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Cerebral cavernous malformation (CCM) is a vascular malformation characterized by clustered enlarged capillary-like channels in the central nervous system. The genes harboring variants in patients with CCM include CCM1/Krev interaction trapped-1, CCM2/MGC4607, and CCM3/programmed cell death protein 10. We aimed to identify pathogenic variants in an ethnic Chinese population in Taiwan. We recruited 95 patients with multiple CCMs or a single lesion with a relevant family history. Sanger sequencing was performed for 41 patients. Variants were identified using sequence alignment tools, and the clinical significance of these variants was determined using American College of Medical Genetics and Genomics standards and guidelines. Several pathogenic variants were found in six patients, including three unrelated patients and three affected members of one family. Two novel pathogenic variants leading to early truncation comprised a deletion variant in exon 18 of CCM1 (c.1846delA; p.Glu617LysfsTer44) and an insertion variant in exon 4 of CCM2 (c.401_402insGCCC; p.Ile136AlafsTer4). One novel pathogenic splice site variant was c.485 + 1G > C at the beginning of intron 8 of CCM1. In this study, we identified novel variants related to CCM in an ethnically Chinese population in Taiwan.

Background Asians have higher frequency of intracranial arterial stenosis. The present study aimed to compare the clinical features and outcomes of ischemic stroke patients with and without middle cerebral artery (MCA) stenosis, assessed by transcranial sonography (TCS), based on the Taiwan Stroke Registry (TSR). Methods Patients with acute ischemic stroke or transient ischemic attack registered in the TSR, and received both carotid duplex and TCS assessment were categorized into those with stenosis (≥50%) and without (

Objectives Little is known about the longitudinal effects of training programs on family caregivers' health-related quality of life (HRQoL) and depressive symptoms over time. Therefore, the purpose of this study was to examine the effects of a home-based caregiver training program on HRQoL and depressive symptoms for family caregivers of older persons with dementia. Methods Outcomes (caregivers' HRQoL and depressive symptoms) were assessed before the training program (baseline), and at 2 weeks, 3 months, and 6 months afterwards. HRQoL was measured using the Medical Outcomes Study 36-item Short Form Survey, Taiwan version. Depressive symptoms were measured using the Chinese version Center for Epidemiologic Studies Depression Scale. Results Family caregivers who received the individualized home-based training program had better health outcomes in bodily pain (b = 12.37, p

Background— Stroke is a leading cause of death around the world. Improving the quality of stroke care is a global priority, despite the diverse healthcare economies across nations. The American Heart Association/American Stroke Association Get With the Guidelines-Stroke program (GWTG-Stroke) has improved the quality of stroke care in 790 US academic and community hospitals, with broad implications for the rest of the country. The generalizability of GWTG-Stroke across national and economic boundaries remains to be tested. The Taiwan Stroke Registry, with 30 599 stroke admissions between 2006 and 2008, was used to assess the applicability of GWTG-Stroke in Taiwan, which spends ≈1/10 of what the United States does in medical costs per new or recurrent stroke. Methods and Results— Taiwan Stroke Registry, sponsored by the Taiwan Department of Health, engages 39 academic and community hospitals and covers the entire country with 4 steps of quality control to ensure the reliability of entered data. Five GWTG-Stroke performance measures and 1 safety indicator are applicable to assess Taiwan Stroke Registry quality of stroke care. Demographic and outcome figures are comparable between GWTG-Stroke and Taiwan Stroke Registry. Two indicators (early and discharge antithrombotics) are close to GWTG-Stroke standards, while 3 other indicators (intravenous tissue plasminogen activator, anticoagulation for atrial fibrillation, lipid-lowering medication) and 1 safety indicator fall behind. Preliminary analysis shows that compliance with selected GWTG-Stroke guidelines is associated with better outcomes. Conclusions— Results suggest that GWTG-Stroke performance measures, with modification for ethnic factors, can become global standards across national and economic boundaries for assessing and improving quality of stroke care and outcomes. GWTG-Stroke can be incorporated into ongoing stroke registries across nations.

MicroRNAs (miRNAs) are a family of small non-coding RNA molecules of about 20–23 nucleotides in length, which negatively regulate protein-coding genes at post-transcriptional level. Using a stem-loop real-time-PCR method, we quantified the expression levels of 270 human miRNAs in 13 nasopharyngeal carcinoma (NPC) samples and 9 adjacent normal tissues, and identified 35 miRNAs whose expression levels were significantly altered in NPC samples. Several known oncogenic miRNAs, including miR-17-92 cluster and miR-155, are among the miRNAs upregulated in NPC. Tumour suppressive miRNAs, including miR-34 family, miR-143, and miR-145, are significantly downregulated in NPC. To explore the roles of these dysregulated miRNAs in the pathogenesis of NPC, a computational analysis was performed to predict the pathways collectively targeted by the 22 significantly downregulated miRNAs. Several biological pathways that are well characterised in cancer are significantly targeted by the downregulated miRNAs. These pathways include TGF-Wnt pathways, G1-S cell cycle progression, VEGF signalling pathway, apoptosis and survival pathways, and IP3 signalling pathways. Expression levels of several predicted target genes in G1-S progression and VEGF signalling pathways were elevated in NPC tissues and showed inverse correlation with the down-modulated miRNAs. These results indicate that these downregulated miRNAs coordinately regulate several oncogenic pathways in NPC.

BackgroundSpontaneous deep intracerebral hemorrhage (SDICH) is a devastating stroke subtype. The causes of SDICH are heterogeneous. Matrix metalloproteinase-9 (MMP-9, Gelantinase B) has been shown to relate to stroke and the development of aneurysm and may increase risks of intracerebral hemorrhage. MMP activities are modulated by their endogenous inhibitors, tissue inhibitors of metalloproteinases (TIMPs). We analyzed the genetic variants of MMP-9 and TIMP-1 and SDICH susceptibility.MethodsAssociations were tested by logistic regression or general linear models with adjusting for multiple covariables. Multiplicative terms between genes were applied to detect the interaction effects on SDICH. Permutation testing of 1,000 replicates was performed for empirical estimates.ResultsIn the group of ≥65 years old (y/o), we found associations of SDICH with rs3787268 (Odds ratio [OR] = 0.48, 95% confidence interval [CI] 0.27 to 0.86, P = 0.01) and haplotype1 (Hap1) (OR = 0.48, 95% CI 0.26 to 0.86, P = 0.014). For TIMP1 gene, rs4898 was associated with SDICH in the elder male group (OR = 0.35, 95% CI 0.15 to 0.81, P = 0.015). In contrast, in the younger male group, there were associations of SDICH with rs2250889 (OR = 0.48, 95% CI 0.27 to 0.84, P = 0.01) and Hap3 (OR = 0.61, 95% CI 0.38 to 0.97, P = 0.04). We found significant genetic interaction between TIMP-1 and MMP-9 in SDICH susceptibility among younger male subjects (P = 0.004). In subjects carrying rs4898 minor allele, carriers with Hap3 had lower SDICH risk than non-carriers (OR = 0.19, 95% CI 0.07 to 0.51, P = 0.001). In addition, this study showed that when young males were exposed to alcohol, Hap3 was a protective factor of SDICH (OR = 0.06, 95% CI 0.01 to 0.27, P = 0.0002). In contrast, when they were exposed to smoke, Hap2 carriers had increased risk of SDICH (OR = 2.45, 95% CI 1.05 to 5.73, P = 0.04).ConclusionsThis study showed modest to moderate effects of MMP-9 and TIMP-1 polymorphisms on SDICH risks with significant age differences. MMP-9 may interact with alcohol to play a role in the SDICH risk in young men.

BACKGROUND/PURPOSE Although a deficit of semantic memory is evident in the dementia of the Alzheimer's type (DAT), the underlying neuropsychologic mechanism remains controversial. Breakdown of the semantic network during the course of DAT and an inability to access semantic information have been postulated as possible explanations, but supporting data are limited, particularly in low-educated patients. This study examined semantic memory in low-educated patients with different degrees of dementia severity. METHODS In total, 197 adult subjects were recruited, including 165 DAT patients and 32 normal controls. Subjects were divided into four subgroups according to their dementia severity. All subjects completed an episodic memory task, the Six-Object Memory Test, and semantic memory tasks including the Object Naming Test, the Remote Memory Test and the Semantic Association of Verbal Fluency Test. One-way ANOVA and ANCOVA with a post hoc Scheffe's procedure were used to evaluate differences between groups. RESULTS All patients, irrespective of the degree of dementia, showed impaired performance on the Six-Object Memory Test [F (4, 163) = 69.95, p < 0.0001 for immediate recall; F (4, 163) = 41.34, p < 0.0001 for delayed recall]. On the semantic memory tasks, patients with moderate to severe dementia showed impaired performances on the Object Naming Test [F (4, 180) = 28.25, p < 0.0001] and the Remote Memory Test [F (4, 167) = 26.22, p < 0.0001 for recall; F (4, 167) = 34.80, p < 0.0001 for recognition], while all patients performed defectively on the Semantic Association of Verbal Fluency Test [F (4, 194) = 70.43, p < 0.0001]. CONCLUSION Our results thus partially support the hypotheses that a loss of semantic structure and an inability to access semantic knowledge occur in the pathogenesis of DAT.

Background and Purpose: Ischemic stroke often co-exists with peripheral arterial disease (PAD). Patients with polyvascular diseases carry higher risks of vascular events and death. We initiated a study to evaluate the safety and efficacy of cilostazol in ischemic Stroke patients with PAD (SPAD) and have been taking aspirin for stroke prevention. Methods: The SPAD study is a prospective, multicenter, national, double-blinded, placebo-controlled, randomized trial. Patients with previous ischemic stroke or TIA who have been taking aspirin (100 mg per day), aged 50 years or older, with PAD in the lower limbs based on ankle-brachial index (ABI) Results: From September, 2010 to July, 2012, a total of 800 patients (male, 59.9%; female, 40.1%; average age, 70.4±9.3 years) were enrolled in the SPAD study, including 722 (90.2%) with ischemic stroke or 78 (9.8%) with TIA. Several atherosclerotic risk factors were commonly seen in this study population, including hypertension (88%), diabetes mellitus (48%), hypercholesterolemia (54%), smoking habit (41%), and carotid stenosis (15%). The distribution of baseline ABI were 0.90-0.99 (n=294), 0.70-0.89 (n=371), and Conclusions: The SPAD trial is the first study to evaluate the safety and efficacy of dual antiplatelets, aspirin plus cilostazol, in ischemic stroke patients with PAD. The trial findings are expected to help in choosing better strategy for prevention of vascular events in this polyvascular disease.

Bacterial meningitis, a world-wide disease, has to be reviewed periodically because the specific micro-organisms responsible for the infection vary with time, geography and patient age. To determine its incidence and clinical features in Taiwan, we reviewed the microbiological records for cerebrospinal fluid (CSF) and blood cultures, and the medical records of patients with bacterial meningitis admitted between 1981 and mid-1998. Bacterial micro-organisms were demonstrated in the CSF and/or blood in 395 patients with 418 episodes of bacterial meningitis. Streptococcus species were the most common causative micro-organism group, at 23. 21% of all episodes. Its prevalence rate significantly decreased from the first 7 years of study (41.9%) to the last 10.5 years (19. 2%). However, Klebsiella meningitis and Staphylococcal meningitis were more frequently noted after 1987. More than 70% of patients had at least one underlying disease or condition. Poor prognostic factors indicated by univariable analysis were: age >60 years; diabetes mellitus; severe neurological deficits on the first day of treatment; infection with Gram-negative bacilli; CSF WBC count >5000x10(6)/l; malignancy; seizure; and bacteraemia. The overall mortality rate was 29.4%, 29.7% in the first 7 years of study and 29. 4% in the last 10.5 years. The use of new antibiotics has not reduced the mortality rate in our patients with bacterial meningitis.

Background and Purpose —In vitro studies have shown that nerve growth factor (NGF) is protective to cortical neurons against various insults. However, the role of NGF in relation to its high-affinity trkA receptor in the cortical neurons has not been well discussed. In this experiment, we studied the possible involvement of the NGF/receptor system in the ischemic injury of cortical neurons after focal cerebral ischemia in rats. Methods —Male Wistar rats received right middle cerebral artery occlusion of 90 minutes’ duration. The rats were decapitated at different reperfusion time points: hour 4 and days 1, 3, 7, and 14 of recirculation. Brain sections at the level of striatum were immunostained against NGF, trkA, glial fibrillary acidic protein (GFAP), and stress protein HSP70. Double immunostaining against NGF and GFAP was also performed. Optical density of NGF immunoreactivity in the ischemic and nonischemic cortexes was compared between sham-control and ischemic animals. Results —In the sham-control rats, NGF immunoreactivity was present in the cortical and striatal neurons. However, beginning at hour 4 after recirculation, there was a significant decrease of NGF in the ischemic cortex and striatum. Beginning at day 1, NGF was absent completely in the infarcted striatum and cortex. However, in the peri-infarct penumbra area, despite a decrease in NGF at hour 4 and day 1, NGF recovered beginning at day 3 and returned almost to the sham-control level at day 14. In the nonischemic cortex, NGF increased beginning at hour 4, peaked at day 7, and returned almost to the sham-control level at day 14. The trkA and HSP70 immunoreactivities were not present in the sham-control cortex. However, trkA was induced at hour 4 in the ischemic cortex and at days 1 and 3 in the peri-infarct penumbra cortex. The HSP70 was induced at days 1 and 3 in the peri-infarct penumbra area. Double immunostaining showed that the number of GFAP-positive cells increased gradually, and NGF immunoreactivity in the GFAP-positive cells became gradually intense after ischemia. Conclusions —Our study demonstrated a temporal profile of NGF and trkA in the ischemic cortex and NGF expression by reactive astrocytes. Our data suggest that the NGF/receptor system may play a role in the astrocyte/neuron interaction under certain pathological conditions, such as focal cerebral ischemia.

OBJECTIVE To identify clinical characteristics of various forms of Guillain-Barre syndrome in Taiwan. METHODS The clinical and electrophysiological data of 167 consecutive patients with Guillain-Barre syndrome admitted to Chang Gung Memorial Hospital, a general paediatric and adult hospital in Taiwan, were reviewed. RESULTS Analysis of age distribution disclosed a high incidence (21%) among patients under the age of 10 years. Seasonal preponderance in Spring (March to May) was found. Utilising clinical and electrophysiological data, these 167 patients with Guillain-Barre syndrome were subclassified; 82 (49%) had acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 32 (19%) had Fisher syndrome (FS), and six (4%) had axonal forms of Guillain-Barre syndrome. The remaining 47 (28%) patients were unclassified. Patients with AIDP and FS had many common clinical features, including seasonal distribution, history of preceding illness, sensory abnormalities, cranial nerve involvement except for extraocular motor nerves, and albuminocytological dissociation on examination of CSF. Follow up study on 145 patients disclosed that 127 (87%) recovered satisfactorily, 14 (10%) were persistently disabled, and four (3%) died during admission to hospital. Clinical features associated with poor outcome (persistent disability or death) were requirement for mechanical ventilation, a low mean compound muscle action potential amplitude (⩽ 10% of the lower limit of normal), and age greater than 40 years. CONCLUSION Guillain-Barre syndrome in Taiwan showed a peculiar age and seasonal distribution and a high frequency of FS not seen in other series. Given that patients with AIDP and FS had many common clinical features, AIDP and FS may have similar underlying pathological mechanisms.

Although the concept of brain death has been widely accepted, the criteria required for making the diagnosis remain controversial. This prospective study was undertaken to examine the reliability of a set of clinical criteria adopted in Taiwan. One hundred and forty deeply comatose patients (101 men, 39 women; mean age 49.5 (SD 17.6) years) requiring ventilation were studied. Seventy three patients met the clinical criteria for brainstem death; all developed cardiac asystole (97% within seven days) despite continued full cardiorespiratory support. Brainstem death was diagnosed in only two of the 21 patients with hypoxic or ischaemic insults. This stresses the rarity of hypoxic or ischaemic encephalopathy as a cause of brainstem death. The results show that if strict attention is paid to preconditions and exclusions, brainstem death can be reliably diagnosed on clinical grounds alone.

Quantitative cardiovascular autonomic function tests were performed longitudinally in nine patients with Fisher syndrome (FS). Parasympathetic autonomic function was evaluated by the Valsalva ratio and RR interval variation during rest and deep breathing. Sympathetic autonomic function was evaluate by blood pressure responses to sustained handgrip and to active standing. None of the patients with FS had clinical signs of autonomic dysfunction during the course of their illness. However, autonomic function abnormalities were seen in up to 83% of patients with FS. Thus, bedside clinical signs of autonomic dysfunctions are inadequate for the assessment of autonomic abnormality compared with quantitative autonomic function examination. Most autonomic function tests tended to improve after 4-12 weeks. Although parasympathetic and sympathetic functions were both involved at the height of FS, parasympathetic fibres were less vulnerable than the sympathetic fibres.

and life satisfaction both showed significant effects of time (ps < 0.05). In general, the caregivers reported declines in depressive symptoms and increasing life satisfaction overtime. Conclusions: We found support for improvements in the mental and emotional health of caregivers of dementia patients. The death of their loved one undoubtedly resulted in a period of loss and grief, yet it is possible the ensuing lifestyle offers increased wellbeing without caregiving responsibilities. Further implications for this research will be considered.

Background: There is a lack of research exploring the influence of the role implementation process of family caregivers of older people with dementia in Taiwan. Purpose: The purpose of this study was to investigate the predictive ability on positive and negative caregiving outcomes of several role implementation variables, including mutuality, preparedness, and balance between competing needs. Methods: A cross-sectional, correlational study design was used. Data were collected from family caregivers of patients with dementia from neurological clinics at a medical center and at local hospitals through a take-home mail survey. One hundred seventy-six family caregivers of patients with dementia completed the Family Caregiving Inventory. Results: After controlling for age and gender of the family caregiver and the cognitive function of the elderly patients, mutuality was found to associate negatively with role strain and depressive symptoms and positively with rewards and mental health. Higher preparedness was associated with higher caregiving rewards and better mental health. Less balance was associated with more severe depressive symptoms and poorer mental health. Conclusions/Implications for Practice: Greater attention, support, and consultation should be directed at caregivers with low mutuality, less preparedness, and less balance between competing needs. Specifically, family caregivers with low mutuality are at risk of higher role strain and more depressive symptoms. Those in such a category should be identified and should receive intervention as early as possible. Interventions to enhance family caregiver preparedness should be developed to increase caregiving rewards and to improve caregiver mental health.

Background and object There was no Chinese questionnaire to evaluate the health-related quality of life (HRQoL) in patients with hemifacial spasm (HFS). In this study, we aimed to validate a new disease-specific HRQoL scale for HFS (HFS-36) in Chinese version, and compared it to SF-36, a generic HRQoL scale. Patients and Methods The HFS-36 Chinese version was modified from English version of HFS-30, including subscales of mobility, activities of daily living (ADL), emotional well-being, stigma, social support, cognition, bodily discomfort, and communication. All the items were scored on the 5-point scales, ranging from 0(never) to 4(always). Patients with HFS were asked to answer HFS-36 and SF-36 questionnaires on the same day before and 6-8 weeks after Botulinum toxin (BTX) injections, respectively. The reliability and validity of HFS-36 scale were evaluated statistically. Results Totally, 103 patients (68 females; 35 males) were recruited in this study, with a mean age of 57.6 ± 11.5 years and a mean duration of HFS for 7.6 ± 5.8 years. The intra-class correlation (ICC) and Cronbach's α were over 0.7 in the majority of items. HFS-36 showed a good correlation to HFS severity before BTX treatment and a significant improvement of subscale scoring after BTX treatment. HFS-36 also had a significant correlation to the mental health of SF-36. Conclusions The Chinese version of HFS-36 demonstrated a good reliability and validity in subscales of motility, ADL, emotion well-being, stigma and bodily discomfort. The HRQoL was significantly improved after BTX treatment assessed by HFS-36 or SF-36. Compared to SF-36, HFS-36 scale was more sensitive and specific to evaluate the HRQoL in HFS.

Elevated serum homocysteine levels have been associated with the development of Alzheimer's dementia (AD). The combined use of a mecobalamin capsule preparation, which contains vitamin B12 0.5 mg with an active methyl base, and an over-the-counter nutritional supplement that contains folic acid 1 mg and pyridoxine hyperchloride 5 mg may be effective as a homocysteine-lowering vitamin regimen.The aim of this study was to determine whether oral multivitamin supplementation containing vitamins B6 and B12 and folic acid would improve cognitive function and reduce serum homocysteine levels in patients with mild to moderate AD.This randomized, double-blind, placebocontrolled trial was conducted at En Chu Kong Hospital, Taipei, Taiwan. Male and female patients aged50 years with mild to moderate AD and normal folic acid and vitamin B12 concentrations were enrolled. All patients received treatment with an acetylcholinesterase inhibitor and were randomized to receive add-on mecobalamin (B12) 500 mg + multivitamin supplement, or placebos, PO QD for 26 weeks. The multivitamin contained pyridoxine (B6) 5 mg, folic acid 1 mg, and other vitamins and iron. Serum homocysteine level was measured and cognitive tests were conducted at baseline and after 26 weeks. The primary efficacy outcome was change in cognition, measured as the change in score from baseline to week 26 on the Alzheimer's Disease Assessment Scale 11-item Cognition subscale. Secondary efficacy outcomes included changes in function in performance of activities of daily living (ADLs) and concentrations of homocysteine, B12, and folic acid. Tolerability was assessed by comparing the 2 study groups with respect to physical examination findings, including changes in vital signs, laboratory test abnormalities, concomitant medication use, and compliance of study medication was assessed using an interview with the patient's caregiver, as well as the monitoring of adverse events (AEs) throughout the study.Eighty-nine patients (45 men, 44 women; all Taiwanese; mean [SD] age, 75 [7.3] years) were enrolled and randomized. Overall, there were no significant differences in cognition or ADL function scores between the 2 groups. At week 26, the mean (SD) between-group difference in serum homocysteine concentration versus placebo was -2.25 (2.85) micromol/L (P = 0.008), and the mean serum concentrations of vitamin B12 and folic acid were significantly higher (but within normal range) in the multivitamin group compared with placebo (., +536.9 [694.4] pg/mL [P0.001] and +13.84 ng/mL [11.17] [P = 0.012] at 26 weeks, respectively). The 2 most common AEs were muscle pain (11.1% and 6.8%) and insomnia (8.9% and 9.1%) in the multivitamin and placebo groups, respectively.In this population of patients with mild to moderate AD in Taiwan, a multivitamin supplement containing vitamins B(6) and B(12) and folic acid for 26 weeks decreased homocysteine concentrations. No statistically significant beneficial effects on cognition or ADL function were found between multivitamin and placebo at 26 weeks.

Background: Prior studies suggested that protein kinase Cη ( PRKCH ) 1425G/A polymorphism was associated with lacunar infarction. This study examined whether the association was independent of traditional risk factors in each of the stroke subtypes. Methods: This study included 206 ischemic stroke patients and 337 controls. Multivariable logistic regression was used for analyses. Co-variables of age, sex, hypertension, diabetes mellitus (DM), and smoking were included to delineate independency of associations. Result: PRKCH 1425G/A was associated with ischemic stroke [odds ratio (OR) =1.5, 95% confidence interval (CI): 1.1-2.2, p = 0.024] when adjusted for age and sex. However, the significance of association became borderline when adjusted for co-variables (OR = 1.5, 95% CI: 1.004-2.3, p = 0.048). Of the infarction subtypes, PRKCH 1425G/A was associated with lacunar infarction (OR = 1.8, 95% CI: 1.1-2.9, p = 0.025), which remained significant when adjusted for co-variables (OR = 2.0, 95% CI: 1.1-3.5, p = 0.015). No association was found between the polymorphism and the other infarction subtypes. When stratified by age group, the magnitude of significance became stronger in patients >65 years old. Specifically, PRKCH 1425G/A was significantly associated with ischemic stroke in patients older than 65 years, when adjusted for all co-variables (OR = 2.0, 95% CI: 1.05-3.8, p = 0.036). Still, in patients older than 65 years, the association was only observed in lacunar infarction when adjusted for all co-variables (OR = 4.2, 95% CI: 1.7-10, p = 0.001). No association of PRKCH 1425G/A with stroke and any of the subtypes was identified in patients >65 years old. Conclusion: The association between PRKCH 1425G/A and lacunar infarction was independent of traditional stroke risk factors. PRKCH 1425G/A in stroke susceptibility differed between infarction subtypes and age groups.

Huei-Ling Huang,1 Yea-Ing L Shyu,2,3 Min-Chi Chen,4 Chin-Chang Huang,5,6 Hung-Chou Kuo,5,6 Sien-Tsong Chen,5,6 Wen-Chuin Hsu6,71Department of Gerontological Care and Management, Chang Gung University of Science and Technology, Taoyuan, Taiwan; 2School of Nursing, College of Medicine, Chang Gung University, Taoyuan, Taiwan; 3Healthy Aging Research Center, Chang Gung University, Taoyuan, Taiwan; 4Department of Public Health and Biostatistics Consulting Center, Chang Gung University, Taoyuan, Taiwan; 5Department of Neurology, Chang Gung Memorial Hospital, Taoyuan, Taiwan; 6School of Medicine, Chang Gung University, Taoyuan, Taiwan; 7Chang Gung Dementia Center, Taoyuan, TaiwanPurpose: The purpose of this study was to explore family caregivers’ role-implementation experiences at different stages of dementia.Patients and methods: For this cross-sectional, exploratory study, 176 dyads of family caregivers and their community-dwelling elderly relatives with dementia were recruited from the neurological clinics of a medical center in Taiwan. The Family Caregiving Inventory was used to assess family caregivers for caregiving activities, role strain, role preparation, and help from others at different stages of care receivers’ dementia.Results: Family caregivers’ caregiving activities were related to patients’ stages of dementia. For patients with mild dementia, caregivers provided more assistance in transportation and housekeeping. In addition to these two activities, family caregivers of patients with moderate dementia provided more assistance with mobility and protection. For patients with severe dementia, family caregivers provided more assistance with personal care, mobility and protection, transportation, and housekeeping. Overall, family caregivers reported having some preparation to provide care; the most difficult caregiving activity was identified as managing behavioral problems.Conclusion: This study’s results provide a knowledge base for designing dementia stage-specific interventions in clinical practice and developing community-based, long-term care systems for families of patients with dementia.Keywords: behavioral problem, illness trajectory, role strain, caregiving activity, prepar­edness

Background Miyoshi distal myopathy (MM) and limb girdle muscular dystrophy type 2B (LGMD2B) were found to map to the same mutant gene encoding for dysferlin on chromosome 2p13. Most reported cases were large inbred kindreds whose members demonstrated both MM and LGMD2B phenotypes. Objective To investigate the clinical, neurophysiological, histopathological, and genetic features in 4 patients with MM from 2 unrelated Chinese families demonstrating linkage to the dysferlin locus. Results All patients were characterized by early adult onset, preferential atrophy, and weakness of calf muscles, marked elevation of serum creatine kinase levels, and absence of dysferlin staining. Magnetic resonance imaging showed fatty and fibrotic tissue signals in the affected muscles. Genetic analysis revealed novel compound heterozygous mutations, 1310+1G to A and GGG to GTC transition at nucleotide 1650 ( G426V ) in one family and another novel compound heterozygous mutation, a deletion of C at nucleotide 477 and a CCG to CTG transition at nucleotide 6576 (P2068L), in the other family. Conclusion Miyoshi distal myopathy in these 2 Chinese families demonstrated a homogenous phenotype and compound heterozygous mutations. Among the 4 mutations, 3 were novel mutations that, to our knowledge, have not been reported previously.

An 18 year old man with congenital basilar invagination developed multiple lower cranial nerve (CN) palsies including CN IX to XII after a traffic accident. Computed tomography of his skull base revealed a two part atlas Jefferson fracture. Normally, lower cranial nerves (CN IX-XII) pass through a space between the styloid process and the atlas transverse process. Atlas burst fractures rarely cause neurological deficits because of a greater transverse and sagittal diameter of the spinal canal at the atlas, and a tendency of the lateral masses to slide away from the cord after injury. However, when associated with a rare condition-congenital basilar invagination-atlas fractures can compromise the space and make CN IX-XII more vulnerable to compression injury. This report discusses the correlation between the anatomical lesions and clinical features of this patient.

Background and Purpose — The etiologic mechanisms of young ischemic stroke in Chinese are largely unknown. This work thus studied the etiologies of young ischemic stroke in Taiwan Chinese and made a comparison with previous reports. Methods — From January 1997 to October 2001, a total of 264 consecutive young ischemic stroke patients (18 to 45 years old) were admitted to the Department of Neurology in our hospital. The risk factors for stroke and the distribution of stroke subtype were studied. The vascular ultrasound and angiographic findings of these patients were also studied. Results — The sample contained 188 men and 76 women. Cerebral infarction was diagnosed in 241 patients and transient ischemic attack in 23 (8.7%). Regarding stroke subtype, stroke of small-vessel occlusion was diagnosed in 20.5% of cases, large-artery atherosclerosis in 7.2%, cardioembolism in 17.8%, other determined etiology in 22.3%, and undetermined etiology in 23.5%. The 4 most common risk factors were hyperlipidemia (53.1%), smoking (49.8%), hypertension (45.8%), and family history of stroke (29.3%). Twenty-three patients (9.6%) had significant stenosis (≥50%) of the carotid (7.5%) and vertebral arteries (2.1%), the most common cause of which was dissection (60.9%). Forty-five patients (26.5%) had significant intracranial stenosis with 18.8% in the carotid and 10.6% in the vertebrobasilar system, and 5 (2.9%) had stenosis in both systems. Premature atherosclerosis (33.3%) was the most common cause of intracranial stenosis. Conclusions — Our study found that strokes of other determined etiology and undetermined etiology were most common among the sample group, and a battery of extensive examinations is indicated to elucidate the etiology for further stroke prevention. Intracranial stenosis is more common than extracranial stenosis in both the carotid and vertebrobasilar systems.

The aim of this study was to examine the impact of radiation treatment on olfactory function in patients with nasopharyngeal carcinoma (NPC). An olfactory function test battery was administered to 25 adult NPC patients having received radiotherapy, 24 adult nasopharyngeal carcinoma patients awaiting to receive radiation treatment, and 36 adult normal control subjects. Members of the three groups were matched in terms of age, educational level, and full-scaled IQ score. Comparing the test results revealed that the NPC patients with radiotherapy had olfactory information processing impairments including absolute threshold, odour-tactile cross-modality matching, verbal identification of odours, and recall and recognition of identity of odours. The deficits of suprathreshold olfactory functioning in these patients did not seem to arise from impaired absolute threshold sensitivity. Provided that the results are reproducible, an evaluation of olfactory functioning in NPC patients during the period of radiotherapy may be useful for detecting or even avoiding side effects of radiation.

Klebsiella infection has been considered to be an uncommon cause of meningitis. To determine its incidence and clinical features, we reviewed the microbiologic records of cerebrospinal fluid (CSF) and blood cultures and the medical records of patients with bacterial meningitis admitted between 1981 and 1995. Klebsiella meningitis was diagnosed in 79 patients with 83 episodes. All patients had klebsiella isolated from CSF and/or blood and typical symptoms and signs of acute bacterial meningitis. Of these, 74 were over 16 years of age and 2 of the 5 children were infants. There was an increased prevalence rate of klebsiella meningitis after 1986. Of the 83 episodes, only 9 occurred between 1981 and 1986, accounting for 7·8% of 115 cases with CSF and/or blood culture-proven acute bacterial meningitis, whereas in 1987–95, there were 74 episodes accounting for 17·7% of 419 bacteriologically proven cases. K. pneumoniae accounted for 69 episodes, K. oxytoca, 11 episodes and K. ozaenae, 3 episodes. Male gender, diabetes mellitus and liver cirrhosis were commonly associated with K. pneumoniae meningitis. Neurosurgical procedures were frequently associated with K. oxytoca meningitis. All three patients with K. ozaenae meningitis had a primary disease of the nasopharyngeal pathway. The mortality rate due to K. pneumoniae was 48·5%, K. oxytoca, 10% and K. ozaenae, 0%. In patients with K. pneumoniae meningitis, poor prognostic factors included age over 60 years, diabetes mellitus, bacteremia and severe neurological deficits on the first day of treatment.

Background Systemic infection is a common complication of stroke. However, brain abscess as a complication of stroke has never been reported. Case Descriptions We report two patients, one with a hypertensive intracerebral hemorrhage and the other with nonseptic cerebral infarction. In both patients, brain abscess developed at the stroke lesion site after an infectious complication. After surgical aspiration and antibiotic treatment, one patient recovered and the other died. Conclusions These two cases demonstrate that brain abscess may occur in a previous hemorrhage or infarction area as a complication of systemic infection. Recognition of the risk is important for early diagnosis and proper treatment of this potentially fatal complication of stroke.

Background Genetic factors may play a role in susceptibility to spontaneous deep intracerebral hemorrhage (SDICH). Previous studies have shown that TNF-α gene variation was associated with risks of subarachnoid hemorrhage in multiple ethnicities. The present case-control study tested the hypothesis that genetic variations of the TNF-α gene may affect the risk of Taiwanese SDICH. We examined the association of SDICH risks with four single nucleotide polymorphisms (SNPs) within the TNF-α gene promoter, namely T-1031C, C-863A, C-857T, and G-308A. Methods Genotyping was determined by PCR-based restriction and electrophoresis assay for 260 SDICH patients and 368 controls. Associations were tested by logistic regression or general linear models with adjusting for multiple covariables in each gender group, and then in combined. Multiplicative terms of gender and each of the four SNPs were applied to detect the interaction effects on SDICH risks. To account for the multiple testing, permutation testing of 1,000 replicates was performed for empirical estimates. Results In an additive model, SDICH risks were positively associated with the minor alleles -1031C and -308A in men (OR = 1.9, 95% CI 1.1 to 3.4, p = 0.03 and OR = 2.6, 95% CI 1.3 to 5.3, p = 0.005, respectively) but inversely associated with -863A in females (OR = 0.5, 95% CI 0.2 to 0.9, p = 0.03). There were significant interaction effects between gender and SNP on SDICH risks regarding SNPs T-1031C, C-863A, and G-308A (p = 0.005, 0.005, and 0.007, respectively). Hemorrhage size was inversely associated with -857T in males (p = 0.04). Conclusions In the Taiwan population, the associations of genetic variations in the TNF-α gene promoter with SDICH risks are gender-dependent.