Canadian Journal of Neurological Sciences最新文献

Aims: To describe neuropathological findings in autopsies of patients with isolated, partial or all classic features of the septo-optic-pituitary dysplasia (SOD) triad, speculate on SOD etiology and ascertain the causes of death.

Method: Retrospective review of autopsy reports of patients with one or more features of the SOD triad.

Results: Twenty-one (14 females) cases were identified. Median age at death was 2.3 years. Two fetuses died soon after birth, and the rest died at postnatal ages of 3 weeks to 50 years. Ten cases had optic nerve hypoplasia (ONH) with either (i) small pituitary gland/hypopituitarism (five cases) or (ii) absent septum pellucidum (SP)/abnormal corpus callosum (CC) (two cases) or (iii) both (three cases). Eleven cases had one or two features of the SOD triad. A wide spectrum of neuropathological findings was evident, with 12 cases resulting from in utero/perinatal vascular lesions in brain regions that led to ONH/chiasm hypoplasia, hypothalamus/pituitary gland anomalies and agenesis or abnormalities in the SP, CC and olfactory bulbs and tracts. Other suspected causes included three genetic (one with holoprosencephaly), two in utero infection and one arachnoid cyst with hydrocephalus. The most common cause of death was a respiratory illness.

Conclusions: The autopsy findings appear to be the result of destructive or less commonly impaired developmental processes. Our findings suggest that SOD is not a single disease entity but represents a syndrome with ONH and one or more of: hypothalamic-pituitary dysfunction and absent SP or abnormal CC. We consider ONH an essential part of SOD.

Background: Kinesiophobia is defined as an excessive and irrational fear of movement and physical activity. Individuals living with Parkinson's disease (PD) can be at risk of developing this phobia, due to the debilitating nature of the disease's motor symptoms such as impaired balance, bradykinesia, rigidity and tremor. This is particularly problematic, as exercise is crucial for people with PD, especially considering its potential to slow down disease progression. The Tampa Scale of Kinesiophobia for Parkinson's disease (TSK-PD) is a valid and reliable instrument for measuring kinesiophobia in PD. However, no French translation of this scale existed prior to this study.

Methods: The English TSK-PD was translated, cross-culturally adapted into Canadian French, and administered to 102 ambulatory French-speaking Canadians living with PD, aged 46-83. Statistical analyses were then conducted to examine the psychometric properties of the translated scale.

Results: Results confirmed the construct validity of the translated version and revealed high internal consistency (Cronbach's alpha = 0.90), good test-retest reliability (ICC = 0.84), with no evidence of floor or ceiling effects. Exploratory and confirmatory factor analyses supported a two-factor structure consisting of "Activity Avoidance" and "Harm."

Conclusion: The French-Canadian TSK-PD can be recommended for use in research and in clinical settings to better identify fear of movement in French-speaking PD patients and promote physical activity.

Background: Emerging evidence suggests that metabolic and hormonal disturbances in polycystic ovary syndrome (PCOS) may increase vulnerability to neurodegenerative disorders. However, the link between PCOS and Alzheimer's disease (AD)-related pathology remains unclear.

Methods: In this cross-sectional study, plasma levels of β-amyloid (Aβ₄₀, Aβ₄₂), phosphorylated tau (p-tau181), neurofilament light chain (NfL) and glial fibrillary acidic protein (GFAP) were quantified in women with PCOS and age-matched controls. Homeostasis model assessment of insulin resistance (HOMA-IR), inflammatory cytokines (IL-6, TNF-α) and hormonal parameters were assessed. Mediation and moderation analyses were conducted to explore metabolic and hormonal pathways underlying biomarker alterations.

Results: Among 400 women (200 PCOS, 200 controls), age and BMI were comparable (P > 0.05). Compared with controls, PCOS participants had increased Aβ₄₀, p-tau181, NfL and GFAP, a slightly higher Aβ₄₂, and a lower Aβ₄₂/₄₀ ratio (all P < 0.05). p-tau181 correlated positively with HOMA-IR (r = 0.41) and IL-6 (r = 0.36), while Aβ₄₂/₄₀ ratio correlated negatively with HOMA-IR (r = -0.27). In multivariable analysis, p-tau181 (aOR = 1.34, 95% CI 1.05-1.71), IL-6 (aOR = 1.19) and TNF-α (aOR = 1.14) were independent predictors of insulin resistance. Mediation analysis indicated that HOMA-IR, IL-6 and TNF-α jointly mediated ∼ 71% of the PCOS-p-tau181 association, suggesting a metabolic-inflammatory pathway linking PCOS to AD-related tau pathology.

Conclusions: PCOS is linked to peripheral markers of early Alzheimer's pathology, largely mediated by insulin resistance and inflammation. PCOS may provide a clinical context to explore metabolic-inflammatory contributors to early neurodegenerative changes.

Background: Timely access to endovascular treatment (EVT) for ischaemic stroke patients is critical for optimal outcomes, but Canada's size and population distribution create barriers to access. EVT is mostly available in tertiary centres located in large urban cities, and patients that arrive at intravenous thrombolysis (IVT)-only stroke centres need to be transferred for EVT.

Methods: Geographic modelling of access to an IVT-only centre and an EVT-capable centre was conducted for Canada. Canada was divided into small grid sections. Drive times from the centre of each grid section to the closest stroke centres and the population of each grid section were obtained. The onset to paramedic arrival time and on-scene time were assumed to be 30 and 30 minutes, respectively. In the suboptimal and optimal scenarios, the door-in-door-out (DIDO) times were 150 minutes and 45 minutes, respectively. The poor access regions and population were calculated for onset to thrombolysis at 4.5 hours and to EVT-capable centre arrival for EVT within 6 and 3 hours.

Results: The results show 99.37% of the population having access to thrombolysis within 4.5 hours. However, with a suboptimal DIDO time, 13.6% (5.2 million people) and 42.7% (16.2 million people) do not have access to EVT within 6 and 3 hours, respectively. With an efficient DIDO time, an additional 5.6% (2.1 million people) and 15.7% (6.0 million people) have access to EVT within 6 and 3 hours, respectively.

Conclusion: There is an imperative to reduce DIDO times to an ambitious median of 45 minutes to ensure optimal access to EVT across Canada.

Objectives: This study aims to evaluate differences in healthcare utilization among patients with ischemic stroke in metropolitan versus non-metropolitan Manitoba.

Methods: This study is a population-level analysis using the Discharge Abstract Database from Manitoba. The database includes all patients who received care in a Manitoba facility for ischemic stroke between April 2019 and March 2023. Data were collected on patient demographics, comorbidities and geographical location of stroke presentation (metropolitan Winnipeg vs non-metropolitan). Outcomes included length of stay (LOS), treatment, discharge disposition and mortality. Regression analysis was performed to analyze outcomes, adjusting for age, sex and comorbidities.

Results: 3704 (71.6%) patients were admitted in Winnipeg, and 1471 (28.4%) patients were admitted in non-metropolitan Manitoba. Patients presenting to Winnipeg were younger (mean age 72.3 vs 74.3 years) and had higher rates of atrial fibrillation, hypertension, diabetes, chronic kidney disease and heart failure. Patients presenting to Winnipeg had a shorter LOS (16.1 days vs 18.4 days, coefficient 0.05, 95% CI -4.54 to -1.27), had higher rates of intravenous thrombolysis (adjOR 5.13, 95% CI 3.85-6.84), were less likely to be discharged home (39.8% vs 57.5%, adjOR 0.47, 95% CI 0.41-0.53) and were more likely to be transferred for inpatient stroke rehabilitation (adjOR 3.46, 95% CI 2.64-4.54). There were no differences in in-hospital mortality. There was a higher incidence of stroke in Winnipeg compared to non-metropolitan Manitoba (F-statistic 23.84, p = 0.0028).

Conclusions: This study illustrates differences in healthcare utilization outcomes between patients living in metropolitan Winnipeg versus non-metropolitan Manitoba presenting with ischemic stroke.

Purpose: Cognitive fatigability (CF), which refers to a decline in performance during sustained cognitive effort, can significantly impact people with multiple sclerosis (PwMS). This study examined the unmet needs related to perceived CF in PwMS.

Methods: One hundred PwMS completed a survey assessing factors known to contribute to CF. Participants indicated whether each factor, including CF itself, was disruptive and whether adequate support was available to address these concerns. A factor identified as disruptive and insufficiently addressed was considered an unmet need (Need Index [NI] ≥50%).

Results: Group-level analysis revealed no significant unmet needs, although fatigue (NI = 30.23), CF (NI = 22.96) and physical activity (NI = 19.55) were more frequently reported. Individual-level analyses revealed that unmet needs varied by community setting (rural vs urban) and socioeconomic status (SES) (lower vs higher SES), with rural participants and those with lower SES reporting higher rates of unmet needs. In addition, PwMS who indicated CF was an unmet need reported more difficulties across most contributory factors, including sleep quality, fatigue, cognitive impairment, depression and contextual factors. The presence of fatigue and CF combined contributed to greater unmet needs across various domains, especially fatigue, CF and cognitive impairment, compared to fatigue alone.

Conclusions: Participants from rural and low socioeconomic backgrounds were more likely to have unmet needs. Notably, 36% of participants (N = 33) reported unmet needs related to perceived CF. The findings highlight the importance of tailoring future interventions to address identified needs more adequately.