A Case Report On Young Onset Frontal Temporal Dementia Presented As Catatonia

INTRODUCTION. § Frontotemporal dementia (FTD) refers to a group of dementias characterized by degeneration in the frontal and temporal lobes of the brain. Rates of early-onset FTD are high with 13% of individuals with FTD being under the age of 50. § Younger cases of FTD tend to exhibit frequent abrupt mood changes, increased aggression, behavioral disinhibition, lack of empathy, and deficits in working memory. There are three clinical presentations of FTD: behavioral variant (bvFTD) and two forms of primary progressive aphasia. Here we report a a 37 yr Old married man was brought with complaints of a progressive history of changes in behaviour and personality.The patient was in good baseline health until age 35 years, when his family noticed an increase in change in behavior with history of cannabis smoking for 2 yrs, H/O multiple extra marital affairs for 2 yrs,with H/O restlessness,dull and withdrawn, sleep disturbance,poor self care, refusing to eat properly, slowly not speaking to anyone, poor work performance for the past 6 months. He was diagnosed as a case of substance induced psychosis with catatonic features and treated. Again after three months pt presented to psychiatric opd with complaints of restlessness, speech difficulty forgetting things, inappropriate laugh,disinterest in daily activities, walking in the house all the time, disinhibited behaviour,weight loss,excessive craving for tobacco,putting inedible things at mouth all these symptoms for one month. On Mse O/E he was alert,ambulant,kempt,yawning frequently during interview, not cooperative, sits in chair and gets up frequently, no Spontaneous speech. He did not attempt to initiate conversation, answering questions with short, terse responses. relevant replies. He often gazed with a prolonged, fixed stare and exhibited motor stereotypies such as clapping his thighs or tapping his feet vigorously. — There were no upper or lower motor neurone signs, frontal release signs positive, and no parkinsonism or limb apraxia. His gait was normal. He scored only 55 out of 100 on the Addenbrooke’s Cognitive Examination-III, with impairments across domains but with particularly poor executive function (naming only two words beginning with P in a minute, and only 10 animals over a minute, leading to a very low verbal fluency score of 4 out of 14). Attention (score 12 out of 18), language (19 out of 26), memory (9 out of 26) and visuospatial (11 out of 16) abilities were also affected. — Further neuropsychological testing identified a global reduction in general intellectual function, with severe executive function deficits as well as poor verbal working — Neurology opinion obtained as a case of young onset FTD — MRI scan of the brain showed substantial bilateral, symmetrical frontal atrophy, with no evidence of white matter changes that would be typical of a vascular, vasculitic or inflammatory cause (figure 1). In addition, autoimmune encephalitis was negative; and HIV and syphilis serology were all normal or negative. Cerebrospinal fluid (CSF) contained no white cells or abnormal cells and therefore no evidence of an active inflammatory, infective or malignant process; the CSF protein concentration was also normal. PET scan suggestive of FTD REFERENCE: 1. Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia. Brain 2011;134:2456?77. 2. Lebert F, Stekke W, Hasenbroekx C, et al. Frontotemporal dementia: a randomised, controlled trial with trazodone. Dement Geriatr Cogn Disord 2004;17:355?9. 3. Warren JD, Rohrer JD, Rossor MN. Clinical review. Frontotemporal dementia. BMJ 2013;347:f4827. 4. Neumann M, Mackenzie IRA. Review: Neuropathology of non-tau frontotemporal lobar degeneration. Neuropathol Appl Neurobiol 2019;45:19?40. 5. Mann DMA, Snowden JS. Frontotemporal lobar degeneration: pathogenesis, pathology and pathways to phenotype. Brain Pathol 2017;27:723?36. Ct brain showed bilateral frontoparietotemporal Subdural HYGROMA. Neurologist opinion obtained. There was no focal neurological defecit and conservative management suggested. Patient was treated with tab risperidone 2mg 1-0-1 tab sodium valproate 200 mg 1-1-1, tab diazepam 5mg 0-0-1, her symptom improved with treatment (ymrs-12.) 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