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Paclitaxel frequently induces peripheral neuropathy and myelosuppression during cancer treatment. According to the National Health Insurance Research Database of Taiwan, traditional Chinese medicine doctors widely use Xiang Sha Liu Jun Zi Tang (XSLJZT) to treat breast cancer patients who have received paclitaxel. We explored the combined therapeutic effects of XSLZJT with paclitaxel. XSLJZT did not exhibit significant cytotoxic effects on P388-D1 cells; however, the combination of XSLJZT (100 and 500 mg/kg) with paclitaxel prolonged the survival rate in P388--D1 tumor-bearing mice compared to paclitaxel-only. In addition, XSLJZT was found to enhance white blood cells (WBC) counts and promote leukocyte rebound in paclitaxel-induced leukopenia in mice. XSLJZT also reduced paclitaxel-induced mechanical pain and inhibited c-Fos protein expression in the L4-6 spinal cords of Wistar rats. Moreover, paclitaxel-induced shortening of the nerve fibers of dorsal root ganglion cells was ameliorated by pretreatment with XSLJZT. Therefore, we suggest that XSLJZT could be used as an adjunct for cancer patients, as the formula could decrease paclitaxel-induced neuropathy and myelosuppression. [ABSTRACT FROM AUTHOR]

Mutations in the neurofilament light polypeptide gene (NEFL) are an uncommon cause of Charcot-Marie-Tooth disease (CMT). The aim of this study is to elucidate the clinical characteristics and genetic spectrum of NEFL -related neuropathy in a Taiwanese CMT cohort. Mutational analysis of the coding regions of NEFL was performed by Sanger sequencing or targeted resequencing. Twenty-one patients from nine CMT pedigrees, identified from a cohort of 508 unrelated CMT patients, were found to have a NEFL mutation. Genetic, clinical and electrophysiological features were analyzed. Six NEFL mutations were identified, including two novel ones (p.P8S, p.N98Y). NEFL p.E396K was the most common mutation, accounting for 33.3% of the patients in our cohort. All patients manifested sensorimotor polyneuropathy with a mean age of disease onset of 13.5 ± 9.6 (1–40) years. Their motor nerve conduction velocities (MNCVs) of the ulnar nerve ranged from 22.1 to 48.7 m/s. Seventy percent of the patients could be classified as intermediate CMT with ulnar MNCVs between 25 and 45 m/s. Six of the 21 patients (28.6%) had additional features of central nervous system (CNS) involvement, including motor developmental delay, spasticity, cerebellar signs, neuropathic pain and scoliosis. NEFL mutations account for 1.8% (9/508) of the CMT patients in Taiwan. The present study delineates the clinical and genetic characteristics of NEFL -related neuropathy in Taiwan, and highlights that ulnar MNCV above 25 m/s and CNS involvement may serve as diagnostic clues for NEFL -related neuropathy. [ABSTRACT FROM AUTHOR]

Aims: The aim of the study was to investigate the socio-demographic factors and systemic conditions associated with non-arteritic anterior ischaemic optic neuropathy (NAION). Methods: This was a nationwide population-based retrospective case-controlled study that recruited 9,261 NAION patients selected from the Taiwan National Health Insurance Research Database. The control group consisted of 9,261 age-, sex-, and index date-matched non-NAION patients recruited from the Taiwan Longitudinal Health Insurance Database, 2000. NAION was designated in the database by the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) as "code 377.41: ischaemic optic neuropathy without ICD-9-CM code 446.5: giant cell arteritis." Associated socio-demographic factors and systemic medical conditions were analysed using the McNemar's test, and continuous variables were analysed using the paired t test. The odds ratio (OR) and adjusted OR of developing NAION were compared using univariate logistic regression and multivariable logistic regression analyses, respectively. Results: Patients with systemic conditions such as diabetes mellitus, hypertension, hyperlipidaemia, chronic kidney disease, and hypotension were more likely to develop NAION than controls (adjusted OR = 1.81, 95% confidence interval [CI] = 1.67–1.97, p < 0.0001; adjusted OR = 1.46, 95% CI = 1.36–1.57, p < 0.0001; adjusted OR = 1.44, 95% CI = 1.33–1.57, p < 0.0001; adjusted OR = 3.26, 95% CI = 2.65–4.01, p < 0.0001; adjusted OR = 2.32, 95% CI = 1.31–4.10, p = 0.0039, respectively). Conclusions: NAION is strongly associated with diabetes mellitus, hypertension, hyperlipidaemia, chronic kidney disease, and hypotension. [ABSTRACT FROM AUTHOR]

Episodic weakness is typically associated with a group of disorders so called periodic paralyses. Their major causes are mutation of ion channels, and have rarely been linked to mitochondrial disorders. We report a 20-year-old man with episodic weakness and axonal sensorimotor neuropathy since the age of 10 years. Analysis of the next generation sequencing data of the entire mitochondrial genome extracted from the blood revealed a homoplasmic m.9185T > C variant in MT-ATP6. Acetazolamide may be responsive for episodic weakness, and supplements with l-carnitine with coenzyme-Q10 seem to be beneficial as well. To the best of our knowledge, this is the first report in Taiwan which reveals episodic weakness and sensorimotor polyneuropathy as a unique phenotype of MT-ATP6 mutations. [ABSTRACT FROM AUTHOR]

Aims: This study aimed to determine the trajectory of diabetic vascular diseases and to investigate the association between vascular diseases and dementia. Methods: We included adults aged ≥ 50 years with newly diagnosed type 2 diabetes (n = 173,118) from 2001 to 2005 who were followed-up until December 31, 2013 in the Taiwan's National Health Insurance Research Database. Multivariable Cox regression models were constructed to estimate hazard ratios (HRs) and confidence limits (CLs) for all-cause dementia in relation to the number, types, and occurrence patterns of vascular disease. Results: Within 1 year of diabetes diagnosis, 26.3% of adults developed their first vascular disease. During the 1,864,279 person-years of follow-up, 17,426 adults had all-cause dementia, corresponding to an incidence of 97.9 cases/10,000 person-years in 127,718 adults with at least one vascular disease and 67.5 cases/10,000 person-years in 45,400 adults without vascular diseases. Across all age groups, adults who subsequently developed a vascular disease in two one-year windows since diabetes diagnosis had the highest incidence of all-cause dementia. In comparison with adults without vascular diseases, HR for all-cause dementia was 1.99 (CL: 1.92–2.07) for those with one vascular disease only; 2.04 (CL: 1.98–2.13) for two or more vascular diseases; 3.56 (CL: 3.44–3.70) for stroke only; and 2.06 (CL: 1.99–2.14) for neuropathy alone. Similar associations were also observed with a smaller magnitude for adults with nephropathy, retinopathy, cardiovascular disease, or peripheral arterial disease. Conclusions: Patients with diabetes-related complications, particularly stroke and neuropathy, and those with rapidly developed vascular diseases appeared to have a high risk of dementia. [ABSTRACT FROM AUTHOR]

Background: To evaluate the risk of developing optic neuropathy (ON) in patient with both non-surgery and surgery-indicated chronic rhinosinusitis (CRS) via the national health insurance research database in Taiwan. Methodology/Principal findings: 44,176 Patients with a diagnostic code of CRS was selected, which included 6,678 received functional endoscopic sinus surgery (FESS) regarded as the surgery-indicated CRS. Each individual in the study group was matched to two non-CRS patients by age and gender. The outcome was set as the occurrence of ON according to the diagnostic codes occurred after the index date. Poisson regression was used to calculate the adjusted relative risk (aRR) and conditional Cox proportional model was used to estimate the adjusted hazard ratio (aHR). There were 131 and 144 events of ON occurred in the study group and the control group respectively during the follow-up period. The whole study group, whether received FESS or not, demonstrated both significant aRR and aHR compared to the control group after adjusting demographic data, prominent ocular diseases, and systemic co-morbidities. In addition, both the aRR and aHR were higher in CRS patient received FESS than those with CRS but without FESS management. Conclusion: The existence of CRS, especially the surgery-indicated CRS is a significant risk factor for the following ON using multivariable analysis. [ABSTRACT FROM AUTHOR]

Aims: The aim of the present study was to investigate the major determinants of diabetic peripheral neuropathy (DPN) in patients with type 2 diabetes (T2D), considering the traditional and newly discovered risk factors, including hypoglycaemia and glycemic variability. Methods: This retrospective case-control study was conducted in a tertiary care hospital in Taiwan. A total of 2,837 patients with T2D were recruited, medical history and biochemical data were obtained, and patients were screened for DPN using the Michigan Neuropathy Screening Instrument (MNSI). DPN was defined as an MNSI exam score > 2. A stepwise selection of variables was used based on the Akaike Information Criterion (AIC) and the Schwarz Criterion (SC). Multivariate analysis was performed using the identified variables obtained from the stepwise selection. Results: Among the recruited patients, 604 (21.3%) were found to have DPN. 275 patients with DPN were selected because of longer follow up period before enrollment and complete data of glycemic parameters, and paired with 351 patients with T2D without DPN and matched for age, gender, and diabetes duration. The results of the stepwise selection showed that the presence of moderately and severely increased albuminuria yielded the lowest values of AIC and SC, which indicate the best predictive performance. Multivariate analysis demonstrated that moderately and severely increased albuminuria and greater long-term glycemic variability significantly increased the risk of DPN, with a corresponding odds ratio of 1.85 and 1.61 (95%confidence intervals of 1.25–2.73and1.02–2.55, respectively), after adjusted for hypoglycaemia and types of diabetes treatment. Conclusions: Albuminuria is a potent predictor of DPN, and greater long-term glycemic variabilityis clearly associated with DPN in adults with T2D. These findings indicate that, in addition to achieve average blood glucose control, screening for albuminuria and reducing blood glucose fluctuations might be useful for improving diabetic microvascular complications. [ABSTRACT FROM AUTHOR]

Background: Nonarteritic anterior ischaemic optic neuropathy (NAION) is the most common acute optic neuropathy in old age. Although there are several known risk factors, the influence of obstructive sleep apnoea (OSA) has not been completely elucidated. The aim of this study was to evaluate the association between NAION and OSA. Methods: This retrospective, longitudinal cohort study used the national health insurance database of Taiwan covering the period 1996–2013. Patients without NAION at the diagnosis of OSA or who developed NAION 1 year after the diagnosis of OSA were enrolled. The patients were followed until death or the last day of the study. Cox proportional hazard regression was used to compute hazard ratios (HRs) and 95% confidence intervals (CIs) to investigate the association between OSA and NAION. Results: There were 8488 patients in the OSA group and 33 952 in the control group (without OSA), for a ratio of approximately 1:4. The percentages of NAION were 0.36% and 0.2% in the OSA and control groups, respectively, with a statistically significant difference (p < 0.01; chi‐square test), and this significant difference remained in multivariate analysis (p = 0.019) with a significantly higher HR (1.66; 95% CI: 1.08–2.55). There was significant difference in the 30–39 years age group in multivariate analysis (p < 0.01, HR: 6.30; 95% CI: 2.28–17.40). Conclusion: There was a strong association between NAION and OSA, and the patients with OSA had a higher risk of NAION. Further large‐scale, prospective studies are warranted to evaluate the effect of OSA on developing NAION. [ABSTRACT FROM AUTHOR]

Background: n-Hexane is a well-known neurotoxicant. Polyneuropathy due to occupational n-hexane exposure has been reported worldwide, however, our case is the first report in the Chinese herb industry.Case Presentation: A 25-year-old Asian man experienced progressive weakness and numbness in his hands and feet after working as an operator in a Chinese medicine pharmaceutical plant for the manufacture of Chinese herbal pain relief patches for 10 months. Electrophysiological studies indicated a reduction in nerve conduction velocity, prolongation of distal latencies, mildly positive sharp waves, and reduced recruitment with polyphasic potentials, particularly at distal sites. Demyelination with axonal degeneration caused by occupational n-hexane exposure was strongly suspected. Through investigation of our patient's workplace, the ambient n-hexane concentration in air was found to considerably exceed the permissible exposure limit/time-weighted average for n-hexane in Taiwan. His symptoms were gradually relieved after 4 months of cessation of exposure to n-hexane. He was then confirmed as a case of occupational n-hexane intoxication. Further effective control measures should be implemented as soon as possible to prevent exposure of workers to n-hexane.Conclusions: Despite a typical clinical presentation, his exposure at workplace was appropriately investigated. Chemical exposure in Chinese medicine pharmaceutical plants could be an emerging issue that may affect workers' health. The lack of knowledge and management of solvents could endanger the health of workers. This case has profound educational implications for occupational health and is worthy of further follow-up for improving hazards control. [ABSTRACT FROM AUTHOR]

Background/purpose: Chronic pain is a common and important medical problem worldwide. Patients with chronic intractable noncancer pain (CINCP) are treated primarily with narcotics. We analyzed the characteristics of patients with CINCP and the pain prescriptions of Taiwan's physicians.Methods: We enrolled 644 patients from 66 hospitals approved by the Taiwan Food and Drug Administration to use long-term narcotics for CINCP between 2003 and 2012.Results: The majority (61.8%) of patients were 40-49-year-old men who had been treated with pethidine more often than with fentanyl in the 20-49 years age group. More than 50% of CINCP patients live in northern Taiwan, and most were treated in the department of pain; the major diagnosis (men 28.9%; women 27.7%) was neuropathy. The most frequently prescribed single analgesic was morphine (52.2%); the most frequently prescribed two-drug combination was morphine plus fentanyl (50.8%). Pethidine, however, was the most frequently prescribed analgesic in the neurology (78.0%) and plastic surgery (50.0%) departments.Conclusion: To decrease malaise and addiction in patients with CINCP, Taiwan's physicians need more education on narcotic analgesics, and greater professional cooperation to develop therapeutic guidelines that will improve pain care for patients with CINCP. [ABSTRACT FROM AUTHOR]

Background: Hereditary neuromuscular diseases (NMDs) are a group of rare disorders, and the diagnosis of these diseases is a substantial burden for referral centers. Although next-generation sequencing (NGS) has identified a large number of genes associated with hereditary NMDs, the diagnostic rates still vary across centers., Methods: Patients with a suspected hereditary NMD were referred to neuromuscular specialists at the National Taiwan University Hospital. Molecular diagnoses were performed by employing a capture panel containing 194 genes associated with NMDs., Results: Among the 50 patients referred, 43 had a suspicion of myopathy, and seven had polyneuropathy. The overall diagnostic rate was 58%. Pathogenic variants in 19 genes were observed; the most frequent pathogenic variant found in this cohort (DYSF) was observed in only four patients, and 10 pathogenic variants were observed in one patient each. One case of motor neuron disease was clinically mistaken for myopathy. A positive family history increased the diagnostic rate (positive: 72.7% vs. negative: 56.3%). Fourteen patients with elevated plasma creatine kinase levels remained without a diagnosis., Conclusion: The application of NGS in this single-center study proves the great diversity of hereditary NMDs. A capture panel is essential, but high-quality clinical and laboratory evaluations of patients are also indispensable., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article., (Copyright © 2022 Formosan Medical Association. Published by Elsevier B.V. All rights reserved.)

The article discusses studies on obesity, diet, and lifestyle of patients with diabetes, published in medical journals in 2012. J. B. Dixon and colleagues identified preoperative predictors of diabetes remission after gastric bypass surgery in ethnic Chinese patients with type 2 diabetes living in Taiwan. J. W. Sacre and colleagues compared heart rate recovery after exercise to assess diabetic cardiac autonomic neuropathy.

Aim To investigate the risk factors and comorbidities associated with ethambutol-induced optic neuropathy (EON). Method Using the Taiwan Longitudinal Health Insurance Database, we conducted a study within a nationwide representative cohort of patients treated with EMB. We identified 231 patients newly diagnosed with EON between 2000 and 2008, and 924 control subjects. Adjusted OR by estimating the risk of EON in relation to comorbidities and EMB prescription protocol was determined. Results Compared with the control group, EON patients were at risk with older age, hypertension (adjusted OR=1.62, 95% CI 1.16 to 2.26) and renal diseases (without end-stage renal diseases (ESRD), adjusted OR=2.11, 95% CI 1.02 to 4.35; with ESRD, adjusted OR=3.73, 95% CI 1.79 to 7.74). Patients with an EMB prescription duration longer than 3 months were not at elevated risk compared with those whose prescription less than 3 months (OR=1.35, 95% CI 0.99 to 1.83, adjusted for age, sex, hypertension and renal diseases). Patients whose average daily dose was greater than 1200 mg, compared with the other two groups (800∼1199 mg, less than 800 mg) were not at increased risk for EON. Conclusions Age, hypertension and renal diseases are risk factors for EON in the Taiwanese population. [ABSTRACT FROM AUTHOR]

Background and purpose: To investigate the relationships of diabetic neuropathy to all-cause and diabetes-related mortality in patients with type 2 diabetes after controlling for significant correlates. Methods: We examined 326 patients diagnosed as diabetic polyneuropathy by nerve conduction study in Keelung city, Taiwan, in 2002 and followed them up to ascertain the cause and date of death until the end of 2006. The cause and date of death were recorded for the deceased patients. Information on significant correlates in association with diabetic polyneuropathy and all-cause and diabetes-related mortality was also collected. Results: With median follow-up time of 62.28 months, 44 patients with type 2 diabetes died. The cause of death related to diabetes accounted for 59% ( n = 26) of the deceased. Univariate analysis shows that the presence of diabetic neuropathy confers higher risk for all-cause mortality (hazard ratio [HR] = 4.88) and mortality from diabetes (HR = 6.58). The significant finding still persisted after adjustment for age, gender, blood pressure, smoking, history of cardiovascular/cerebrovascular disease, duration of diabetes, waist circumference, fasting plasma glucose, total cholesterol, hemoglobin, and creatinine (adjusted HR = 4.44 for all-cause death and adjusted HR = 11.82 for diabetes-related mortality, respectively). Conclusions: Diabetic polyneuropathy was an independent predictor for all-cause and diabetes-related mortality. The presence of neuropathy together with other significant prognostic factors is informative to predict all-cause death and death from diabetes-related disease for patients diagnosed as type 2 diabetes. [ABSTRACT FROM AUTHOR]

Giant cell arteritis with arteritic anterior ischemic optic neuropathy has rarely been diagnosed in Taiwan. Recently, we encountered a 76-year-old Taiwanese patient who presented with right visual impairment and marked pale swelling of his right disc. He also suffered body weight loss, general malaise and many typical manifestations of giant cell arteritis, such as jaw claudication, a tender, non-pulsating engorgement of his temporal arteries, and a highly elevated erythrocyte sedimentation rate and C-reactive protein level. Biopsy of his right superficial temporal artery revealed a granulomatous inflammation with multinucleated giant cell infiltration. This was a biopsy-proven case of giant cell arteritis with arteritic anterior ischemic optic neuropathy and indicated that although rare, this disease could occur in patients in Taiwan. [ABSTRACT FROM AUTHOR]

Hereditary optic neuropathy (HON) is a group of genetically heterogeneous diseases that cause optic nerve atrophy and lead to substantial visual impairment. HON may present with optic nerve atrophy only or in association with various systemic abnormalities. Although a genetic survey is indispensable for diagnosing HON, conventional sequencing techniques could render its diagnosis challenging. In this study, we attempted to explore the genetic background of patients with HON in Taiwan through capture-based next-generation sequencing targeting 52 HON-related genes. In total, 57 patients from 48 families were recruited, with 6 patients diagnosed as having Leber hereditary optic neuropathy through initial screening for three common variants (m.3460G>A, m.11778G>A, m.14484T>C). Disease-causing genotypes were identified in 14 (33.3%) probands, and OPA1 variants were the most prevalent cause of autosomal HON. Exposure to medications such as ethambutol could trigger an attack of autosomal dominant optic atrophy. WFS1 variants were identified in three probands with variable clinical features in our cohort. Hearing impairment could occur in patients with OPA1 or WFS1 variants. This is the first comprehensive study investigating the genetic characteristics of HON in Taiwan, especially for autosomal HON. Our results could provide useful information for clinical diagnosis and genetic counseling in this field. [ABSTRACT FROM AUTHOR]

The purpose of the current study was to evaluate the incidence of glaucoma in patients diagnosed with sensorineural hearing loss (SNHL) via the application of the National Health Insurance Research Database in Taiwan. A retrospective cohort study was conducted. Patients with a diagnosis of SNHL were enrolled in the study group after an exclusion procedure and a propensity score matched group without SNHL was served as the control group with a 1:2 ratio. The main outcome was regarded as the emergence of glaucoma diagnostic codes. Cox proportional hazard regression was applied to analyze the incidence and adjusted hazard ratio (aHR) of glaucoma in the multivariate model. A total of 15,686 patients diagnosed with SNHL were enrolled in the study group while another 31,372 non-SNHL individuals served as the control group. There were 444 glaucoma events in the study group and 647 glaucoma events in those non-SNHL individuals after the follow-up interval of 16 years. The study group demonstrated a significantly higher aHR compared to the control group after adjusting for multiple possible risk factors. In the subgroup analysis, both the normal tension glaucoma and angle closure glaucoma subgroups revealed a higher aHR in the study group. In conclusion, the patients with SNHL demonstrated a higher incidence of developing glaucoma. Moreover, the incidence was more prominent for patients diagnosed with normal tension glaucoma and angle closure glaucoma.

Background: n-Hexane is a well-known neurotoxicant. Polyneuropathy due to occupational n-hexane exposure has been reported worldwide, however, our case is the first report in the Chinese herb industry., Case Presentation: A 25-year-old Asian man experienced progressive weakness and numbness in his hands and feet after working as an operator in a Chinese medicine pharmaceutical plant for the manufacture of Chinese herbal pain relief patches for 10 months. Electrophysiological studies indicated a reduction in nerve conduction velocity, prolongation of distal latencies, mildly positive sharp waves, and reduced recruitment with polyphasic potentials, particularly at distal sites. Demyelination with axonal degeneration caused by occupational n-hexane exposure was strongly suspected. Through investigation of our patient's workplace, the ambient n-hexane concentration in air was found to considerably exceed the permissible exposure limit/time-weighted average for n-hexane in Taiwan. His symptoms were gradually relieved after 4 months of cessation of exposure to n-hexane. He was then confirmed as a case of occupational n-hexane intoxication. Further effective control measures should be implemented as soon as possible to prevent exposure of workers to n-hexane., Conclusions: Despite a typical clinical presentation, his exposure at workplace was appropriately investigated. Chemical exposure in Chinese medicine pharmaceutical plants could be an emerging issue that may affect workers' health. The lack of knowledge and management of solvents could endanger the health of workers. This case has profound educational implications for occupational health and is worthy of further follow-up for improving hazards control.