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Abstract Background Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease. Pathologically, it is characterized by eosinophilic hyaline intranuclear inclusions in the cells of the visceral organs as well as central, peripheral, and autonomic nervous system cells. Recently, a GGC repeat expansion in the NOTCH2NLC gene has been identified as the etiopathological agent of NIID. Interestingly, this GGC repeat expansion was also reported in some patients with a clinical diagnosis of amyotrophic lateral sclerosis (ALS). However, there are no autopsy-confirmed cases of concurrent NIID and ALS. Case presentation A 60-year-old Taiwanese woman reported a four-month history of progressive weakness beginning in the right foot that spread to all four extremities. She was diagnosed with ALS because she met the revised El Escorial diagnostic criteria for definite ALS with upper and lower motor neuron involvement in the cervical, thoracic, and lumbosacral regions. She died of respiratory failure at 22 months from ALS onset, at the age of 62 years. Brain magnetic resonance imaging (MRI) revealed lesions in the medial part of the cerebellar hemisphere, right beside the vermis (paravermal lesions). The subclinical neuropathy, indicated by a nerve conduction study (NCS), prompted a potential diagnosis of NIID. Antemortem skin biopsy and autopsy confirmed the coexistence of pathology consistent with both ALS and NIID. We observed neither eccentric distribution of p62-positive intranuclear inclusions in the areas with abundant large motor neurons nor cytopathological coexistence of ALS and NIID pathology in motor neurons. This finding suggested that ALS and NIID developed independently in this patient. Conclusions We describe a case of concurrent NIID and ALS discovered during an autopsy. Abnormal brain MRI findings, including paravermal lesions, could indicate the coexistence of NIID even in patients with ALS showing characteristic clinical phenotypes.

To ensure high-reliability communication in healthcare networks, this paper presents a smart gateway system that includes an angle-of-arrival (AOA) estimation and a beam steering function for a small circular antenna array. To form a beam toward healthcare sensors, the proposed antenna estimates the direction of the sensors utilizing the radio-frequency-based interferometric monopulse technique. The fabricated antenna was assessed based on the measurements of complex directivity and the over-the-air (OTA) testing in Rice propagation environments using a two-dimensional fading emulator. The measurement results reveal that the accuracy of the AOA estimation agrees well with that of the analytical data obtained through the Monte Carlo simulation. This antenna is embedded with a beam steering function employing phased array technology, which can form a beam spaced at 45° intervals. The ability of full-azimuth beam steering with regard to the proposed antenna was evaluated by beam propagation experiments using a human phantom in an indoor environment. The received signal of the proposed antenna with beam steering increases more than that of a conventional dipole antenna, confirming that the developed antenna has great potential of achieving high-reliability communication in a healthcare network.

This paper presents an over-the-air testing method in which a full-rank channel matrix is created for a massive multiple-input multiple-output (MIMO) antenna system utilizing a fading emulator with a small number of scatterers. In the proposed method, in order to mimic a fading emulator with a large number of scatterers, the scatterers are virtually positioned by rotating the massive MIMO antenna. The performance of a 64-element quasi-half-wavelength dipole circular array antenna was evaluated using a two-dimensional fading emulator. The experimental results reveal that a large number of available eigenvalues are obtained from the channel response matrix, confirming that the proposed method, which utilizes a full-rank channel matrix, can be used to assess a massive MIMO antenna system.

This paper presents a method of implementing a 4 × 4 correlation matrix for evaluating the uplink channel properties of multiple-input multiple-output (MIMO) antennas using an over-the-air measurement system. First, the implementation model used to determine the correlation coefficients between the signals received at the base station (BS) antennas via the uplink channel is described. Then, a methodology is introduced to achieve a 4 × 4 correlation matrix for a BS MIMO antenna based on Jakes’ model by setting the initial phases of the secondary wave sources in the two-dimensional channel model. The performance of the uplink channel for a four-element MIMO terminal array antenna is evaluated using a two-dimensional bidirectional fading emulator. The results show that the measured correlation coefficients between the signals received via the uplink channel at the BS antennas using the proposed method are in good agreement with the BS correlation characteristics calculated using Monte Carlo simulation and the theoretical formula, thereby confirming the effectiveness of the proposed method.

This paper presents a multiple-input, multiple-output (MIMO) antenna system with the ability to perform full-azimuth beam steering, and with the aim of realizing greater than 20 Gbps vehicular communications. The MIMO antenna described in this paper comprises 64 elements arranged in a daisy chain array structure, where 32 subarrays are formed by pairing elements in each subarray; the antenna yields 32 independent subchannels for MIMO transmission, and covers all communication targets regardless of their position relative to the array. Analytical results reveal that the proposed antenna system can provide a channel capacity of more than 200 bits/s/Hz at a signal-to-noise power ratio (SNR) of 30 dB over the whole azimuth, which is equivalent to 20 Gbps for a bandwidth of 100 MHz. This remarkably high channel capacity is shown to be due to two significant factors; the improved directivity created by the optimum in-phase excitation and the low correlation between the subarrays due to the orthogonal alignment of the array with respect to the incident waves. Over-the-air (OTA) experiments confirm the increase in channel capacity; the proposed antenna can maintain a constant transmission rate over all azimuth angles.

Oxidative stress occurs early in the progression of Alzheimer disease, significantly before the development of the pathologic hallmarks, neurofibrillary tangles and senile plaques. In the first stage of development of the disease, amyloid-β deposition and hyperphosphorylated tau function as compensatory responses and downstream adaptations to ensure that neuronal cells do not succumb to oxidative damage. These findings suggest that Alzheimer disease is associated with a novel balance in oxidant homeostasis.

An in situ approach was used to identify the oxidized RNA nucleoside 8-hydroxyguanosine (8OHG) in the frontal cortex of familial Alzheimer's disease (FAD) with a mutation in presenilin-1 (PS-1) or amyloid β protein precursor (AβPP) gene (n = 13, age 47–81 years). Neurons with marked 8OHG immunoreaction in the cytoplasm were widely distributed in the superior/middle frontal gyrus of FAD. Relative intensity measurements of neuronal 8OHG immunoreactivity showed that there was a significant increase in FAD compared with controls (n = 15, age 59–81 years), while there was no difference in relative 8OHG between the PS-1 and the AβPP FAD. Interestingly, a presymptomatic case carrying a PS-1 mutation showed a considerable level of relative 8OHG, and the increased levels of neuronal 8OHG in FAD were more prominent in cases with a lower percentage area of Aβ42 burden. These results suggest that oxidative stress is an early event involved in the pathological cascade of FAD.

Elevated interleukin (IL)-1 concentrations in synovial fluid have been implicated in joint bone and cartilage destruction. Previously, we showed that IL-1β stimulated the expression of prostaglandin (PG) receptor EP4 via increased PGE2 production. However, the effect of IL-1β on osteoclast formation via chondrocytes is unclear. Therefore, we examined the effect of IL-1β and/or celecoxib on the expression of macrophage colony-stimulating factor (M-CSF), receptor activator of NF-κB ligand (RANKL), and osteoprotegerin (OPG) in human chondrocytes, and the indirect effect of IL-1β on osteoclast-like cell formation using RAW264.7 cells. OPG and RANKL expression increased with IL-1β; whereas M-CSF expression decreased. Celecoxib blocked the stimulatory effect of IL-1β. Conditioned medium from IL-1β-treated chondrocytes decreased TRAP staining in RAW264.7 cells. These results suggest that IL-1β suppresses the formation of osteoclast-like cells via increased OPG production and decreased M-CSF production in chondrocytes, and OPG production may increase through an autocrine mechanism involving celecoxib-related PGs.