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There is limited information on rare spinocerebellar ataxia (SCA) variants, particularly in the Canadian population. This study aimed to describe the demographic and clinical features of uncommon SCA subtypes in Canada and compare them with international data.
Methods:
We conducted a case series and literature review of adult patients with rare SCA subtypes, including SCA5, SCA7, SCA12, SCA14, SCA15, SCA28, SCA34, SCA35 and SCA36. Data were collected from medical centers in Ontario, Alberta and Quebec between January 2000 and February 2021.
Results:
We analyzed 25 patients with rare SCA subtypes, with onset ages ranging from birth to 67 years. Infantile and juvenile-onset cases were observed in SCA5, SCA7, SCA14 and SCA34. Most patients presented with gait ataxia, with no significant differences across groups. Additional common features included saccadic abnormalities (22 of 25), dysarthria (19 of 25) and nystagmus (12 of 22, except in SCA7). Less common findings included dystonia (8 of 25), cognitive impairment (7 of 25), tremor (9 of 25) and parkinsonism (3 of 25).
Conclusion:
Our study highlights the heterogeneity of rare SCA subtypes in Canada. Ongoing longitudinal analysis will improve the understanding, management and screening of these disorders.
This chapter proposes a framework for estimating the investment in human capital from health improvement or activities that improve life expectancy and reduce morbidity rates. The measurement framework builds on and extends the Jorgenson-Fraumeni income-based approach for estimating human capital to account for the effect of health on human capital. This economic approach to measuring health human capital differs from the welfare-based approach that estimates the economic effect of health improvements on the quality of life and well-being of individuals. The framework is then implemented for Canada, and the investment in health human capital for the period from 1970 to 2020 is estimated. The estimated investment in health human capital based on the income approach was found to be lower than health expenditures in Canada. This suggests that much of the health expenditures should be classified as consumption rather than as an investment that increases earnings.
Why did the Liberal Party of Canada (LPC) and the New Democratic Party (NDP) enter into a supply-and-confidence agreement in March 2022? Interparty cooperation among federal parties is rare during minority governments, and yet the agreement created a formal alliance in the House of Commons. In this article, we argue that ideational factors led to the 2022 agreement. We examine the role of programmatic beliefs and strategic learning during the COVID-19 crisis and the 2019-2021 election sequence to shed light on changes in federal parliamentary strategies in Canada. From ad-hoc voting coalitions to extended cooperation on social policymaking, the LPC and the NDP learned how to work together in the House of Commons while using the agreement as a tool to compete with each other in anticipation of the next federal election.
For over fifty years, Canada’s language regime has centered - in theory, policy, and practice - on a binary: linguistic duality and authority of the two settler colonial powers, English and French. The legislative enshrinement of status for these colonial languages, by way of the 1969 Official Languages Act, has on most accounts failed in multiple ways. As is well documented, legislated equality between French and English has rarely manifested itself in practice. Less attention - scholarly or political - has been paid to the Indigenous languages erased by both political discourse and public policy in Canada. What limited policy attention there has been has focused on Indigenous languages as second languages. The development of the Canadian Parliament’s Indigenous Languages Act, launched by the Government of Canada on December 5, 2016, attempted to fill this gap. Analysis of this process reveals the tensions within Canada’s established language regime, while putting into sharp relief the difficulties of policy and policymakers to attend to - and move beyond - Canada’s colonial past and framework.
This chapter is the first of two that examine the legal encounter with Jewishness in public space by focussing on the Orthodox practice of the eruv. The eruv is a distinctly Orthodox practice and fault lines here do not run simply between Jews and non-Jews but also between different Jews. In the modern secular legal arena, questions of non-establishment and the boundaries of religious freedom serve as the dominant legal frames, turning the eruv into a matter of excessive religiosity to be contained by law. Yet underneath the lofty language of constitutional separation often lurk concerns about national and local identity as well as sovereignty and ownership. Moreover, while circumcision has often galvanised Jews of different denominations, the eruv exposes internal Jewish rifts about Jewish identity and difference in contemporary societies. Indeed, some Jews themselves have not shied away from mobilising the authority of secular law to enforce their vision of what they consider the acceptable boundaries of Jewishness today.
How are dictionaries shaped by social history, and how far do dictionaries themselves shape social history? Wordlists and dictionaries (broadly defined) reflect particular perspectives and may be adapted for new audiences. This chapter maps the most significant historical intersections of English dictionaries and Anglophone societies. It spans the shift from English as a colonized to a colonizing language, from the medieval period to around 1900. Its building blocks include intersecting conceptions of gender roles, the family, social status, work and industrialization, as well as urbanization and racialization. Some other concepts remain implicit. Education (inside as well as outside the home) interconnects every section. It was in religious contexts that Latin was codified and methods were perfected for organizing words within books as well as books within libraries. The idea of the nation was later shaped by the Oxford English Dictionary with history and by the state with nineteenth-century mass primary education. Overall, tensions between human agency and determinism are brought constantly into the foreground. The focus on English lets me contrast revisions of the ‘same’ text within the limits of a handbook chapter. My anecdotal approach relates social changes to identifiable revisions and initiatives by individual lexicographers.
Do Indigenous peoples in present-day Canada display lower levels of diffuse support than non-Indigenous settlers? Given settler colonial relations (both historic and contemporary) and Indigenous peoples’ own political thought, we can expect that Indigenous peoples would have even lower perceptions of state legitimacy than non-Indigenous peoples. However, there are conflicting expectations regarding whether the descriptive representation of Indigenous peoples in settler institutions is likely to make a difference: on one hand, Indigenous people may see themselves reflected in these institutions and consequently feel better represented; on the other hand, these forms of representation do not challenge the underlying colonial nature of these institutions. Using data from the 2019 and 2021 Canadian Election Studies, our statistical analysis demonstrates that: (1) diffuse support is significantly lower among Indigenous peoples than non-Indigenous peoples, including people of color; (2) Indigenous respondents across multiple peoples have similarly low levels of diffuse support, and (3) being represented by an Indigenous Member of Parliament does not change the levels of diffuse support among Indigenous peoples. Overall, our research highlights the outstanding challenges to achieving reconciliation through the Canadian state and points to ways large-N analyses may be made more robust.
As Western society becomes increasingly digitally dependent and many older adults actively engage in the online world, understanding the experiences of those who largely do not use digital technology in their daily lives is crucial. Individual interviews were conducted (pre-pandemic) with 23 older adults who, based on self-identification, did not regularly use digital technology, exploring how their experiences as limited digital technology users may have impacted their daily lives. An iterative collaborative qualitative analysis demonstrated three main themes: internet concerns, frustrations with digital technology, and conflicting motivators to use digital technology. Findings suggest that addressing digital concerns and providing effective digital skill learning opportunities may encourage some older adults to become more digitally engaged. However, as people, including older adults, can be uninterested in using these technologies, organizations and institutions should work to offer ways to support people of all ages who are not engaged online.
Rock art can be useful as a factor in reclaiming Indigenous identities. One example of this phenomenon is work by contemporary artists who explore and integrate rock art in their creations. The author considers how and why a selection of artists in Siberia/Central Asia and Canada use these ancient images.
To characterise consumption of ultra-processed foods and drinks (UPF) across a range of socio-demographic characteristics of Canadians.
Design:
Cross-sectional study. The national-level 2015 Canadian Community Health Survey–Nutrition provided data on all foods and drinks consumed on the previous day via a 24-hour dietary recall. All food items were classified according to the type of industrial processing using the NOVA system. Multivariable linear regression models examined associations between a range of socio-demographic characteristics and the mean energy contribution (% of total daily energy intake) from total UPF and UPF subgroups.
Setting:
The ten Canadian provinces.
Participants:
Canadians aged 2 or older (n 20 103).
Results:
UPF contributed, on average, nearly half (44·9 %) of total daily energy intake of Canadians. Children aged 6–12 and adolescents aged 13–18 consumed over half of total daily energy from UPF (adjusted means of 51·9 % and 50·7 %, respectively). Recent and long-term immigrants consumed a significantly lower share of energy from UPF (adjusted means of 42·2 % and 45·1 %, respectively) compared with non-immigrants (54·4 %), as did the food secure (42·8 %) v. those in moderately (48·1 %) or severely food-insecure households (50·8 %). More modest differences were observed for intake of total UPF and UPF subgroups by sex, education, income adequacy and region of residence.
Conclusion:
Levels of UPF consumption in 2015 in Canada were pervasive in all socio-demographic groups and highest among children and adolescents, non-immigrants and those living in food-insecure households. These findings can inform public health interventions to reduce UPF consumption and promote healthier diets in various socio-demographic groups.
Les instruments auxquels un État peut avoir recours pour atténuer les risques que font peser les inégalités économiques sur la démocratie sont nombreux et peuvent prendre différentes formes. Dans cet article, nous cherchons à mettre en lumière la dimension normative des trois principaux instruments auxquels on a généralement recours pour mitiger l'influence de l'argent dans la compétition électorale, ainsi que le contexte dans lequel ils furent institués, remodelés – et parfois démantelés – au Canada. Ces trois mécanismes sont la limitation des dépenses électorales, le plafonnement des contributions privées et le financement public des partis. Il ne s'agit toutefois pas uniquement de décrire ces instruments, mais de réfléchir aux justifications normatives spécifiques à chacun, et d'en comprendre leur complémentarité. Plus largement, il s'agit d'offrir un cadre pour penser les enjeux de financement électoral en philosophie politique, un sujet trop souvent laissé dans l'ombre par la théorie démocratique.
Seven new species of Verrucaria are described from Finland: Verrucaria hakulinenii sp. nov., V. juumaensis sp. nov., V. linkolae sp. nov., V. lohjaensis sp. nov., V. norrlinii sp. nov., V. oulankajokiensis sp. nov., and V. vainioi sp. nov. Verrucaria linkolae is also reported from the Czech Republic, Germany and the United Kingdom, V. norrlinii from Norway and V. juumaensis from Canada based on a previously unidentified soil sample. Based on ITS sequences, V. hakulinenii and V. juumaensis probably belong to the Verrucaria hydrophila group whereas V. linkolae, V. norrlinii, V. oulankajokiensis and V. vainioi are closely related to V. hunsrueckensis and V. nodosa. The new species are characterized by a thin brown or green thallus, rather small perithecia and a predominantly thin involucrellum reaching the exciple base level. Verrucaria hakulinenii is characterized by a thin thalline cover of the perithecia, a green thallus and fairly large spores (18–22 × 8–10 μm). Verrucaria juumaensis, V. linkolae, V. norrlinii and V. vainioi are characterized by a predominantly brown thallus, often with goniocyst-like units. Verrucaria linkolae has densely occurring perithecia (100–330 perithecia per cm2) whereas in V. juumaensis, V. norrlinii and V. vainioi perithecia occur more sparsely (40–160 perithecia per cm2). Verrucaria juumaensis and V. vainioi usually have a minute thallus. Verrucaria juumaensis differs from V. vainioi by slightly larger perithecia (0.18–0.27 mm diam.) and longer and wider spores. Verrucaria lohjaensis is characterized by a mosaically dark brown and white, small areolate thallus and conspicuous ostioles. Verrucaria oulankajokiensis has small perithecia that are often thinly covered by thalline tissue and a thallus partly surrounded by dark lines. Most species occur on calcareous rocks, but V. vainioi is restricted to siliceous rocks. Verrucaria linkolae and V. norrlinii are widely distributed both on calcareous, serpentine and siliceous rocks, preferring pebbles. Epiphytic occurrences of V. linkolae and V. norrlinii are confirmed. A key to the new species and species with a similar morphology in Finland is provided.
This article examines two venues where historians of education have in the past addressed serious, publicly significant questions: commissions of inquiry and courtrooms where education rights and educational injustices are litigated. The article argues that these two examples demonstrate historians’ particular skills and abilities as evidence-gatherers, clear communicators, strong generalists, and experts in making sense of change over time. The article also suggests that these particular skills and abilities can be the basis for historians’ continued contributions to answering questions of public significance.
During the Second World War, Germans, Austrians and Italians living in Great Britain were designated as ‘enemy aliens’ and consequently interned. The worsening situation on the continent in May and June 1940 stirred up hysteria that spies and saboteurs could be amongst the Germans and Austrians. Mass arrests started in May 1940, and Italians were soon caught up in the detentions when Mussolini declared war on 10 June, thus filling internment camps to capacity. Canada and Australia agreed to take some of the ‘most dangerous characters’, facilitating the most controversial aspect of internment – deportation – which led to the ultimate tragedy when the SS Arandora Star was torpedoed and sunk on 2 July 1940. Building on previous scholarship that focuses on either German or Italian internment, this article examines both government policy towards and the internee experience of these two groups on an equal footing, thus furthering integration of the Italian narrative within internment historiography.
Heritage language speakers often feel discouraged from using their heritage language because they are told they do not speak it well. This book offsets such views by investigating heritage language variation and change across generations in eight languages spoken in Toronto. It introduces new methodology to help readers understand and apply variationist sociolinguistic approaches to quantitatively analyze spontaneous speech. This approach, based on a corpus of 400+ speakers, shows that variation and change across the grammar of heritage languages resemble the patterns in hegemonic majority languages, contrasting with the simplification/attrition patterns in experimental heritage language studies. Chapters compare patterns across generations, across languages, across ten variables in Cantonese, and between indexical and non-indexical patterns. Heritage language speakers are quoted, showing that this research increases heritage language usage and pride. Providing a tool for language revitalization, this book is essential reading for anyone interested in learning about and/or conducting research on heritage languages.
It has been widely recognized that how languages behave, particularly under conditions of contact with other languages, depends on their context. Using the Ethnolinguistic Vitality framework, this chapter describes the demographics, linguistic attitudes and institutional supports for heritage languages, defining the concepts and illustrating them with examples from Toronto, the context in which the HLVC project is conducted. Demographic information includes population sizes, language shift rates, and history of settlement in Toronto. Status information includes both reflections on the status of heritage languages, as a whole, in Canada and labels attributed to the specific varieties. The institutional support section reports on the number of language classes available for each language. The chapter also includes discussion of language policy, particularly for education, and the demographics of the university where the research is centered, enabling other researchers to best consider what aspects of the project might need adjusting for adaptation in other contexts.
Using data envelopment analysis, we examine the efficiency of Canada's universal health care system by considering a set of labour (physicians) and capital (beds) inputs, which produce a level of care (measured in terms of health quality and quantity) in a given region. Data from 2013–2015 were collected from the Canadian Institute for Health Information regarding inputs and from the Canadian Community Health Survey and Statistics Canada regarding our output variables, health utility (quality) and life expectancy (quantity). We posit that variation in efficiency scores across Canada is the result of regional heterogeneity regarding socioeconomic and demographic disparities. Regressing efficiency scores on such covariates suggests that regional unemployment and an older population are quite impactful and associated with less efficient health care production. Moreover, regional variation indicates the Atlantic provinces (Newfoundland, Prince Edward Island, Nova Scotia, New Brunswick) are quite inefficient, have poorer economic prospects, and tend to have an older population than the rest of Canada. Oaxaca-Blinder decompositions suggest that the latter two factors explain about one-third of this efficiency gap. Based on our two-stage semi-parametric analysis, we recommend Canada adjust their transfer payments to reflect these disparities, thereby potentially reducing inequality in regional efficiency.
The COVID-19 pandemic has presented numerous challenges to older adults in Canada, including the ability to volunteer. The purpose of this study is to improve the understanding of the social context surrounding volunteering in Canada, by (a) determining changes in associations between human, social, and cultural capital and volunteering among older adults; and (b) examining the relationship between ethnic minority status and volunteering, using data from the Canadian Longitudinal Study on Aging (CLSA), collected prior to and during the pandemic. This study utilized data from 24,306 CLSA Baseline, Follow-up 1 (FUP1), and COVID-19 Baseline Survey participants (aged 55+). Results confirm a decrease in volunteering during the early stages of the pandemic. Compared to pre-pandemic associations, volunteers during the early stages of the pandemic were more likely to be young–old, male, employed, and not involved in religious activities. Findings provide evidence of pandemic effects on volunteering among older adults in Canada.
In Canada, there is interest in expanding medical assistance in dying (MAID) to include advance requests (AR) for people living with dementia (PLWD). However, operationalizing the intolerable suffering criterion for MAID in ARs for PLWD is complicated by the Canadian legal context—in which MAID is understood as a medical intervention and suffering is conceptualized as subjective—and the degenerative nature of dementia. ARs that express a wish to receive MAID when the PLWD develops pre-specified impairments are problematic because people are unlikely to accurately predict the conditions that will cause intolerable suffering. ARs that express a wish to receive MAID when the PLWD exhibits pre-specified behaviors that likely represent suffering are problematic because they are inconsistent with the subjective conceptualization of suffering. Further research is required to determine whether adopting an objective conceptualization of suffering is justified in these cases and, if so, how to reliably identify intolerable suffering in PLWD.
Two differing ideas characterized the city of Toronto throughout the twentieth century. The first, Toronto the Good, represented the aspirations of religious leaders, reformers, politicians, and police officers to create a city modelled after Christian morality. Sexuality was meant to be expressed in the confines of the private, monogamous, heteronormative family home. Sex was for procreation, not pleasure. Contrary to Toronto the Good was a second idea, Toronto the Gay, a 1950s tabloid reference to the variety of spaces available for sexual exploration and desire. Sex work, queer sex, interracial marriage, divorce, birth control, and abortion endured despite intense enforcement of sexual morality. This chapter explores the tensions between the idealism of Toronto the Good and the sexual opportunities of Toronto the Gay.