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Fronto Temporal Dementia Spectrum

Col K Seby
Dementia
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◻ Major Neurocognitive disorder


◻ De Mentia (Latin) = “Devoid of mind”
◻ 20% are treatable/reversible (Heji et al 2002)
Importance of FTD for Psychiatrists
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Importance of FTD for Psychiatrists
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◻ Early onset compared to Alzheimer’s Dementia (AD)


◻ Early behavioural & personality changes mimicking
primary psy disorder
¤ Memory impairment later
Spectrum of FTD
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◻ Non-homogenous group
◻ Varied etiology
◻ Frontal or temporal or both affected out of proportion
to other parts of brain
History of FTD
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◻ 1892 Arnold Pick (German neurologist) – Pick’s


disease
¤ Insidious personality/behavioural change
¤ Disinhibition
¤ Subsequent gradual loss of speech
◻ 1990 paper by Gustafson & Brun
Epidemiology of FTD
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◻ 0.15% prevalence
◻ 10-15% of neurodegenerative dementias

◻ Onset 45-65 yrs (50-60 yrs)

¤ except SD – onset 70 yrs

◻ 50% familial

¤ Autosomal Dominant commonly


Variants
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◻ Bv FTD or fv FTD
◻ PFNA

◻ SD

◻ FTD-MND
bvFTD - Features
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◻ Early decline of IP conduct


◻ Early decline in regulation of personal conduct

◻ Early emotional blunting

◻ Early loss of insight


bvFTD – Features contd
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◻ Decline in personal hygiene


◻ Mental inflexibility/impersistence/ distractibility

◻ Hyperorality

◻ Dietary changes

◻ Perseverative or stereotyped behaviour

◻ Utilization behaviour

◻ Purposeless behaviour, lacking in goals

◻ OC symptoms – hoarding, pica, eating same kind of


food like sweets
◻ Emotional lability, apathy
bvFTD contd
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◻ Orbitofrontal type
¤ Overactive, disinhibited

◻ Other frontal areas


¤ Apathetic
bvFTD contd
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◻ Males = Females
◻ Duration 2-15 yrs

◻ Psychometry
¤ Executive dysfunction
¤ Reduced verbal recall
¤ Phonemic fluency affected more than categorical
fluency
¤ Preserved visual memory
¤ Preserved recognition memory
bvFTD contd
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PNFA
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◻ Non-fluent aphasia with intact comprehension


◻ Anomia

◻ Phonemic paraphasia

◻ Social skills preserved & behavioural symptoms mild

◻ Eventually mutism
PNFA
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SD
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◻ Temporal variant FTD


◻ Semantic knowledge loss

◻ Supra-ordinate paraphasia

◻ Surface dyslexia

◻ More behavioural symptoms than PPA

◻ Progresses to mutism
SD
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FTD-MND
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◻ 10% of ALS patients develop FTD


◻ ALS symptoms – fasciculations, weakness,

hyperreflexia, spasticity, upgoing plantars


◻ Swallowing difficulties, choking
Rule out
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◻ Structural frontal pathology


¤ Meningioma

¤ Chronic SDH

◻ Vit B12 deficiency

◻ Neurosyphilis

◻ Hexa Nucleotide repeat in strongly familial cases


Treatment
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◻ SSRI
¤ Apathy, depressive symptoms

◻ Low dose SDA like Quetiapine

¤ Agitation

◻ No role for AchEI like Donepezil

¤ Memantine may be tried

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