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Phenocopy Frontotemporal Dementia: A Case Series from a National Memory Clinic and a Review of the Literature

Published online by Cambridge University Press:  23 March 2020

C. Power ,
O. Hannigan ,
M. Gibb ,
I. Bruce ,
M. McCarthy ,
R. Coen ,
D. Robinson  and
B.A. Lawlor
C. Power
Affiliation:
St James's Hospital, Memory Clinic – Mercer's Institute for Research in Ageing, Dublin, Ireland
O. Hannigan
Affiliation:
Memory Clinic, Mercer's Institute for Research in Ageing, Dublin, Ireland
M. Gibb
Affiliation:
Memory Clinic, Mercer's Institute for Research in Ageing, Dublin, Ireland
I. Bruce
Affiliation:
Memory Clinic, Mercer's Institute for Research in Ageing, Dublin, Ireland
M. McCarthy
Affiliation:
Memory Clinic, Mercer's Institute for Research in Ageing, Dublin, Ireland
R. Coen
Affiliation:
Memory Clinic, Mercer's Institute for Research in Ageing, Dublin, Ireland
D. Robinson
Affiliation:
Memory Clinic, Mercer's Institute for Research in Ageing, Dublin, Ireland
B.A. Lawlor
Affiliation:
Memory Clinic, Mercer's Institute for Research in Ageing, Dublin, Ireland

Abstract

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Introduction

The existence of a frontotemporal dementia phenocopy (phFTD) syndrome remains controversial. Opinions differ on whether the phenocopy presentation represents the neuropsychological manifestation of a mid-life decompensation in vulnerable pre-morbid personalities or an indolent prodrome of behavioral-variant FTD (bvFTD). Literature on this topic is sparse and clinicians and patients have little guidance around prognosis and management.

Objectives

To describe the demographic, neuropsychological and biomarker profiles of a case series of phFTD patients, attending the memory clinic and review relevant literature.

Methods

Retrospective review of all cases diagnosed with phFTD.

Results

Eleven cases were identified (male = 9, female = 2). Mean age 55.8 years. Subjective complaints comprised memory and language difficulties. Collateral reports described apathy, aggression, impulsivity, disinhibition, hyperorality. Function was relatively preserved though motivation or supervision for higher-level tasks was sometimes required. All had non-neurodegenerative MRI and PET scans. Neuropsychological test (NPT) findings predominantly showed executive dysfunction and fluency impairment. A total of 3/11 had non-amnestic memory impairment. Follow-up imaging and NPT were invariably unchanged; 1/11 had a pre-morbid psychiatric diagnosis; 5/11 had unusual personality traits pre-morbidly. Major psychosocial stressors were documented in 7/11. Management consisted of psychosocial interventions to support function and interpersonal relationships.

Conclusions

The literature describes the phFTD syndrome as predominantly affecting males though we include 2 females who meet the criteria. In keeping with our findings, personality traits and psychosocial stressors may be more common in phFTD than bvFTD. More severe symptoms, memory impairment at presentation and C9ORF72 gene mutation may predict eventual progression. Those who do not progress have minimal long-term functional impairment though behavioral symptoms persist.

Disclosure of interest

The authors have not supplied their declaration of competing interest.

Type
e-Poster Viewing: Neuroscience in Psychiatry
Information
European Psychiatry , Volume 41 , Issue S1: Abstract of the 25th European Congress of Psychiatry , April 2017 , pp. S636 - S637
Copyright
Copyright © European Psychiatric Association 2017
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