You Can Manage a Dementia without Cure: Frontotemporal Degeneration

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.

Assessment of Language Function in Dementia

David F. Tang-Wai, MDCM, FRCPC, Assistant Professor, Department of Medicine, University of Toronto; University Health Network Memory Clinic, University of Toronto, Toronto, ON.
Naida L. Graham, PhD, Research Associate, Department of Speech-Language Pathology, University of Toronto; University Health Network Memory Clinic, University of Toronto; Toronto Rehabilitation Institute, Toronto, ON.

Impairment in language is a common finding among individuals with dementia and can be a presenting symptom, particularly in Alzheimer’s dementia and primary progressive aphasia. Early recognition of language dysfunction can help with an accurate diagnosis, management, and prognosis. There are numerous established and validated language evaluation protocols. This article provides a simple means for the primary care physician to identify and evaluate language disorders in dementia, but it is not meant to replace established protocols.
Key words: aphasia, dementia, primary progressive aphasia, semantic dementia, Alzheimer’s disease.

Frontotemporal Dementia -- March 2005

Andrew Kertesz, MD, FRCP(C), Director of Cognitive Neurology, St. Joseph’s Health Center, University of Western Ontario, London, ON.
David Munoz, MD, FRCP(C), Department of Pathology, St. Michael’s Hospital, University of Toronto, Toronto, ON.

Frontotemporal dementia (FTD or Pick’s disease) is a relatively common but underdiagnosed form of presenile dementia. Estimated prevalence is 20% of dementias and 50% of dementia in patients under age 65. Common presentations are disinhibition with indifference; progressive aphasia; semantic dementia; unexplained falls, vertical gaze palsy, and dysarthria; and dementia with motor neuron disease. Neuroimaging is essential to exclude a slow tumour. Tau mutations are found in some families. Atypical neuroleptics and antidepressants can effectively treat some of the characteristics of FTD.

Key words: frontotemporal dementia, Pick’s disease, primary progressive aphasia, corticobasal degeneration, progressive supranuclear palsy.