2. CASE VIGNETTE:
• 45 year old Mr. X. normotensive and nondiabetic was brought to psychiatry opd with
complaints of abnormal behavior like inadvertently entering other peoples’ house,
irrelevant talking, over familiarity, unprovoked anger outburst mostly towards family
members, destroying household things without any proper reason since last 7-8
months.
• This period was preceded by lack of interaction, reduced activity level, though the
patient denied any depressed mood.
• Off late he seldom uses some unknown words to describe a familiar object. [e.g.,
“Rata” for “Ruti”]
• No significant past h/o psychiatric illness.
• General examination and Neurological examination did not reveal any abnormality.
3. CONTD. HCF revealed intact immediate, recent and remote
memory with normal orientation.
Judgement was impaired with absent insight.
MMSE score was 22/30.
Routine hematological investigations were WNL.
MRI Brain revealed atrophy of B/L frontal lobe. [Left >
Right] and also Left anterior temporal cortex.
5. INTRODUCTION:
3rd most common cause of dementia.
Previously called Fronto Temporal Lobar
Degenration.
Ist described by Arnold Pick, also called
Picks’s Disease.
Pick Body: Intrea neuronal inclusion body
with neuronal ballooning.
Language and behavioural symptoms
more predominant than dementia.
6. ETIOLOGY:
SPORADIC
FAMILIAL
Genes involved:
1. C9ORF72: 26% FAMILIAL, 5% SPORADIC
Association with ALS, MND
2. GRN Mutation: in Chr 17, 5-10 % sporadic
3. MAPT: Chr 17, Tau protein, Association with PSP, CBD
4. Others: Mutation of CHMP2B, VCP, FUS, TARDBP
9. COURSE AND PROGNOSIS:
Progressive deterioration.
On average, survival is 8 years from the onset of disease.
Those associated with MND, have significant shorter life span.
Overall, patients with FTD show rapid decline than patients with Alzheimer’s
Disease.
10. TREATMENT:
Currently there are no treatments for the cognitive deficits associated with FTD,
and no treatments to prevent progression.
Symptomatic use of anti-depressants, anti-psychotics, mood stabilisers for
agitation and disinhibited behavior.