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Tiffany W. Chow, MD, MSc, Baycrest Health Sciences Rotman Research Institute, and Ross Memory Clinic; University of Toronto Depts. of Medicine (Neurology Division) and Psychiatry (Geriatric Psychiatry Division).
Abstract
Much of the published clinical research in dementia has focused on diagnostic biomarkers and neuroimaging analyses that are not yet validated for routine clinical practice or on unsuccessful clinical drug trials. Primary care providers can nonetheless make a significant difference in the management of patients with dementia and their families, based on appropriate referrals of non-Alzheimer's dementia cases to specialists and supporting informal caregivers.
Frontotemporal degeneration, a non-Alzheimer's dementia that strikes in the 6th decade of life, provides many opportunities for the entire healthcare team to educate and back families up through a harrowing neurodegenerative illness. This paper is intended to highlight for primary care physicians what can be done and how to accomplish it through a team approach. Some concepts, such as a switch from medicalized views of "behavioural and psychiatric symptoms of dementia" to "Responsive Behaviours" can be generalized across dementia etiologies, but the age at onset and marked social disability and dysfunction caused by frontotemporal degeneration warrant some additional guidelines to assure the safety and highest quality of life possible for the patient and those around him. In particular, refitting a day program to accommodate clients with frontotemporal degeneration and attending to the needs of children who find themselves in informal caregiver roles are addressed.
Keywords: caregiver, dementia, frontotemporal dementia, primary progressive aphasia.
New Diagnostic Criteria for Frontotemporal Degeneration
Over the past 10 years, more progress has been made in understanding the genetics and proteinopathy underlying frontotemporal degeneration than in Alzheimer's disease.1 These recent discoveries have led to new 2011 diagnostic criteria. Physicians should consider the diagnosis of frontotemporal degeneration in the following instances: 1) onset age younger than 65, 2) complaint of early and marked personality change (e.g., previously introspective and earnest is now crass, boisterous, and uncaring), 3) marked language difficulties unaccompanied by memory loss, 4) MMSE score > 26 out of 30 despite lost instrumental activities of daily living (e.g., change from workplace exemplar to demotion or on probation; inability to organize billpaying), 5) anterior brain atrophy on structural imaging, as opposed to generalized finding. Frontotemporal degeneration (previously referred to as "Pick's disease" or frontotemporal dementia) in this article is used to denote the umbrella that includes both the variant manifesting most prominently with behavioural changes (bvFTD) and the variant that shows mostly aphasic changes in the first 2-3 years of illness, also known as primary progressive aphasia (PPA). The criteria for bvFTD and for the primary progressive aphasias highlight the cardinal signs of these frontotemporal degeneration subtypes.
Behavioural variant frontotemporal degeneration (bvFTD)
The criteria for bvFTD have shifted since the 1998 Neary consensus criteria were developed. The new criteria allow for patients to be diagnosed at least with possible bvFTD early in the course of illness, before all possible symptoms have arisen (see Table 1).2 Positive imaging evidence to support a frontal and/or temporal neurodegenerative process can bring a patient up to a probable bvFTD diagnosis, while showing 4-6 of the symptom criteria along with evidence of tauopathy, TAR DNA protein-43 (TDP-43), or fused-in-sarcoma inclusions on neuropathology or positive carrier status for mutations in microtubule-associated protein tau (MAPT), progranulin, or C9ORF72 will propel the diagnosis of bvFTD to the realm of definite.
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Three Primary Progressive Aphasias
As reflected in the 2011 diagnostic criteria for PPA, there are now 3 subtypes or variants: 1) non-fluent/agrammatic, 2) semantic, and 3) logopenic.3 It can be difficult for clinicians without speech and language pathology training