Effects of Vibration Therapy on the Physical Function of Critically Ill Adults Trial: A Randomized Controlled Trial.

OBJECTIVES: Vibration therapy uses vibration to rehabilitate physical functions. Recently, it has been demonstrated to be safe for critically ill patients. However, its effects on physical functions are unclear. DESIGN: Randomized controlled trial. SETTING: A single-center, ICU. PATIENTS: Patients were randomly assigned to either vibration therapy coupled with protocolized mobilization or protocolized mobilization alone. We included patients who could sit at the edge of the bed or in a wheelchair during their ICU stay. The exclusion criteria were based on the early mobilization inhibition criteria. INTERVENTIONS: The primary outcome was the Functional Status Score for the ICU (FSS-ICU) at ICU discharge. Secondary outcomes were the Medical Research Council score, ICU-acquired weakness, delirium, ICU Mobility Scale (IMS), and ventilator- and ICU-free days. For safety assessment, vital signs were monitored during the intervention. MEASUREMENTS AND MAIN RESULTS: Among 180 patients, 86 and 90 patients remained in the vibration therapy and control groups, respectively. The mean age was 69 ± 13 vs. 67 ± 16 years in the vibration therapy and control groups, and the Acute Physiology and Chronic Health Evaluation (APACHE) II score was 19 (14-25) vs. 18 (13-23). The total FSS-ICU at ICU discharge was 24 (18-27) and 21 (17-26) in the intervention and control groups, respectively ( p = 0.09), and the supine-to-sit ability significantly improved in the intervention group ( p < 0.01). The secondary outcomes were not significantly different. Vital signs remained stable during vibration therapy. In the predefined subgroup analysis, FSS-ICU improved in the population with a higher body mass index (≥ 23 kg/m 2 ), lower APACHE II scores (< 19), and higher IMS scores (≥ 6). CONCLUSIONS: Vibration therapy did not improve the total FSS-ICU. However, the supine-to-sit ability in the FSS-ICU improved without any adverse event.

Intensive surveillance endoscopy for multiple gastrointestinal tumors in a patient with constitutional mismatch repair deficiency: case report.

BACKGROUND: Constitutional mismatch repair deficiency (CMMRD) is an extremely rare autosomal recessive hereditary disease characterized by the absence of mismatch repair gene activity from birth, which results in brain tumors, colonic polyposis, gastrointestinal cancers, and lymphomas later in life. An aggressive approach, including colectomy or proctocolectomy, is recommended for the treatment of colorectal cancer. Additionally, partial colectomy with subsequent endoscopic surveillance may be an alternative strategy due to poor patient's condition, although there is no evidence of surveillance endoscopy after partial colectomy for CMMRD. CASE PRESENTATION: A 13-year-old male patient with a history of T-lymphoblastic lymphoma underwent total gastrointestinal endoscopy, which revealed rectal cancer, colorectal polyposis, and duodenal adenoma. Differential diagnosis included constitutional mismatch repair deficiency according to its scoring system and microsatellite instability, and subsequent germline mutation testing for mismatch repair genes confirmed the diagnosis of constitutional mismatch repair deficiency based on a homozygous mutation in mutS homolog 6 (MSH6). The patient and his family refused colectomy due to the high risk of malignancies other than colorectal cancer, which could require radical surgery. Therefore, the patient underwent low anterior resection of the rectosigmoid colon for rectal cancer and intensive surveillance endoscopy for the remaining colon polyposis. During the 3-year period after initial surgery, 130 polyps were removed and the number of polyps gradually decreased during 6-months interval surveillance endoscopies, although only one polyp was diagnosed as invasive adenocarcinoma (pT1). CONCLUSIONS: Our experience of short surveillance endoscopy illustrates that this strategy might be one of options according to patient's condition.

Clinical, cytogenetic, and molecular analyses of 17 neonates with transient abnormal myelopoiesis and nonconstitutional trisomy 21.

BACKGROUND: Transient abnormal myelopoiesis (TAM) is a unique myeloproliferative disorder that occurs in neonates with constitutional trisomy 21/Down syndrome (DS). Although TAM also develops in neonates without constitutional trisomy 21, the clinical, cytogenetic, and molecular characteristics of those patients are not fully understood. PROCEDURE: We retrospectively evaluated the clinical and cytogenetic findings and GATA1 mutation status of 17 neonates with TAM and nonconstitutional trisomy 21 tested for GATA1 mutations at our institute, and compared the findings with those of 64 neonates with TAM and constitutional trisomy 21/DS. RESULTS: DS clinical features were observed in five of the 17 (29%) patients. In all patients, both trisomy 21 and GATA1 mutations were detected in diagnostic samples. Over a median follow-up of 33 (range, 0-139) months, early death (< 6 months of age) occurred in four patients (24%). Overall and event-free survivals were not significantly different between the patients with TAM and nonconstitutional trisomy 21 and those with TAM and constitutional trisomy 21/DS (five-year overall survival: 76% ± 10% vs 53% ± 13%, P = 0.40; five-year event-free survival: 55% ± 13% vs 48% ± 12%, P = 0.90). The five-year cumulative incidence of progression to myeloid leukemia of DS was also similar between the groups (21% vs 24%, P = 0.80). CONCLUSIONS: Patients with TAM and nonconstitutional trisomy 21 exhibited similar biology and outcomes to those with TAM and constitutional trisomy 21/DS. The possibility of TAM should be considered even in phenotypically normal neonates with TAM symptoms, for appropriate management.

Thalamic pulvinar metabolism, sleep disturbances, and hallucinations in dementia with Lewy bodies: Positron emission tomography and actigraphy study.

OBJECTIVES: Although sleep disturbances are prevalent among patients with dementia with Lewy bodies (DLB), their neural substrates remain unclear. We aimed to clarify the neural substrates of sleep disturbances in patients with DLB. METHODS: We evaluated sleep disturbances, neuropsychiatric symptoms, and brain glucose metabolism in 22 patients with probable DLB using actigraphy, the Neuropsychiatric Inventory (NPI), and 18 F-fluorodeoxyglucose (FDG) positron emission tomography, respectively. Total sleep time (TST) and average activity count per minute (AAC) during sleep were calculated for seven consecutive days via actigraphy. We investigated associations between FDG uptake and the actigraphy parameters using Statistical Parametric Mapping version 12b. Spearman's rank correlation coefficients were used to investigate associations among TST, AAC, and clinical symptoms. The level of statistical significance was set at P < .05. P values were adjusted using the Benjamini-Hochberg method for multiple comparisons. This study was registered with ClinicalTrials.gov (NCT00776347). RESULTS: TST exhibited a significant positive association with FDG uptake in the bilateral orbitofrontal cortex and left thalamus, while AAC exhibited a significant negative association with FDG uptake in the left thalamus and the left parieto-occipital region. FDG uptake in the left pulvinar was associated with both TST and AAC. In addition, TST exhibited a significant negative association with the NPI hallucinations score (r = -0.66, P = .001), while AAC exhibited significant positive associations with the NPI delusions (r = 0.70, P < .001) and hallucinations (r = 0.63, P = .002) scores. CONCLUSIONS: TST and bodily activity during sleep are associated with dysfunction of the left pulvinar and the severity of hallucinations in patients with DLB.

Gastric cancer in children and adolescents in Japan.

BACKGROUND: Very limited data are available on childhood gastric cancer. Using a retrospective survey and literature review, we assessed the clinical features of gastric cancer in children and adolescents. METHODS: We collected information on childhood gastric cancer from pediatricians of 518 hospitals that issue the title of "certified board pediatrician" approved by Japan Pediatric Society, using a questionnaire on background, diagnosis year, onset symptoms, tumor location, histology, nodular gastritis, Helicobacter pylori testing, treatment, and prognosis. Studies were collected using PubMed and the NPO Japan Medical Abstracts Society database. Data for childhood gastric cancer were abstracted from the Japanese Vital Statistics database. RESULTS: Of the 518 hospitals, 349 returned the questionnaire, which identified four patients. Literature review identified 77 cases of gastric cancer, and we analyzed data for 80 children <16 years old. Most patients were >10 years old, and there were no sex differences. Onset symptoms ranged from abdominal pain to non-localized. Sixteen of 44 children had a family history of cancer; 10 had a family history of gastric cancer. Histologically, approximately 80% had undifferentiated-type carcinoma. Prognosis was extremely poor, and two of three tested children were positive for H. pylori infection. Childhood gastric cancer death has been declining. CONCLUSIONS: Childhood gastric cancer is rare in Japan, and information on H. pylori in childhood gastric cancer patients is limited. Declining childhood gastric cancer rates may reflect the decreasing prevalence of infection but further study is necessary to clarify the relationship between H. pylori and gastric cancer.

The possible clinical impact of risperidone on P-glycoprotein-mediated transport of tacrolimus: A case report and in vitro study.

The authors encountered the case of an 8-fold increase in the concentration/dose (C/D) ratio of tacrolimus (TAC) following the coadministration of voriconazole (VRCZ) in a hematopoietic stem cell transplantation (HSCT) recipient. The interaction observed was much greater than expected and the patient had also been treated with oral risperidone (RSP). It was hypothesized that cytochrome P450 (CYP)3A inhibition of the small intestine by voriconazole and P-glycoprotein (P-gp) inhibition of the small intestine by risperidone exerted a synergistic effect on the bioavailability of tacrolimus. The aim of the present study was to evaluate the effect of risperidone on the P-gp-mediated transport of tacrolimus. The transcellular transport of P-gp substrates was examined in Caco-2 and P-gp-expressing renal epithelial LLC-GA5-COL150 cells. In Caco-2 cells, the apical-basal (A-B) transport of rhodamine123 (Rh123) after a 120 min incubation was increased by 47.1%, whereas that in the B-A direction was decreased by 61.7% in the presence of risperidone (100 µm). These results indicate that risperidone showed an inhibitory effect on the P-gp-mediated transport of Rh123. In LLC-GA5-COL150 cells, the A-B transport of tacrolimus after 120 min incubation was increased by 21.7% in the presence of risperidone (100 µm), whereas that in the B-A direction was decreased by 10.7%. These results suggest that risperidone was at least partly involved in the mechanism of the marked increase in the C/D ratio of tacrolimus. This case report provides new insights into the diversity of drug interactions of tacrolimus triggered by the combination of two concomitant drugs.

[Hashimoto's encephalopathy presenting with vertigo and muscle weakness in a male pediatric patient].

Hashimoto's encephalopathy is an anti-thyroid antibody-positive autoimmune encephalopathy. We herein report the case of a 13-year-old male patient with subacute vertigo, muscle weakness in the extremities and gait disturbance who was diagnosed with Hashimoto's encephalopathy. He showed no severe impairment of consciousness and no seizures, and there were no abnormalities on the brain MRI. However, epileptic spike and wave complexes were observed on an electroencephalogram, and a decline in blood flow was diffusely observed on brain SPECT (single photon emission computed tomography). His thyroid function was normal, but he was positive for anti-thyroid antibodies, such as anti-TPO (thyroid peroxidase) antibodies. He was also positive for serum anti-NAE (NH2-terminal alpha-enolase) antibodies. Systemic corticosteroid therapy and high-dose intravenous immunoglobulin therapy were effective, greatly improving his quality of life.

Allogeneic bone marrow transplantation appears to ameliorate IgA nephropathy in a patient with X-linked thrombocytopenia.

Wiskott-Aldrich syndrome (WAS) is caused by a mutation in the WAS gene, and it is clinically characterized by the triad of thrombocytopenia, eczema and immunodeficiency. X-linked thrombocytopenia (XLT), which is a clinically mild form of WAS, is also caused by a WAS gene mutation. Patients with WAS/XLT sometimes also have autoimmune diseases such as IgA nephropathy. Progression of IgA nephropathy may lead to chronic renal failure with a poor prognosis. Here, we describe an XLT patient who also had IgA nephropathy. The patient underwent bone marrow transplantation (BMT) because of an associated-lymphoproliferative disorder, and clinical and histological improvement in his IgA nephropathy was observed after BMT. The amount of galactose-deficient IgA in the patient's serum markedly decreased after BMT. Therefore, immunological reconstitution might improve autoimmune diseases in patients with WAS/XLT.

Estimation of muscle strength from actigraph data in Duchenne muscular dystrophy.

BACKGROUND: The purpose of this study was to evaluate the utility of a wrist actigraph for estimating muscle strength in Duchenne muscular dystrophy patients. METHODS: Twenty-two patients aged 4-19 years wore a wrist actigraph to monitor activities of daily living, and underwent a test of knee extension strength and the 6 min walk test. These measures were made at baseline and at 1 year later. The actigraph data were quantified using the zero crossing mode (ZCM), which indicates the frequency of movement, and the proportional integration mode (PIM), which indicates activity level or vigor of motion. RESULTS: The ZCM and PIM scores of ambulatory patients were higher than those of non-ambulatory patients (P < 0.001). The correlation coefficient between ZCM score and 6 min walk distance, ZCM score and knee extension strength, PIM score and 6 minute walk distance, and PIM score and knee extension strength was -0.44, 0.25, 0.58, and 0.63, respectively. This indicates that the PIM score had a moderate-good association with 6 min walk distance and knee extension strength. CONCLUSION: Muscle strength can be estimated using the PIM score calculated from actigraph data. The PIM score is a good tool for the estimation of muscle strength.

Patients With Subacute Sclerosing Panencephalitis in Japan: A 2022 Nationwide Survey.

BACKGROUND: In Japan, the incidence of subacute sclerosing panencephalitis (SSPE) has reduced; however, the medical conditions and factors associated with disease progression remain unclear. METHODS: A nationwide survey of SSPE was conducted using a questionnaire in 2022. We conducted a descriptive analysis of the patients with SSPE in 2022 and Cox proportional hazards analyses for disease progression. We compared the patients with SSPE with those in a 2007 survey. RESULTS: A total of 37 surviving patients with SSPE were enrolled [median age: 32 years (range: 16-52 years)]. No new cases have been identified since 2017 in the survey. Jabbour stage IV was the most common stage (66.7%). The hazard ratios (95% confidence intervals) of male sex and age at the time of measles infection (years) were 2.56 (1.13-5.76) and 0.57 (0.34-0.93), respectively. Compared with those in 2007, the proportion of patients in hospitals decreased from 13.7% to 2.7%, whereas that of patients in nursing facilities increased from 17.6% to 29.7%. The proportions of patients prescribed inosine pranobex, interferon and ribavirin at the time of the survey decreased from 96.1% to 79.4%, 74.8% to 14.3% and 25.3% to 0%, respectively. The proportions of patients with gastrostomy, tracheostomy and ventilator use increased from 5.9% to 69.7%, 23.3% to 60.0% and 10.8% to 32.4%, respectively. CONCLUSIONS: Decreased measles cases in Japan reduced new SSPE cases. However, surviving patients in 2022 had advanced disease stages and needed medical care. Male sex and early measles infection were significantly associated with disease progression.

Association between milder brain deformation before a shunt operation and improvement in cognition and gait in idiopathic normal pressure hydrocephalus.

We investigated the association between the degree of deformation of the brain before shunt operation and improvement of gait and cognitive impairment after shunt operation in 16 patients with idiopathic normal pressure hydrocephalus (iNPH). We evaluated gait and cognitive impairment and measured the cerebrospinal fluid volume in the ventricles/sylvian fissure (vVS) and the subarachnoid space at high convexity/midline areas (vHCM) using MR images with voxel-based morphometry before and 3 months after shunt operation. We used the ratio of vVS to vHCM (vVS/vHCM) as an index of the severity of brain deformation. After shunt operation, improvements were observed in gait, as shown by the Timed Up and Go (TUG) test and 10-meter reciprocating walking test (WT), and in cognitive function, as shown by the Mini-Mental State Examination, Alzheimer Disease Assessment Scale, Frontal Assessment battery (FAB), and Trail Making test A (TMT-A). The vVS/vHCM ratio was negatively correlated with improvement of the FAB, TMT-A and TUG. Preoperative vVS/vHCM was not significantly correlated with preoperative clinical assessments. The rate of change of vVS/vHCM was positively correlated with improvement in the WT. The improvements of gait and cognitive function were larger in iNPH patients with milder deformation of the brain before shunt operation.

Successful bone marrow transplantation with reduced intensity conditioning in a patient with delayed-onset adenosine deaminase deficiency.

In this case report, we describe successful BMT with RIC in a patient with delayed-onset ADA deficiency. A three-yr-old Japanese boy was diagnosed with delayed-onset ADA deficiency because of recurrent bronchitis, bronchiectasia, and lymphopenia. In addition, autoimmune thyroiditis and neutropenia were present. At four yr of age, he underwent BMT with a RIC regimen, including busulfan and fludarabine, from an HLA-identical healthy sister. Engraftment after BMT was uneventful without GVHD. Decreased ADA levels in blood immediately increased following BMT, and the patient was disease-free 13 months after BMT. These results suggest that BMT with RIC may sufficiently restore immune regulation in delayed-onset ADA deficiency. A longer follow-up period is needed to confirm these observations.

Predictors of prolonged hospital stay for the treatment of severe neuropsychiatric symptoms in patients with dementia: a cohort study in multiple hospitals.

BACKGROUND: Long hospitalization is often needed to treat severe behavioral and psychological symptoms of dementia (BPSD), which places heavy demands on hospital resources. Consequently, patients with severe BPSD usually wait for a long time to be admitted. There is a need to identify factors related to long hospitalization to better manage resources of a psychiatric hospital. METHODS: We surveyed 150 consecutive patients hospitalized in the neuropsychiatric units of three hospitals for treatment of BPSD from 11 May 2009 to 30 November 2010. Only patients with reliable relatives were included in the study. We evaluated data of the patients (demographics, cognitive impairment, activities of daily living, causal disease for dementia, dementia severity, and the amount of pension), their primary caregivers (demographics and care burden), and their doctors' years of experience in treating dementia. We followed up to 180 days and assessed the effect of these factors on the length of stay. RESULTS: Of the 150 patients, 104 were discharged within 180 days and 46 were hospitalized for more than 180 days. Average length of stay for patients was 110.4 ± 58.1 days. In both univariate and multivariate Cox proportional hazard analyses, length of stay was longer for patients with smaller pensions and patients whose doctors had fewer years of experience in treating dementia. Length of stay was not found to be associated with any of the other variables. CONCLUSIONS: Patients with smaller pensions and whose doctors had less experience in treating dementia tended to require longer hospitalization.

Giardiasis in a patient undergoing chemotherapy for retinoblastoma and acute myelogenous leukemia.

Giardiasis is a common cause of diarrhea in undeveloped countries, but is very rare in developed countries. A patient with acute myelogenous leukemia and retinoblastoma presented with a high fever and severe watery diarrhea during induction chemotherapy. On microscopy, cysts were seen in her stool, suggesting Giardia intestinalis, which was confirmed on polymerase chain reaction (PCR). G. intestinalis was also detected in the patient's asymptomatic parents, who may have transmitted it to the patient. Giardiasis should be tested for in patients with severe and persistent diarrhea during chemotherapy, when other etiologies have been excluded. PCR used to amplify the DNA of G. intestinalis is rapid and sensitive.

Reversibility of brain morphology after shunt operations and preoperative clinical symptoms in patients with idiopathic normal pressure hydrocephalus.

AIM: Brain deformations might prevent clinical symptoms from worsening in patients with idiopathic normal pressure hydrocephalus (iNPH). We investigated the relationship between reversibility of brain morphology after shunt operations and preoperative clinical symptoms in iNPH patients. METHODS: Using head magnetic resonance images with voxel-based morphometry, we measured the cerebrospinal fluid volume in the combined areas of the lateral and third ventricles and Sylvian fissure (the volume of the ventricles and Sylvian fissure (vVS)) and the volume of the subarachnoid space at high convexity and midline areas (vHCM) before and 1 year after lumboperitoneal shunt operations in 12 patients with shunt-responsive iNPH. We used the ratio of normalized vVS to normalized vHCM (nvVS/nvHCM) as an index of the severity of the brain deformation. The degree of reversibility of the brain morphology after the shunt operation was defined as the change ratio of the preoperative nvVS/nvHCM to the postoperative nvVS/nvHCM (CR-nvVS/nvHCM). Higher CR-nvVS/nvHCM values indicated more improvement in the brain deformation. In addition, we rated the severity of the white matter lesions on the preoperative magnetic resonance images based on the Fazekas scale. Dependency in activities of daily living, gait and cognition were evaluated before and 1 year after the shunt operations. RESULTS: After the shunt operations, the nvVS/nvHCM and nvVS decreased significantly, and nvHCM increased significantly. The CR-nvVS/nvHCM negatively correlated with the preoperative severity of dependency in activities of daily living, gait and cognitive impairments. The CR-nvVS/nvHCM negatively correlated with the Fazekas scale, but not with age, duration of the disease and cerebrospinal fluid pressure. CONCLUSIONS: Reversibility of brain morphology, which varied among iNPH patients, would prevent clinical symptoms from worsening in iNPH patients. The presence of white matter lesions reduced the degree of reversibility of the brain deformations in iNPH patients.

Gene therapy for spinal muscular atrophy is considerably effective when administered as early as possible after birth.

Introduction: Spinal muscular atrophy (SMA) is a neuromuscular disease characterized by muscle atrophy and progressive muscle weakness. Insurance-approved treatments in Japan include antisense oligonucleotide therapy, gene therapy, and small molecule therapy. The efficacy of these therapies varies depending on the timing of treatment initiation. Case presentation: We report the cases of two infants with SMA born in the same region. Patient 1, who had two copies of SMN2, was born before newborn screening (NBS) was started and received onasemnogene abeparvovec therapy at the age of 4 months. Patient 2, who had three copies of SMN2, was born after the start of NBS and was diagnosed and treated with onasemnogene abeparvovec before symptoms appeared. Unfortunately, Patient 1 became bedridden despite receiving gene therapy, while Patient 2 achieved normal motor development. Discussion: Our findings show that treatment timing is an essential factor affecting patients' motor neurodevelopmental outcomes, although our patients did have differences in the number of copies of SMN2. Therefore, a system should be established to allow all newborns to undergo publicly funded NBS for SMA.

Questionnaire survey on the conceptual framework, optimal evaluation, and support measures for children's language disorders in Japan using the Delphi method.

Objectives: To compile the opinions of native Japanese speakers on the conceptual framework, optimal evaluation, and support measures for children with language disorders to devise materials on which a consensus can be formed. Design: A quantitative descriptive study using the Delphi method. Setting: Using the Delphi method, 43 clinicians with at least 15 years of experience working professionally with children's language disorders in Japan were surveyed three times via a web-based questionnaire. Thirty-nine items that were carefully selected by the working group were surveyed, and the agreement level was set to ≥80%. Main Outcome Measures: We investigated the following aspects related to developmental language disorder (DLD) among Japanese children: definition, core symptoms, evaluation of core symptoms, relationship with a second language, relationship with other related disorders, support systems, and information availability. Results: Overall, 43 qualified panel members were included in this study. Among the 39 items in the questionnaire, a high level of consensus (≥80%) from the responses of the participants was achieved for five items in Round 1, whereas no consensus (<50%) was achieved for seven items. After revising and integrating the questionnaires into 22 items, we conducted Rounds 2 and 3 and obtained high and medium levels of agreement in 20 items on disease concept, core symptoms, coexisting disorders, and manner of support of DLD in children. Conclusion: Our results clarify the previously ambiguous image of DLD in Japan. Information-sharing strategies that connect professionals, patients, their families, and community members are required in the future. Level of Evidence: 5.

Classification of delusions in Alzheimer's disease and their neural correlates.

BACKGROUND: Previous findings on neural correlates of delusion in Alzheimer's disease (AD) have been inconsistent because of methodological issues, such as treating multiple delusions as a single entity. In this retrospective study, we classified AD delusions and investigated their neural correlates by using single-photon emission computed tomography data. METHODS: We selected AD patients with delusions from our consecutive outpatients from 2004 to 2010. In this study, eight types of delusions were evaluated with Neuropsychiatric Inventory and classified by factor analysis. Twenty-five of the patients also had single-photon emission computed tomography data, which we used to assess the relationships between cerebral regions of hypoperfusion and hyperperfusion and each classified delusion. The relations were assessed using Statistical Parametric Mapping with normalization to the white matter cerebral blood flow. RESULTS: The delusions were classified into three factors. Factor 1 consisted of a belief that his/her house is not his/her home, phantom boarder symptom, delusion of abandonment, and belief that one's spouse or others are not who they claim to be. Factor 1 was related to hypoperfusion in the right temporal pole and hyperperfusion in the medial frontal and precentral regions. Factor 2 consisted of delusion relating to the television and delusion of persecution. Factor 2 was related to hypoperfusion in the precuneus and hyperperfusion in the insula and thalamus. Factor 3 consisted of delusion of abandonment and delusional jealousy. Factor 3 was related to hypoperfusion in the right inferior temporal and frontal regions and hyperperfusion in the middle frontal gyrus, insula and posterior cingulate gyrus. Delusion of theft was not included in any factors, and it was related to hypoperfusion in the bilateral thalami and left posterior cingulate gyrus and hyperperfusion in the left inferior frontal regions and anterior cingulate gyrus. CONCLUSIONS: Delusions in AD were classifiable, and each classified delusion was related to different neural networks.

BCOR-CCNB3 sarcoma arising in the proximal tibia: A case report.

BCL6 corepressor-cyclin B3 (BCOR-CCNB3) fusion sarcoma was classified as an emerging subgroup of undifferentiated small round cell sarcoma in 2020. The incidence of BCOR-CCNB3 fusion sarcoma is reportedly 1.5-14% among undifferentiated unclassified sarcomas, representing a rare entity among primary malignant bone tumors. The present study reports a case of BCOR-CCNB3 fusion sarcoma in the proximal tibia of a boy. A 12-year-old boy presented with a 6-month history of knee pain and a slowly growing mass in the anteromedial aspect of the left proximal tibia. Plain radiography and computed tomography of the knee demonstrated a lytic lesion with cortical destruction of the proximal tibia. Magnetic resonance imaging showed the bone tumor expanding into soft tissue with almost homogeneous hypointensity on T1-weighted imaging and slightly hyperintensity on T2-weighted imaging. On histopathological evaluation, the tumor comprised a proliferation of small, round to ovoid-shaped mesenchymal cells without osteoid formation. Histopathologically, BCOR-CCNB3 sarcoma of bone was finally diagnosed based on immunohistochemical staining and additional molecular analyses. The patient underwent bone tumor resection followed by pre- and post-operative chemotherapy according to a Ewing sarcoma protocol. The patient showed no evidence of local recurrence or distant metastasis at 12 months after completion of adjuvant chemotherapy. We present herein an additional case of BCOR-CCNB3 sarcoma of the proximal tibia, and review the relevant literature on BCOR-CCNB3 sarcoma of bone.