Page 197 - Hospital Authority Convention 2018

Corporate Scholarship Presentations

C3.1 Chronic Disease Management 14:30 Room 428

Frontotemporal Lobar Degeneration – An Under-recognised Condition and Its Significance in Treatment and
Services
Yan CTY
Department of Psychiatry, Shatin Hospital, Hong Kong HOSPITAL AUTHORITY CONVENTION 2018

Frontotemporal lobar degeneration (FTLD) is a pathologic endophenotype leading to three frontotemporal dementia (FTD)
syndromes. FTD is gaining recognition as a clinically heterogeneous syndrome of progressive decline in behavioural,
executive, language or motor functions associated with frontal and anterior temporal lobe degeneration. Psychiatrists often
encounter the behavioural variant (bvFTD) and the two primary progressive aphasias (PPA) – the nonfluent-agrammatic
(nfvPPA) variant and the semantic variant (svPPA). They had common molecular bases leading to pathological protein
accumulation with overlapping microscopic findings but had unique neuroimaging patterns.

Epidemiologically, FTLD is the third most common cause of degenerative disorder with dementia after Alzheimer’s Disease
(AD) and Dementia with Lewy Bodies (DLB), accounting for 5 to 15% of confirmed cases and is the second most common
cause of presenile (<65) neurodegenerative dementia. The onset age is typically in the 60, though subtypes may vary. The
prevalence is similar in Asians, and FTD was indeed the second most common aetiology in early-onset dementias from the
preliminary data of a Neurology study from The Chinese University of Hong Kong, echoing the worldwide picture. Reasons
for misconstrued impression of FTD being uncommon include the clinical heterogeneity and the absence of standardised
diagnostic criteria for detection and identification; and there has yet to be a recent study on its local prevalence.

The Memory and Aging Center (MAC) of the University of California, San Francisco (UCSF) is the world’s leading centre
specialising in FTD diagnosis and research, and its multidisciplinary team comprises experts responsible for proposing
the new criteria on the diagnostic certainty for FTD variants in the 2011 international brain consortium. During the four-
week attachment, the author had an ample opportunity to observe and learn from the MAC’s multi-faceted care pathway
and service delivery from suspecting to diagnosing clients with FTD. With the updated criteria, we are establishing an FTD
registry in our unit to (1) elicit factors to design FTD protocols allowing for early identification, diagnosis and interventions; (2)
enhance clinicians’ understanding and awareness of FTD. As our existing services mainly catered for the needs of AD clients,
this translates as service remodeling on assessment, diagnosis, treatment and caregiver support. Tuesday, 8 May 2018

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