait of children with childhood-onset motor disabilities. This study aimed to investigate the long-term benefits of training using a wearable Hybrid Assistive Limb (HAL) in these patients. Training using a HAL was performed for 20 min a day, two to four times a week, over four weeks (12 sessions in total). The Gross Motor Function Measure (GMFM) was the primary outcome measure, and the secondary outcome measures were gait speed, step length, cadence, 6-min walking distance (6MD), Pediatric Evaluation of Disability Inventory, and Canadian Occupational Performance Measure (COPM). Patients underwent assessments before the intervention, immediately after the intervention, and at 1-, 2-, 3-month and 1-year follow-ups. Nine participants (five males, four females; mean age: 18.9 years) with cerebral palsy (n = 7), critical illness polyneuropathy (n = 1), and encephalitis (n = 1) were enrolled. After training using HAL, GMFM, gait speed, cadence, 6MD, and COPM significantly improved (all p < 0.05). Improvements in GMFM were maintained one year after the intervention (p < 0.001) and in self-selected gait speed and 6MD three months after the intervention (p < 0.05). Training using HAL may be safe and feasible for childhood-onset motor disabilities and may maintain long-term improvements in motor function and walking ability.

Prevalence of Sarcopenic Obesity and Factors Influencing Body Composition in Persons with Spinal Cord Injury in Japan.

This study aims to investigate the prevalence of sarcopenic obesity and factors influencing body composition in persons with spinal cord injury (SCI) in Japan. Adults with SCI aged ≥ 20 years who underwent whole-body dual-energy X-ray absorptiometry between 2016 and 2022 were retrospectively analyzed. Data from 97 patients were examined. The primary outcome was appendicular skeletal muscle mass (ASM). Multiple linear regression analysis was conducted to assess factors influencing the lean and adipose indices in persons with SCI. Sarcopenia, obesity, and sarcopenic obesity were prevalent in 76%, 85%, and 64% of patients, respectively. Multivariate linear regression analysis revealed that sex (ß = 0.34, p < 0.001), lesion level (ß = 0.25, p = 0.007), severity (ß = 0.20, p = 0.043), and ability to walk (ß = 0.29, p = 0.006) were independently associated with ASM. Sex (ß = −0.63, p < 0.001) was independently associated with percent body fat. In conclusion, sarcopenia, obesity, and sarcopenic obesity were prevalent among patients with SCI in Japan. Female sex, tetraplegia, motor-complete injury, and inability to walk were risk factors for sarcopenia, whereas female sex was a risk factor for obesity in persons with SCI. A routine monitoring of body composition is necessary, especially among those with multiple risk factors, to identify individuals in need of preventive and therapeutic interventions.

Short-Term Outcome of Rehabilitation Program with Hybrid Assistive Limb after Tendon Lengthening in Patients with Cerebral Palsy.

In this study, we aimed to evaluate the short-term outcomes of a rehabilitation program with the Hybrid Assistive Limb® after soft tissue lengthening in young patients with cerebral palsy. We assessed six patients with cerebral palsy who underwent soft tissue surgery followed by gait training using the Hybrid Assistive Limb®. Clinical assessments were conducted preoperatively, before, immediately after, and at 1, 2, and 3 months after gait training. Gross Motor Function Measure was improved 5.93 ± 6.11% (mean ± standard deviation, p < 0.05), Canadian Occupational Performance Measure performance was improved 3.12 ± 1.53 points, and satisfaction was improved 3.80 ± 2.14 points (p < 0.05). The knee extension strength on the operated side was changed 7.75 ± 4.97 Nm after the intervention (p = 0.07). In ambulatory patients, gait speed was changed 8.37 ± 1.72 m/min, stride length was changed 10 ± 6.16 cm, and 6 min walking distance was changed 52 ± 16 m after the intervention. Training with the Hybrid Assistive Limb® may improve walking ability and clinical outcomes in young patients with cerebral palsy after soft tissue lengthening.

Voluntary-assisted Upper Limb Training for Severe Cerebral Palsy Using Robotics Devices and Neuromuscular Electrical Stimulation: Three Case Reports.

Background: Constraint-induced movement therapy (CIMT) improves the motor function of paralyzed upper limbs of adults after stroke. However, in patients with severe spastic cerebral palsy (CP), the use of CIMT is not warranted. Our aim was to investigate the feasibility and effectiveness of repetitive voluntary-assisted upper limb training (VAUT) for three patients with severe CP using a combination of robotics [Hybrid Assistive Limb (HAL)] and functional electrical stimulation [Integrated Volitional Control Electrical Stimulation (IVES)]. Case: Three patients with CP were enrolled. Patients 1, 2, and 3 were 8-, 19-, and 18-year-old males, respectively. Patient 1 had spastic hemiplegia, while patients 2 and 3 had spastic quadriplegia. VAUT using single-joint HAL was performed for 1 or 2 sessions/month for 50 min/session over an 8-month period for 9-13 sessions in total. One patient's voluntary hand movement was insufficient, affecting his upper limb exercise performance; therefore, IVES was required in addition to HAL. Outcome measures included motor function of the upper limbs and use of paralyzed hands, which were measured before and after intervention. No adverse events were observed during VAUT. After intervention, the Action Research Arm Test scores showed improvements in all three patients. The Children's Hand-use Experience Questionnaire showed improvements in two patients. Discussion: The use of VAUT, together with new systems such as HAL and IVES, for severe CP is safe and may be effective. Our study suggested that upper limb function can be improved for patients with severe CP.

Robot-Assisted Ankle Rehabilitation Using the Hybrid Assistive Limb for Children after Equinus Surgery: A Report of Two Cases.

After equinus corrective surgery, repetitive exercises for ankle dorsiflexion and plantar flexion are crucial during rehabilitation. The single-joint Hybrid Assistive Limb (HAL-SJ) is an advanced exoskeletal robotic device with a control system that uses bioelectrical signals to assist joint motion in real time and demonstrates joint torque assistance with the wearer's voluntary movement. We present two cases of robot-assisted ankle rehabilitation after equinus surgery using the HAL-SJ in children. Case 1 was an 8-year-old boy, whereas case 2 was a 6-year-old boy. When they were allowed to walk without braces, training with the HAL-SJ was performed postoperatively for 20 min per session a total of eight times (2-4 sessions per week). Assessments were performed before and after HAL-SJ training. During gait analysis, case 1 had improved joint angles during the stance phase on the operated side; however, case 2 had improved joint angles during the stance and swing phases. The co-activation index values of the medial gastrocnemius and tibialis anterior muscles, which were high before training, decreased after training and approached the standard value. The HAL-SJ may provide systematic feedback regarding voluntary ankle dorsiflexion and plantar flexion and is considered to have motor learning effects.

Setting Mechanical Insufflation-Exsufflation (MI-E) Pressures for Amyotrophic Lateral Sclerosis (ALS) Patients to Improve Atelectasis and Reduce Risk of Pneumothorax: A Case Report.

Mechanical insufflation-exsufflation (MI-E) has been used to supplement the ability to cough and expel pulmonary secretions in patients with neuromuscular disease who have a reduced ability to cough. The manufacturer's guidelines for MI-E recommend a setting of inspiratory pressure of +40 cmH2O and expiratory pressure of -40 cmH2O. However, patients with small stature and restricted ventilatory impairment are prone to pneumothorax, so the manufacturer's recommendations are not used as is, and should be adjusted for the physical and pulmonary characteristics of each patient. Here, we report a case in which MI-E was used for an amyotrophic lateral sclerosis (ALS) patient with short height, low BMI, and restricted lung capacity at inspiratory and expiratory pressures lower than the manufacturer's recommendations. In adjusting MI-E pressure, physical observations such as chest auscultation, visual chest dilation, and observation of secretion movement toward the tracheal tube were performed to avoid unnecessary pressure. As a result, the pressure level set was lower than the manufacturer's recommendation (25 cmH2O) but sufficient to improve atelectasis and no pneumothorax occurred. The method we practiced in this study is feasible in any clinical setting. We also believe that MI-E, when performed in conjunction with treatment response observation, can be expected to improve at lower pressures than generally recommended, thereby reducing the risk of lung injury and providing safer treatment.

Evaluation of Anterior Coverage in Children With Developmental Dysplasia of the Hip Using Transverse Magnetic Resonance Imaging at 2 Years Is Predictive of Future Radiographic Coverage.

BACKGROUND: Although normal anterior acetabular coverage provides stability to the hip, acetabular retroversion leads to femoroacetabular impingement related to hip osteoarthritis. Previous studies have focused on acetabular version and anteroposterior coverage in children with developmental dysplasia of the hip (DDH); however, the correlation between anteroposterior coverage and acetabular development is unclear. We measured anteroposterior acetabular coverage in DDH patients using transverse magnetic resonance imaging (MRI) and subsequent bony acetabular growth, and evaluated the correlation of those findings. METHODS: We evaluated 37 DDH (dislocations) in 36 patients who underwent MRI at 2 years of age. The mean age was 2.2±0.3 years at the time of MRI (1.6±0.4 y after reduction) and 6.0±0.1 years at the time of plain radiography for the Severin classification. On MRI scans, we measured the cartilaginous center-edge angle (CCEA) and cartilaginous acetabular-head index (CAHI) in the coronal plane and the anterior and posterior cartilaginous center-edge angles (AC-CEA and PC-CEA, respectively) in the transverse plane. Severin I or II was defined as a good outcome and III or IV as a poor outcome. RESULTS: In the evaluations conducted at 2 years of age, the mean CCEA, CAHI, AC-CEA, and PC-CEA were 14±9 degrees, 66%±10%, 39±8 degrees, and 77±7 degrees, respectively; the CEA at 6 years of age was 13±7 degrees. Twelve and 25 hips were classified in the good and poor outcome groups, respectively. Although CCEA, CAHI, and AC-CEA were significantly associated with the outcome in a single regression analysis ( P <0.05), only AC-CEA was significant in the multiple regression analysis with a stepwise selection method ( P =0.018). The cutoff AC-CEA value for a good outcome was 38 degrees (sensitivity, 67%; specificity, 68%) using a receiver operating characteristic curve. CONCLUSIONS: Among MRI findings for acetabular cartilaginous morphology, AC-CEA was strongly associated with the outcome. Anteroposterior coverage was correlated with bony acetabular growth in childhood, and anterior coverage was particularly important for subsequent acetabular growth. LEVEL OF EVIDENCE: Level IV-case series.

Endogenous human retrovirus-K is not increased in the affected tissues of Japanese ALS patients.

Activation of human endogenous retrovirus-K (HERV-K) is one of the proposed risk factors for amyotrophic lateral sclerosis (ALS). The HERV-K envelope protein has been reported to show neurotoxicity, and development of therapy with reverse transcriptase inhibitors is being investigated. On the other hand, some reports have failed to show HERV-K activation in ALS. In this study, we analyzed the expression of HERV-K mRNA in the motor cortex and spinal cord of 15 Japanese patients with sporadic ALS and 19 controls using reverse transcriptase droplet digital PCR. This revealed no significant increase of HERV-K expression in ALS-affected tissues, suggesting that the association between ALS and HERV-K remains questionable.

Migration and phenotype switching of macrophages at early-phase of bone-formation by secretomes from bone marrow derived mesenchymal stem cells using rat calvaria bone defect model.

BACKGROUND/PURPOSE: Conditioned media of cultured mesenchymal stem cells (MSCs) contain numerous kinds of secretomes such as cytokines and chemokines. We previously reported that conditioned media of bone marrow-derived MSCs (MSC-CM) promote bone formation. Recently, macrophage phenotype switching from the pro-inflammatory M1 type to the anti-inflammatory M2 type has been reported to be an important phenomenon during tissue regeneration. Some studies reported that this phenotype switching is regulated by secretomes. In this study, macrophage phenotype during bone formation by MSC-CM was investigated. MATERIALS AND METHODS: Human MSCs (hMSCs) were cultured in serum-free medium and the collected medium was defined as MSC-CM. Macrophage-related gene expressions in hMSCs cultured with MSC-CM were evaluated by quantitative real-time polymerase chain reaction. MSC-CM was implanted and the evaluations by micro-CT and immunohistochemistry were performed using a rat the calvaria bone defect model. RESULTS: Two and four weeks after implantation, the MSC-CM group demonstrated enhanced bone regeneration. Gene expressions of C-C motif chemokine 2 (CCL2), colony-stimulating factor 2 (CSF2) and CD163 was significantly upregulated in cells exposed to MSC-CM. Immunohistochemical staining revealed that iNOS-positive M1 macrophages were reduced, while CD204-positive M2 macrophages were increased in the MSC-CM group at 72 h after implantation, and the M2/M1 ratio increased only in the MSC-CM group. CONCLUSION: MSC-CM enhances macrophage migration and induces M1 to M2 type macrophage switching at an early stage of osteogenesis. Such phenotype switching provides a favorable environment for angiogenesis, cellular migration, and osteogenesis and contributes to MSC-CM-induced early bone formation.

Influence of commuting methods on low back pain and musculoskeletal function of the lower limbs in elementary school children: A cross-sectional study.

BACKGROUND: Lower limb flexibility is known to be decreased in those living in mountainous areas, and musculoskeletal growth is often influenced by lifestyle factors. The purpose of this study was to determine the relationship between the means of transportation to school and lower limb musculoskeletal function and low back pain. METHODS: During routine medical examinations conducted in two cities in a prefecture in 2016, a questionnaire was administered. The parents of 15,259 students who attended elementary school answered questions evaluating the following parameters: (1) anteflexion limit in the standing posture, (2) crouching failure, (3) one leg standing failure, (4) low back pain during lumbar extension, (5) lower limb alignment abnormalities, and (6) flat foot. Students were divided into a walking commuting group (13,569 students) and a vehicle commuting group (1690 students), and findings were compared between the two groups using chi-square tests. RESULTS: In the walking and vehicle commuting groups, anteflexion limit in the standing posture was present in 23.3% and 26.1% of students, respectively (p = 0.013). Crouching failure was present in 4.6% and 7.3% of students (p < 0.001); one leg standing failure in 5.3% and 8.5% of students (p < 0.001); low back pain in 5.0% and 7.1% of students (p < 0.001); positive leg alignment abnormalities in 7.3% and 8.0% of students (p = 0.260); and flat foot in 7.4% and 8.7% of students (p = 0.067), respectively. Significant differences were observed in all four of the failure measures. No significant differences were observed in the two methods evaluating morphological abnormalities. CONCLUSIONS: The decreased walking duration and increased sitting duration associated with vehicle commuting had little effect on lower limb morphology but could induce lower limb dysfunction and low-back pain in children. Thus, supplemental walking should be instated for children who commute by car.

The relationship between gluteus medius and minimus muscle volumes and hip development in developmental dysplasia of the hip.

BACKGROUND: Several studies in adult hips have revealed the role of the gluteus medius (Gmed) and gluteus minimus (Gmin) muscles in maintaining the stability and centripetal force of the hip joint. Hip centripetality in developmental dysplasia of the hip (DDH) patients contributes to subsequent healthy hip development later in life. The purpose of this study is to investigate the relationship between Gmed and Gmin volume and centripetality of the hip in infant DDH patients. METHODS: We retrospectively enrolled 41 unilateral DDH patients (4 males, 37 females) who were treated by closed reduction from 2006 to 2016 and underwent magnetic resonance imaging at around 2 years old. Gmed, and Gmin volume was measured in magnetic resonance imaging. We defined both Gmin and Gmed together as hip abductor gluteus muscles (GMs; Gmed + Gmin). The muscle volume ratio of the affected side was calculated by dividing the GMs volume of the affected side by the contralateral side. Relationships between center-head distance discrepancy (CHDD) at 2 years old, and at 4-6 years old and GMs volume ratio were investigated by Pearson's correlation coefficient within the same patients. RESULTS: Mean age of closed reduction was 0.8 years old and mean age at MRI was 2.2 years old with a mean follow-up period of 3.7 years. Mean GMs volume in the affected side, contralateral side, and muscle volume ratio were 25.3 cm3, 27.0 cm3, and 0.94, respectively. GMs volumes were significantly higher in the contralateral side (p < 0.001). GMs volume ratio at 2 years old significantly correlated with CHDD at 4-6 years old (p < 0.05). CONCLUSION: GMs volume at 2 years old was found to be associated with later hip afferents. Promoting the healthy development of GMs by properly maintaining the infant's natural hip movement is important for the healthy hip development. LEVEL OF EVIDENCE: Level III.

The use of an ultrasonic curettage device in orthognathic surgery decreases surgery-related blood loss.

Objective: This study aimed to compare the use of a powered instrument (PI) and ultrasonic curettage device (ULCD) with intraoperative blood loss (IOBL), drain volume (DV), calculated blood loss (CBL), and hidden blood loss (HBL) in orthognathic surgery. Methods: We included 163 patients who underwent bimaxillary surgery in our department. CBL was calculated from the preoperative and postoperative hemoglobin levels using the "hemoglobin balance method." CBL is an indicator of the amount of perioperative blood loss. HBL was calculated by subtracting IOBL and DV from CBL. Results: The PI group consisted of 61 patients (17 males and 44 females, age: 24.9 ± 9.5 years), and the ULCD group consisted of 102 patients (40 males and 62 females, age: 23.1 ± 7.8 years). In the PI group, the median IOBL, DV, CBL, and HBL were 540.0 (interquartile range [IQR] 380.0-670.0), 113.0 (IQR 77.0-147.0), 1000.0 (IQR 751.4-1248.6), and 285.8 (IQR 151.0-476.4) ml, respectively. In the ULCD group, the median IOBL, DV, CBL, and HBL were 327.5 (IQR 200.0-455.0), 105.5 (IQR 75.3-136.0), 759.5 (IQR 594.9-944.2), and 294.2 (IQR 120.8-456.9) ml, respectively. IOBL and CBL were significantly reduced with ULCD use, but no significant differences were observed in DV and HBL. Conclusions: This study showed that IOBL decreased with ULCD use, resulting in a decrease in CBL. Conversely, bleeding parameters (DV and HBL), which reflect the amount of bleeding that occurs after wound closure, did not show a decrease with ULCD use.

Melanotic neuroectodermal tumor of infancy in the mandible: A case report.

RATIONALE: Melanocytic neuroectodermal tumor of infancy (MNTI) is a rare benign pigmented neoplasm that arises from the neural crest and has an aggressive growth pattern. It is predominantly seen in infants under 1 year of age, and the most common site of involvement is the maxilla. The currently accepted treatment is removal by surgical resection. Herein, we report a case of MNTI that involved the anterior alveolar ridge of the mandible in a 6-month-old infant. PATIENT CONCERNS: A case of a 6-month-old male child with a huge mass in the anterior alveolar ridge of the mandible. DIAGNOSIS: The tumor was diagnosed using histopathological and immunohistochemical techniques on the biopsy specimen obtained following incisional biopsy. Based on the findings, a final diagnosis of MNTI was established. INTERVENTIONS: Radical resection of the tumor was performed, after determining the extent of resection by referring to the mandibular 3D model created using the pre-operative CT data. OUTCOMES: The postoperative course was uneventful, and no recurrence has been observed to date for more than 4 years after surgery. LESSONS: This case emphasizes that early diagnosis and radical surgery are critical to the effective treatment, as MNTI exhibits rapid and destructive growth. It also requires careful and close follow-up because of high recurrence rates.

"Burnt-out" progressive multifocal leukoencephalopathy in idiopathic CD4+ lymphocytopenia.

Progressive multifocal leukoencephalopathy (PML) is a fatal disease caused by John Cunningham virus (JCV) infection; however, a growing number of PML patients now survive longer and achieve remission, largely due to the advent of combination antiretroviral therapy. Several reports have suggested that the pathology in such patients presents only chronic demyelination without characteristic cellular changes, being referred to as "burnt-out" PML. On the other hand, our knowledge of "burnt-out" PML is still substantially limited, especially in patients with non-human immunodeficiency virus infection. Here, we report a case of PML associated with idiopathic CD4+ lymphocytopenia (ICL) who presented with spontaneous remission and survived for 11 years after onset. Notably, postmortem examination revealed surprisingly broad "burnt-out" lesions lacking the classic histopathological findings. However, pathogenic JCV-specific DNA sequences was still present in the autopsied brain tissue. This case suggests that complete remission can be achieved with a persistent presence of JCV-specific pathogenic sequences, even after a catastrophic infection. Considering that there have been a few reported cases of PML with ICL with long survival, the long-term survival of our case may share a favorable immunological response that is unique to a subgroup of ICL.

Conditioned medium from mesenchymal stem cells improves condylar resorption induced by mandibular distraction osteogenesis in a rat model.

Condylar resorption (CR) after surgical orthognathic treatment is defined as dysfunctional remodeling of the temporomandibular joint manifested by morphological changes with decreased condylar head volume that cause occlusal and esthetic changes. Although both conservative and surgical treatment strategies have been employed for the treatment of CR, effective procedures have not been established till date. In this study, the effects of MSC-CM on CR were investigated. Bone marrow-derived MSCs of rats (rMSCs) were cultured until 80% confluent, cultured in serum-free conditioned medium for 48 h; the collected medium was defined as MSC-CM. Osteogenesis, chondrogenesis, and angiogenesis-related gene expression in rMSCs cultured with MSC-CM was evaluated by quantitative real-time polymerase chain reaction. A rat CR model was used for animal studies, in which CR occurred after mandibular distraction osteogenesis for 10 days. MSC-CM was injected via the tail vein and quantitative and qualitative evaluations were performed by micro-computed tomography (micro-CT) and histology. MSC-CM enhanced osteogenesis-, chondrogenesis-, and angiogenesis-related gene expression in rMSCs. Micro-CT showed CR in control groups; however, it was observed to be improved in the MSC-CM group. Histologically, an enlarged cartilage layer was seen in the MSC-CM group, while cartilage layers had almost thinned or disappeared in control groups. These results indicate that MSC-CM improved CR.

Genetic Variations and Neuropathologic Features of Patients with PRKN Mutations.

BACKGROUND: Mutations in PRKN are the most common cause of autosomal recessive juvenile parkinsonism. The objective of this study was to investigate the association between genotype and pathology in patients with PRKN mutations. METHODS: We performed a sequence and copy number variation analysis of PRKN, mRNA transcripts, Parkin protein expression, and neuropathology in 8 autopsied patients. RESULTS: All the patients harbored biallelic PRKN mutations. Two patients were homozygous and heterozygous, respectively, for the missense mutation p.C431F. Seven patients had exon rearrangements, including 2 patients from a single family who harbored a homozygous deletion of exon 4, and 3 patients who carried a homozygous duplication of exons 6-7, a homozygous duplication of exons 10-11, and a heterozygous duplication of exons 2-4. In the other 2 patients, we found a compound heterozygous duplication of exon 2, deletion of exon 3, and a heterozygous duplication of exon 2. However, sequencing of cDNA prepared from mRNA revealed 2 different transcripts derived from triplication of exon 2 and deletion of exons 2-3 and from duplication of exons 2-4 and deletion of exons 3-4. Western blotting and immunohistochemistry revealed faint or no expression of Parkin in their brains. In the substantia nigra pars compacta, a subfield-specific pattern of neuronal loss and mild gliosis were evident. Lewy bodies were found in 3 patients. Peripheral sensory neuronopathy was a feature. CONCLUSIONS: Genomic and mRNA analysis is needed to identify the PRKN mutations. Variable mutations may result in no or little production of mature Parkin and the histopathologic features may be similar. © 2021 International Parkinson and Movement Disorder Society.

Cross-Sectional Survey of Childhood Acetabular Development in Japan.

INTRODUCTION: We aimed to clarify childhood acetabular development and to identify the incidence of children's hip dysplasia in Japan using radiographs of the contralateral hip. METHODS: We performed radiological cross-sectional evaluation of hip development in 211 patients (106 boys, 211 hips) in different age groups (age range: 3-9 years). We excluded patients who complained of bilateral coxalgia at the first visit or had received a diagnosis of acetabular dysplasia. We measured the acetabular index (AI), center-edge angle (CEA), and acetabular head index (AHI) in plain radiographs taken at the first visit. RESULTS: A significant correlation was found between age and CEA in boys, but other parameters had no significant correlation. The mean AI values in boys and girls were 18 ± 3° and 20 ± 4° (p < 0.01), respectively, and the mean CEA values were 25 ± 5° and 24 ± 5° (p = 0.43), respectively. The mean AHI values in boys and girls were 83 ± 6% and 81 ± 7%, respectively (p < 0.01). Two of the 120 children (66 boys and 54 girls) aged ≥6 years old had a hip CEA < 15°; both were girls. CONCLUSIONS: We found decreased acetabular development in girls, and 4% (2/54) of girls without any history of dislocation belonged to Severin's group III. Acetabular dysplasia was observed more frequently in children from Japanese than in those from other countries. Girls with less than two standard deviations in hip dysplasia indices had an AI of 28°, an AHI of 67%, and a CEA of 14°. These reference values may be useful as prognostic indicators for hip dysplasia and OA in adulthood.