FRONTO TEMPORAL DEMENTIA
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A 45-year-old man presented with abnormal behavior such as entering other people's homes without permission, irrelevant talking, anger outbursts, and destroying household items over the past 7-8 months. Neurological examination and tests were normal except for frontal and temporal lobe atrophy seen on MRI. The patient was diagnosed with frontotemporal dementia based on behavioral changes, memory intactness, and imaging findings. Frontotemporal dementia is the third most common cause of dementia, characterized by predominant language and behavioral symptoms over cognitive decline. It has genetic and sporadic forms associated with genes like C9ORF72, GRN, and MAPT. Treatment is currently limited to managing behavioral symptoms.
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![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.](data:image/gif;base64,R0lGODlhAQABAIAAAAAAAP///yH5BAEAAAAALAAAAAABAAEAAAIBRAA7)
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FRONTO TEMPORAL DEMENTIA
- 1. FRONTO TEMPORAL DEMENTIA: CASE REPORT Dr. Aratrika Sen Senior Resident MSD MCH
- 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
- 8. TYPES AND CLINICAL FEATURES:
- 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.
- 11. THANK YOU