Case study of partner training in corticobasal degeneration

Autor: Lauren Sell, Jerry K. Hoepner, Heather Kooiman
Rok vydání: 2016
Předmět:
Zdroj: Journal of Interactional Research in Communication Disorders. 6:157-186
ISSN: 2040-512X
2040-5111
Popis: introductionCorticobasal degeneration (CBD) is a rare neurodegenerative disease that is often diagnosed in adults in their early 60s, yet may be identified in some cases as early as the mid-40s (Stover and Watts, 2001). While no systematic epidemiological studies identifying incidence and prevalence have been conducted, Togasaki and Tanner (2000) estimate prevalence of 4.9 to 7.9/100,000. However, there is much disagreement about the accuracy of those estimates. CBD is characterized by asymmetrical extrapyramidal impairments, prominent apraxia, unilateral rigidity, and focal cortical syndromes, given a prominent underlying pathology of distinctive tau-positive histology (McMonagle, Blair, and Kertesz, 2006; Okazaki et al., 2010). Diagnosis can only be made post-mortem due to the substantive overlap between symptomology in CBD, progressive supranuclear palsy (PSP), frontotemporal dementia (FTD), and primary progressive aphasia (PPA) (Boeve, Lang, and Litvan, 2003; Lang, 2003; McMonagle et al., 2006). While a diagnosis may only be confirmed through post-mortem pathology, a number of clinical diagnostic criteria exist. The following features are used in presumptive clinical diagnosis: progressive asymmetrical rigidity, asymmetrical apraxia of an upper extremity, alien limb phenomenon, aphasia, myoclonus, bradykinesia, tremor, and dystonia (Boeve et al., 2003; Lang, 2003; Kertesz, Martinez-Lange, Davidson, and Munoz, 2000; McMonagle et al., 2006). A systematic literature review generated a summary of clinical features based upon analysis of 37 nonoverlapping articles describing 103 clinical cases and 106 brain bank cases (Armstrong et al., 2013). This review identified prominent motoric and higher-cortical features, as well as identifying variants or CBD phenotypes. Higher-cortical impairments include apraxia, alien limb phenomena, cortical sensory loss, cognitive impairment, behavioural changes, and aphasia. The five phenotypes identified include corticobasal syndrome, progressive supranuclear palsy syndrome, frontotemporal dementia, Alzheimer's disease-like dementia, and primary progressive aphasia variants. Given the heterogeneity and overlap between diagnostic criteria for these phenotypes, Armstrong and colleagues proposed two diagnostic classifications for CBD variants, probable CBD (cr-CBD) and possible CBD (p-CBD). For a comprehensive review, see Armstrong et al., 2013.Information concerning the aetiology and demographic distribution of CBD is limited. There is not adequate data to suggest that CBD affects males or females more, yet studies have indicated a predominance of female cases (Stover and Watts, 2001). Most individuals with CBD have no family history, with the exception of some FTD phenotypes and rare familial CBD variants (Armstrong et al., 2013). Disease duration is 6.6 years (SD 2.4, range 2.0 - 12.5) (Armstrong et al., 2013).Cognitive impairments associated with CBD include memory impairments, constructional apraxia, executive dysfunction, and behavioural changes (Donovan, Kendall, Bacon Moore, Rosenbek, and Gonzalez Rothi, 2007; Frattali, Grafman, Patronas, Makhlouf, & Litvan, 2000; Kertesz et al., 2000). Frontal lobe symptoms including apathy, irritability, disinhibition, personality changes, impulsivity, and excessive eating or drinking are common (Rinne, Lee, & Thompson, 1994; Wenning et al., 1998). It is noted that nature and severity of cognitive impairment is variable, ranging from subtle executive dysfunction to severe dementia and aphasia (Bak and Hodges, 2008).Empirical evidence describing the clinical speech and language profiles of individuals with CBD is growing. Existing evidence is drawn from case reports (Donovan et al., 2007; Mimura et al., 2001) and retrospective chart reviews (Graham, Bak, and Hodges, 2003; Kertesz et al., 2000). Progressive onset of fluent and non-fluent aphasias, either preceding or proceeding onset of movement disorders have been well described (McMonagle et al. …
Databáze: OpenAIRE