To learn more about the needs and experiences of young carers for patients of frontotemporal dementia (FTD) in order to create a relevant support website for young caregivers to dementia patients.

Two focus groups were held with a total of fourteen young carers aged 11-18. The data corpus was collected through a semi-structured interview facilitated by a medical journalist who had prior experience as a caregiver to a patient with FTD. The transcripts were narrowed to a dataset for descriptive analysis using a coding scheme to reveal the main themes of their responses.

Seven overlapping theme areas were: emotional impact of living with a parent with FTD, caregiving, coping, symptoms, diagnosis, relationships, and support. Based on the participants' responses, a website was launched providing supportive information and counsel for young carers.

Young carers saw the experience of caring for a parent with early-onset dementia as positive overall, but identified opportunities for professionals to assist them in overcoming stigma and the challenge of balancing childhood and adolescent development within this context.

Reflex assessment, an essential element in the investigation of the motor system, is currently assessed through qualitative description, which lacks of normal values in the healthy population. This study quantified the amplitude and latency of patellar tendon reflex in normal subjects using motion analysis to determine the factors affecting the reflex amplitude.

100 healthy volunteers were recruited for patellar tendon reflex assessments which were recorded using a motion analysis system. Different levels of input strength were exerted during the experiments.

A linear relationship was found between reflex input and reflex amplitude (r = 0.50, P <0.001). The left knee was found to exhibit 26.3% higher reflex amplitude than the right (P <0.001). The Jendrassik manoeuvre significantly increased reflex amplitude by 34.3% (P = 0.001); the effect was especially prominent in subjects with weak reflex response. Reflex latency normality data were established, which showed a gradual reduction with increasing input strength.

The quantitative normality data and findings showed that the present method has great potential to objectively quantify deep tendon reflexes.

Determining factors predictive of the natural risk of rupture of cerebral aneurysms is difficult. We studied morphological factors associated with rupture in a study model of patients with mirror location intracranial aneurysms, one aneurysm that had ruptured and one that had not, each patient served as their own control attempting to eliminate confounding variables.

We collected five one-dimensional measurements and four two-dimensional indices from three-dimensional rotational digital subtraction angiography images of patients in the proposed study model and explored their correlation with aneurysm rupture. Parameters were analyzed with a paired Student's t test for significance and significant parameters were further examined by multivariate conditional logistic regression analysis.

Fifty-two patients with 52 pairs of intracranial aneurysms in a mirror location were studied. The maximum perpendicular height, neck diameter, maximum width, maximum height, aspect ratio, size ratio, and bottleneck factor were significantly associated with ruptured aneurysms on bivariate analysis. A logistic regression analysis showed that only size ratio, which was defined as the ratio of the maximal height to parent artery average diameter, is independently correlated with ruptured intracranial aneurysms.

In a case-control study of patients with mirror location intracranial aneurysms, size ratio was identified as the unique morphological factor associate with the rupture of cerebral aneurysms.

the maternally inherited MTTL1 A3243G mutation in the mitochondrial genome causes MelaS (Mitochondrial encephalopathy lactic acidosis with Stroke-like episodes), a condition that is multisystemic but affects primarily the nervous system. Significant intra-familial variation in phenotype and severity of disease is well recognized.

retrospective and ongoing study of an extended family carrying the MTTL1 A3243G mutation with multiple symptomatic individuals. tissue heteroplasmy is reviewed based on the clinical presentations, imaging studies, laboratory findings in affected individuals and pathological material obtained at autopsy in two of the family members.

there were seven affected individuals out of thirteen members in this three generation family who each carried the MTTL1 A3243G mutation. the clinical presentations were varied with symptoms ranging from hearing loss, migraines, dementia, seizures, diabetes, visual manifestations, and stroke like episodes. three of the family members are deceased from MelaS or to complications related to MelaS.

the results of the clinical, pathological and radiological findings in this family provide strong support to the current concepts of maternal inheritance, tissue heteroplasmy and molecular pathogenesis in MelaS. neurologists (both adult and paediatric) are the most likely to encounter patients with MelaS in their practice. genetic counselling is complex in view of maternal inheritance and heteroplasmy. newer therapeutic options such as arginine are being used for acute and preventative management of stroke like episodes.

Seizures while driving are a well known occurrence in established epilepsy and have significant impact on driving privileges. There is no data available on patients who experience their first (diagnosed) seizure while driving (FSWD).

Out of 311 patients presenting to the Halifax First Seizure Clinic between 2008 and 2011, 158 patients met the criteria of a first seizure (FS) or drug-naïve, newly diagnosed epilepsy (NDE). A retrospective chart review was conducted. FSWD was evaluated for 1) prevalence, 2) clinical presentation, 3) coping strategies, and 4) length of time driving before seizure occurrence.

The prevalence of FSWD was 8.2%. All 13 patients experienced impaired consciousness. Eleven patients had generalized tonic-clonic seizures, one starting with a déjà-vu evolving to visual aura and a complex partial seizure; three directly from visual auras. Two patients had complex partial seizures, one starting with an autonomic seizure. In response to their seizure, patients reported they were i) able to actively stop the car (n=4, three had visual auras), ii) not able to stop the car resulting in accident (n=7), or iii) passenger was able to pull the car over (n=2). One accident was fatal to the other party. Twelve out of 13 patients had been driving for less than one hour.

FSWD is frequent and possibly underrecognized. FSWD often lead to accidents, which occur less if preceded by simple partial seizures. Pathophysiological mechanisms remain uncertain; it is still speculative if complex visuo-motor tasks required while driving play a role in this scenario.

The prevalence of unhealthy lifestyle habits such as smoking has seldom been described in neuromuscular disorders, including myotonic dystrophy type 1 (DM1). However, it is essential to document the unhealthy lifestyle habits as they can exacerbate existing impairments and disabilities. The objectives are: 1) To determine the prevalence of risk factors among individuals with DM1; 2) To compare the prevalence among classic and mild phenotypes.

A survey was done on a sample of two-hundred (200) patients with DM1 as part of a larger study. Lifestyle risk factors included being overweight or obese, tobacco smoking, illicit drug use, excessive alcohol consumption and physical inactivity. A registered nurse administered the validated public health survey. Categorization of risk factors were based on national standards and compared with provincial and regional prevalences.

50% of DM1 patients were overweight or obese, 23.6% were regular smokers, and 76% were physically inactive. Except for overweight and obesity, significant differences were observed between patients with classic and mild phenotypes for all the other lifestyle risk factors: those with the classic phenotype being more often regular smokers, consuming more often illicit drugs and being less physically active.

The results of this study will provide guidance for the development of better adapted and focussed health promotion interventions in the future.

The aim of the study is to explore the association of serum bilirubin levels with admission severity and short term clinical outcomes among acute ischemic stroke patients.

Data were collected from 2361 acute ischemic stroke patients in four hospitals of Shangdong Province during January 2006 and December 2008. National Institutes of Health Stroke Scale (NIHSS) was used to assess admission and discharge severity. NIHSS≥10 at discharge or in-hospital death was defined as short-term clinical outcomes. Logistic regression and trend test were used to examine the association of serum bilirubin levels with admission severity and short term clinical outcomes.

Serum bilirubin levels were significantly and positively associated with admission severity (P for trend <0.05). The age-sex adjusted odds ratios (95% confidential intervals) of NIHSS≥10 associated with the second, third and fourth quartile of total bilirubin/direct bilirubin were 1.245 (0.873, 1.777)/1.276 (0.895, 1.818), 1.484 (1.048, 2.102)/1.628 (1.158, 2.289) and 2.869 (2.076, 3.966)/2.765 (1.996, 3.828), respectively, compared with the lowest quartile; the multivariate adjusted odds ratios of NIHSS≥10 associated with the second, third and fourth quartile of total bilirubin/direct bilirubin were 1.088(0.711, 1.665)/1.436(0.94, 2.193), 1.328(0.877, 2.011)/1.647(1.092, 2.485) and 2.336(1.579, 3.458)/3.079 (2.049, 4.623), respectively, compared with the lowest quartile. However, no association between serum bilirubin levels and short-term clinical outcomes was observed in our study.

Serum bilirubin levels were associated with initial stroke severity closely. Nevertheless, there is no significant relationship between serum bilirubin levels and short-term clinical outcomes among acute ischemic stroke patients.

We conducted a retrospective chart review of 53 patients diagnosed with sporadic Inclusion Body Myositis (sIBM) who have been followed at the McMaster Neuromuscular Clinic since 1996.

We reviewed patient medical histories in order to compare our findings with similar cohorts, and analyzed quantitative strength data to determine functionality in guiding decisions related to gait assistive devices.

Patient information was acquired through retrospective clinic chart review.

Our study found knee extension strength decreased significantly as patients transitioned to using more supportive gait assistive devices (P < 0.05). A decline to below 30 Nm was particularly indicative of the need for a preliminary device (i.e. cane)(P < 0.05). Falls and fear of falling poses a significant threat to patient physical well-being. The prevalence of dysphagia increased as patients required more supportive gait devices, and finally a significant negative correlation was found between time after onset and creatine kinase (CK) levels (P < 0.01).

This study supports that knee extension strength may be a useful tool in advising patients concerning ambulatory assistance. Further investigations concerning gait assistive device use and patient history of falling would be beneficial in preventing future falls and improving long-term patient outcomes.