Amyloid Positive Hydrocephalus: A Hydrocephalic Variant of Alzheimer's Disease?

BACKGROUND: Alzheimer's disease (AD) and normal pressure hydrocephalus (NPH) commonly coexist. OBJECTIVE: We aimed to characterize an overlapping syndrome of AD and NPH that presents with gait disturbance, ventriculomegaly on magnetic resonance imaging, and significant amyloid deposition on positron emission tomography (PET). METHODS: Of 114 patients who underwent cerebrospinal fluid (CSF) drainage for a possible diagnosis of NPH between 2015 and 2020 in Samsung Medical Center, we identified 24 patients (21.1%) with the NPH patients with amyloid deposition on PET, which we referred to as hydrocephalic AD in this study. We compared their clinical and imaging findings with those of 123 typical AD without hydrocephalic signs/symptoms. We also investigated the frequency and potential predictors of the tap test response in hydrocephalic AD. RESULTS: Evans' index was 0.36±0.03, and a disproportionately enlarged subarachnoid space was present in 54.2% of the hydrocephalic AD patients. The mean age (75.2±7.3 years) and the APOE4 frequency (68.2%) did not differ from those of AD controls. However, the hydrocephalic AD patients showed better memory and language performance, and a thinner cingulate cortex. About 42% of the hydrocephalic AD patients responded to the tap test, of whom seven underwent shunt surgery. Cognition did not improve, whereas gait improved after shunt surgery in all. CONCLUSION: Hydrocephalic AD has different neuropsychological and imaging characteristics from typical AD. Future studies are warranted to further investigate the effect of CSF removal on their clinical course and to elucidate the pathophysiological interaction between amyloid and NPH.

Exploratory Analysis of Behavioral Impulsivity, Pro-inflammatory Cytokines, and Resting-State Frontal EEG Activity Associated With Non-suicidal Self-Injury in Patients With Mood Disorder.

Introduction: Non-suicidal self-injury (NSSI) is a rapidly increasing mental health problem that requires more clinical attention. In this study, we aimed to explore the biobehavioral markers of NSSI in participants with mood disorders. Methods: A total of 45 participants with mood disorders (bipolar I, II, and major depressive disorder) were included in the study. Behavioral impulsivity was measured using the immediate memory task (IMT)/delayed memory task (DMT) and the go-no-go (GNG) tests. Plasma levels of tumor necrosis factor-α (TNF-α), interleukin 1 beta (IL-1 ß), and interleukin 6 (IL-6) and resting-state quantitative electroencephalography (qEEG) were measured. Results: The NSSI group had shorter GNG reaction time (GNG-RT) and higher TNF-α levels compared to the non-NSSI group. TNF-α was positively correlated with frontal theta power. In addition, GNG-RT showed a significant positive association with frontal alpha activity. Conclusion: NSSI in mood disorders was associated with increased behavioral impulsivity and greater inflammation. Increased pro-inflammatory cytokines were associated with frontal theta power. Increased inflammation might change major neurotransmitter metabolism, which eventually affects frontal function and decreases response inhibition. Further studies to explore their causal relationship are warranted.

Allogeneic Hematopoietic Cell Transplantation in Patients with Primary Immunodeficiencies in Korea: Eleven-Year Experience in a Single Center.

PURPOSE: We aimed to report our single-center experience of allogeneic hematopoietic cell transplantation (HCT), which has been the only curative option for certain patients with lethal primary immunodeficiencies (PIDs). METHODS: We summarized the results of HCT performed for patients with PIDs for 11 consecutive years from 2006 to 2016 at Samsung Medical Center, Seoul, Korea. Twenty-six patients with PIDs received HCT. Most had chronic granulomatous disease (42.3%), Wiskott Aldrich syndrome (15.4%), or severe combined immunodeficiency (11.5%). RESULTS: Nine patients (34.6%) received HCT during the former half period and 17 patients (65.4%) during the latter half period. Donor types were categorized as: matched sibling donor (n = 5), unrelated donor (n = 17), and familial mismatched donor (FMMD) (n = 4). Unrelated HCT and FMMD transplantation were increasingly performed in the latter half period compared to the first (5 vs. 16, P = 0.034). Five patients experienced initial engraftment failure, but all of them were eventually engrafted after additional HCTs. The 3-year probability of overall survival was 72.0%. Seven patients (26.9%) died, and the causes of death were bacterial sepsis (n = 4), pneumonia (n = 1), chronic graft-versus-host disease (GVHD) (n = 1), and diffuse alveolar hemorrhage (n = 1). Two patients with bacterial sepsis and a patient with pneumonia also had chronic GVHD. Unrelated HCT and use of methotrexate were associated with poor outcome. Complete chimerism was attained in 85.0% at 1 year after HCT. CONCLUSION: PID candidates have been increasingly identified for allogeneic HCT in Korea, and the majority of them could be cured by HCT. Establishment of a systematic registry of PID patients for HCT is needed.

Comparison of the Microtensile Bond Strength of a Polyetherketoneketone (PEKK) Tooth Post Cemented with Various Surface Treatments and Various Resin Cements.

The aim of this in-vitro research was to evaluate the microtensile bond strength in the newly introduced PEKK tooth post with various surface treatments and resin cements. A fiberglass tooth post was included in order to compare it with PEKK as a possible post material. The microtensile bond strengths of the fiberglass post (FRC Postec Plus) and the PEKK post (Pekkton®) were tested using three kinds of self-adhesive resin cements (G-CEM LinkAce, Multilink Speed, and RelyX U200) and one self-etching resin cement (PANAVIA F2.0). The surface treatments of the fiberglass posts were processed according to the manufacturer's recommendations (F1, application of 37% phosphoric acid etching gel and silanization). For the PEKK post groups, various surface treatments were performed like no surface treatment (P1), sandblasting (P2), silica-coating and silanization (P3), and sandblasting with a composite primer (P4). In the surface treatment, PEKK posts with silica coating and silane treatment (P3) showed a significantly higher microtensile bond strength (mean MPa: 18.09, p < 0.05). The highest microtensile bond strength was shown when the PEKK posts were treated with a silica coating and silane treatment and cemented with RelyX U200 (mean MPa: 22.22). The PEKK posts with surface treatments of silica-coating and silanization or sandblasting displayed superior microtensile bond strengths (mean MPa: 18.09 and 16.25, respectively) compared to the conventional fiberglass posts (mean MPa: 14.93, p < 0.05).

Accuracy of several implant bite registration techniques: an in-vitro pilot study.

PURPOSE: This study evaluated the accuracies of different bite registration techniques for implant-fixed prostheses using three dimensional file analysis. MATERIALS AND METHODS: Implant fixtures were placed on the mandibular right second premolar, and the first and second molar in a polyurethane model. Aluwax (A), Pattern Resin (P), and Blu-Mousse (B) were used as the bite registration materials on the healing abutments (H) or temporary abutments (T). The groups were classified into HA, HP, HB, TA, TP, and TB according to each combination. The group using the bite impression coping was the BC group; impression taking and bite registration were performed simultaneously. After impression and bite taking, the scan bodies were connected to the lab analogs of the casts. These casts were scanned using a model scanner. The distances between two reference points in three-dimensional files were measured in each group. One-way ANOVA and Duncan's test were used at the 5% significance level. RESULTS: The smallest distance discrepancy was observed in the TB group using the temporary abutments. The Blu-Mousse and HP groups showed the largest distance discrepancy. The TB and BC groups showed a lower distance discrepancy than the HP group (P=.001), and there was no significant difference between the groups using the temporary abutments and healing abutments (P>.05). CONCLUSION: Although this study has limitations as an in-vitro investigation, the groups using the temporary abutments to hold the Blu-Mousse record and bite impression coping showed greater accuracy than the group using the healing abutments to hold the pattern resin record.

Preparation and Characterization of Resorbable Bacterial Cellulose Membranes Treated by Electron Beam Irradiation for Guided Bone Regeneration.

Bacterial cellulose (BC) is an excellent biomaterial with many medical applications. In this study, resorbable BC membranes were prepared for guided bone regeneration (GBR) using an irradiation technique for applications in the dental field. Electron beam irradiation (EI) increases biodegradation by severing the glucose bonds of BC. BC membranes irradiated at 100 kGy or 300 kGy were used to determine optimal electron beam doses. Electron beam irradiated BC membranes (EI-BCMs) were evaluated by scanning electron microscopy (SEM), attenuated total reflectance-Fourier transform infrared (ATR-FTIR) spectroscopy, thermal gravimetric analysis (TGA), and using wet tensile strength measurements. In addition, in vitro cell studies were conducted in order to confirm the cytocompatibility of EI-BCMs. Cell viabilities of NIH3T3 cells on 100k and 300k EI-BCMs (100 kGy and 300 kGy irradiated BC membranes) were significantly greater than on NI-BCMs after 3 and 7 days (p < 0.05). Bone regeneration by EI-BCMs and their biodegradabilities were also evaluated using in vivo rat calvarial defect models for 4 and 8 weeks. Histometric results showed 100k EI-BCMs exhibited significantly larger new bone area (NBA; %) than 300k EI-BCMs at 8 weeks after implantation (p < 0.05). Mechanical, chemical, and biological analyses showed EI-BCMs effectively interacted with cells and promoted bone regeneration.

A novel PRF1 gene mutation in a fatal neonate case with type 2 familial hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) occurs in the primary form (genetic or familial) or secondary form (acquired). The familial form of HLH (FHL) is a potentially fatal autosomal recessive disorder that occurs because of constitutional defects in cell-mediated cytotoxicity. Here, we report a fatal neonatal case of type 2 FHL (FHL2) that involved a novel frameshift mutation. Clinically, the newborn presented with severe sepsis-like features and required mechanical ventilation and continuous venovenous hemodiafiltration. Flow cytometry analysis showed marked HLH and complete absence of intracytoplasmic perforin expression in cytotoxic cells; therefore, we performed molecular genetic analyses for PRF1 mutations, which showed that the patient had a compound heterozygous mutation in PRF1, that is, c.65delC (p.Pro22Argfs*2) and c.1090_1091delCT (p.Leu364Glufs*93). Clinical and genetic assessments for FHL are required for neonates with refractory fever and progressive multiple organ failure, particularly when there is no evidence of microbiological or metabolic cause.