Telbivudine-Induced Myopathy: Clinical Features, Histopathological Characteristics, and Risk Factors.

Background And Purpose: Oral nucleos(t)ide analogs (NAs) are the mainstay treatment for chronic hepatitis B (CHB). Myotoxicity is an important extrahepatic effect related to NA treatment. Telbivudine is the NA for CHB that is frequently associated with muscle-related side effects.

High-fat diet reduces novelty-induced expression of activity-regulated cytoskeleton-associated protein.

Chronic high-fat-diet (HFD) consumption can lead to the development of brain insulin resistance, which then exerts deleterious effects on learning and memory. Activity-regulated cytoskeleton-associated protein (Arc) is a memory-related protein, and its expression can be induced by insulin stimulation. In HFD-fed animals, their basal Arc protein levels in cerebral cortex and hippocampus are reduced.

Factors affecting the health-related quality of life of caregivers of patients with muscular dystrophy.

Muscular dystrophy (MD) is a group of progressive muscle weakness diseases. The caregiver burden, increasing as the disease progresses, can be associated with impaired health-related quality of life (HRQOL). The aims of this study were to investigate the HRQOL in caregivers of patients with MD and identify the factors associated with HRQOL.

Quality of life of patients with Duchenne muscular dystrophy: from adolescence to young men.

Purpose: This study investigated quality of life (QOL) in adolescent and young men with Duchenne muscular dystrophy (DMD).

Methods: Health-related QOL and global QOL were assessed with the Short Form 36 (SF-36) and World Health Organization Quality of Life-BREF (WHOQOL-BREF). Associations between functional status and QOL were assessed.

The Cholinergic Signaling Responsible for the Expression of a Memory-Related Protein in Primary Rat Cortical Neurons.

Cholinergic dysfunction in the brain is closely related to cognitive impairment including memory loss. In addition to the degeneration of basal forebrain cholinergic neurons, deficits in the cholinergic receptor signaling may also play an important role. In the present study, to examine the cholinergic signaling pathways responsible for the induction of a memory-related postsynaptic protein, a cholinergic agonist carbachol was used to induce the expression of activity-regulated cytoskeleton associated protein (Arc) in primary rat cortical neurons.

The implication of integrated training program for medical history education.

Background: A full spectrum of medical education requires not only clinical skills but also humanistic qualities in the medical professionals, which can be facilitated by an integrated training program. An integrated project was created to improve one's medical intellectual and communication competence and to enable them to become docents who can perform well, as well as for development of their humanitarian nature. The aim of this study was to suggest an integrated program that provided approaches for creating positive effects in medical history education.

Di-(2-ethylhexyl)-phthalate reduces MyoD and myogenin expression and inhibits myogenic differentiation in C2C12 cells.

The purpose of this study was to investigate the effects of di-(2-ethylhexyl) phthalate (DEHP) treatment on MyoD and myogenin expression and myotube formation in the murine C2C12 cells. Myogenic differentiation is principally regulated by activities of myogenic regulatory factors, such as MyoD and myogenin, leading the elongation and fusion of mononucleated myoblasts into multinucleated myotubes. In the present study, myogenic differentiation of C2C12 cells was induced by serum deprivation with medium containing vehicle or DEHP (10, 100, 1,000 μg/ml) for 5 days.

The medicolegal issue of tissue plasminogen activator in ischemic stroke: a review of judiciary decrees in Taiwan.

Purpose: Tissue plasminogen activator (tPA) was approved by the Food and Drug Administration for ischemic stroke treatment since 1996 at the United States of America and also 2002 at Taiwan. Since after it is strongly advertised for a promising benefit to early thrombolysis that is further echoed by a recommendation in clinical guidelines from multiple medical associations in worldwide. Because of an overwhelming data of positive benefit collected in the evidence-based medicine database, legal dispute subsequently occurs when tPA is failed to be administrated in appropriate time.

Activation of Trim17 by PPARγ is involved in di(2-ethylhexyl) phthalate (DEHP)-induced apoptosis on Neuro-2a cells.

Di-(2-ethylhexyl) phthalate (DEHP) is widely used as a plasticizer in plastics. Its reproductive toxicity and teratogenic effects are well known. DEHP can cause liver damage and peroxisome proliferation, as well as carcinogenesis.

Dichotic multiple-frequency auditory steady-state responses in evaluating the hearing thresholds of occupational noise-exposed workers.

An objective, fast, and reasonably accurate assessment test that allows for easy interpretation of the responses of the hearing thresholds at all frequencies of a conventional audiogram is needed to resolve the medicolegal aspects of an occupational hearing injury. This study evaluated the use of dichotic multiple-frequency auditory steady-state responses (Mf-ASSR) to predict the hearing thresholds in workers exposed to high levels of noise. The study sample included 34 workers with noise-induced hearing impairment.

Rapid progressive course of later-onset Pompe disease in Chinese patients.

Background: Pompe disease presents with a wide variety of phenotypes ranging from a fatal disease in infancy (the infantile-onset form) to other milder later-onset forms. Currently, the clinical manifestations in Chinese patients with later-onset Pompe disease are still not well understood.

Methods: Fifteen Chinese patients who were clinically diagnosed with Pompe disease at later than one year of age at the National Taiwan University Hospital from 1993 to 2009 were included in this study.

Predicting hearing thresholds and occupational hearing loss with multiple-frequency auditory steady-state responses.

Objective: An objective investigation is needed to verify the existence and severity of hearing impairments resulting from work-related, noise-induced hearing loss in arbitration of medicolegal aspects. We investigated the accuracy of multiple-frequency auditory steady-state responses (Mf-ASSRs) between subjects with sensorineural hearing loss (SNHL) with and without occupational noise exposure.

Design: Cross-sectional study.

The protective effect of Rho-associated kinase inhibitor on aluminum-induced neurotoxicity in rat cortical neurons.

Aluminum (Al) is a neurotoxicant and is implicated in several neurodegenerative diseases, including Alzheimer's disease (AD). In AD brains, one of the pathological hallmarks is the extracellular deposition of senile plaques, which are mainly composed of aggregated amyloid-beta (Abeta). Endoproteolysis of the amyloid-beta precursor protein (AbetaPP) by the beta-secretase and the gamma-secretase generates Abeta.

Incidence of Creutzfeldt-Jakob disease in Taiwan: a prospective 10-year surveillance.

This study was performed to estimate the incidence of Creutzfeldt-Jakob Disease (CJD) in Taiwan from 1998 to 2007. Suspected cases of CJD were reported to the Taiwan Creutzfeldt-Jakob Disease Surveillance Unit, a nationwide, hospital-based case report system initiated since 1996 to prospectively conduct a CJD epidemiological study. Consecutive patients who met the diagnostic criteria recommended by the World Health Organization were enrolled.

Longitudinal study of carbon monoxide intoxication by diffusion tensor imaging with neuropsychiatric correlation.

Background: White matter damage is common after carbon monoxide (CO) intoxication, but in vivo follow-up studies about the mechanism of white matter damage are not possible in pathology series. Diffusion tensor imaging (DTI) and voxel-based morphometry (VBM) can quantify diffusion parameters and volumetric changes in white matter that can be correlated with neuropsychological performances in longitudinal studies.

Methods: We examined 9 patients with CO intoxication using DTI, VBM and neuropsychologic tests at an average of 3 and 10 months after CO exposure.

Measurement of the functional status of patients with different types of muscular dystrophy.

Muscular dystrophy (MD) comprises a group of diseases characterized by progressive muscle weakness that induces functional deterioration. Clinical management requires the use of a well-designed scale to measure patients' functional status. This study aimed to investigate the quality of the functional scales used to assess patients with different types of MD.

Damage of white matter tract correlated with neuropsychological deficits in carbon monoxide intoxication after hyperbaric oxygen therapy.

Carbon monoxide (CO) intoxication can result in cognitive deficits and demyelinating changes of the white matter (WM), for which hyperbaric-oxygen (HBO) treatment is considered effective in reducing neuropsychiatric symptoms. This study aimed to analyze cognitive functions and WM diffusion properties in CO intoxication after standard HBO treatment. Seventeen CO intoxicated patients were evaluated 4-6 months after HBO treatment.

Amyloid-beta interrupts the PI3K-Akt-mTOR signaling pathway that could be involved in brain-derived neurotrophic factor-induced Arc expression in rat cortical neurons.

The deposition of amyloid-beta (Abeta) contributes to the pathogenesis of Alzheimer's disease. Even at low levels, Abeta may interfere with various signaling cascades critical for the synaptic plasticity that underlies learning and memory. Brain-derived neurotrophic factor (BDNF) is well known to be capable of inducing the synthesis of activity-regulated cytoskeleton-associated protein (Arc), which plays a fundamental role in modulating synaptic plasticity.

Uncoupling of protein C and antithrombin III activity in cerebral ischemia patients associated with cutis marmorata.

Purpose: Cutis marmorata is a cutaneous livedoid disorder which can be differentiated from livedo reticularis in both clinical and pathological presentations. Unlike Sneddon syndrome, a detailed immunocoagulation profile has not yet been delineated for cutis marmorata in patients with cerebral ischemia.

Methods: To analyze the immunocoagulation profile in cutis marmorata patients associated with cerebral ischemia (CMCI) in a series of 135 cerebral ischemia patients.

Functional changes on ascending auditory pathway in rats caused by germanium dioxide exposure: an electrophysiological study.

The semiconductor element, germanium (Ge), is essential for the manufacture of modern integrated circuits. Because of its anti-tumor and immunomodulative effects, Ge-containing compounds are also used as health-promoting ingredients in food. However, some histological studies have shown the toxic effects of Ge-containing compounds on various organs, including the central nervous system.

Kennedy disease mimics amyotrophic lateral sclerosis: a case report.

Kennedy disease (KD) is an X-linked inherited motor neuron disease that is often accompanied by androgen insensitivity. Its estimated incidence in the US is approximately 1 case in 40,000 men. KD has also been reported in individuals of different racial backgrounds, especially in Japanese but the prevalence rate in Taiwan has not been fully investigated.

Crossed cheiro-oral syndrome.

Objectives: Cheiro-oral syndrome is characterized by sensory impairment confined to perioral area and ipsilateral fingers/hand. It results from an involvement of the ascending sensory tracts above the pons. However, a crossed pattern of perioral and acral paresthesia was rarely reported before.

Decreased muscular radionuclide uptake in Tc-99m MIBI scintigraphy during paralytic phase of thyrotoxic periodic paralysis.

The underlying pathophysiology of thyrotoxic periodic paralysis (TPP) is still obscure. From histologic surveys, vacuole formation and abundant mitochondrial abnormalities ranged from swelling, matrical pallor, pleomorphism, and reduced cristae were often disclosed in the muscle fibers during paralytic periods. In a 47-year-old man experiencing 2 episodes of transient paralysis, hyperthyroidism with TPP was diagnosed.

Statin-induced heme oxygenase-1 increases NF-kappaB activation and oxygen radical production in cultured neuronal cells exposed to lipopolysaccharide.

With potentially neuroprotective properties, heme oxygenase-1 (HO-1) has been suggested to be the main mediator of cholesterol-independent anti-inflammatory and antioxidant actions of statins. However, we had demonstrated that simvastatin-induced HO-1 increased apoptosis of Neuro 2A cells in glucose deprivation, and iron production from HO-1 activity may be responsible for the toxicity. This study was designed to explore the effect of simvastatin-induced HO-1 on cultured Neuro 2A and C6 cells exposed to lipopolysaccharide (LPS).

Loss of small fibers in entrapment neuropathy and their regeneration after surgical decompression in a rat model.

The study was carried out to investigate the loss of small fibers during entrapment neuropathy and their regeneration after surgical decompression. Entrapment neuropathy was mimicked in a rat model by wrapping the right sciatic nerve of Sprague-Dawley rats with a silastic tube of 1.3 mm inner diameter.