FRONTOTEMPORAL DEMENTIA IN A CASE OF SUICIDAL HANGING Dr. Kirti Y. Tandel, Junior Resident, Department of Psychiatry, Jagjivanram Western Railway Hospital, Mumbai Dr. Charles Pinto, Dr. Jagdeo P. Rawat, Dr. Malay D. Dave Case History ABSTRACT CLINICAL SUBTYPES Procedures Results (Continued) DIAGNOSIS The different subtypes are [1]: Behavioral variant (bvFTD) Semantic Dementia (SD) Progressive Nonfluent Aphasia (PNFA) Progressive Supranuclear Palsy (PSP) Corticobasal Degeneration (CBD) FTD with Motor Neuron Disease (FTD-MND) ALS/CTE (Chronic Traumatic Encephalopathy) Almost 60% of patients have bvFTD variant. The clinical features that differentiates it from Alzheimer's Disease are [2]: Recent advances in genetics have resulted in greater understanding of the pathophysiology of Frontotemporal dementia. While imaging may support the diagnosis, it is essentially a clinical diagnosis based on the presence of typical clinical features and the findings of neuropsychological tests. . Histological changes in the cerebral cortex in FTD: (A) Microvacuolation (DLDH features) in upper layers of the cerebral cortex (B) Ubiquitin inclusions in the cerebral cortex (C) Pick's bodies in patient with FTD and parkinsonism with chromosome 17 (FTDP-17; Q336R mutation; (D) Neurofibrillary tangles in patient with FTDP-17 (+16 exon 10 splice mutation; D). NEUROPATHOLOGY CASE HISTORY A 47 years old male patient, Locopilot by occupation was referred to Jagjivanram Hospital in an unconscious state with quadriparesis following suicidal hanging at home. After initial resuscitation, patient regained consciousness only after 5 days. He started speaking after 20 days and was discharged. After 5 months, relatives noticed that he started remaining aloof, not interacting socially with forgetfulness, irritability and abnormal behavior in form of disinhibited behavior like disrobing of clothes, hyper sexuality towards wife and making inappropriate gestures towards females. His self care gradually decreased with poor performance at work place. He was again admitted and kept on Atypical antipsychotic along with eleven settings of Electroconvulsive Therapy. According to the relatives there was 30% improvement. However there was considerable deficit in initiation of even basic daily activities. Patient could not perform his duties competently and hence he was decategorised. His FDG-PET scan showed signs of fronto-temporal dementia with hypo-metabolism in bilateral fronto temporal lobes and thalami. Over the next six months patient was also tried on Clozapine and Memantine since there was no further improvement in the cognitive domain. Patients recent whole body PET scan shows no abnormality however he still shows signs of disinhibited behavior. Features Frontotemporal Alzheimer's Age at Onset Rarely > 75 Increases with age Behavioral prob Early disinhibition, socially inappropriate Moderate to severe, increases with age Language Isolated Language + Memory Visuospatial dysfunction Rare in mild/moderate Common Motor signs Unusual Mood Alexithymia Sadness, anhedonia Psychosis Somatic, religious, bizarre delusions Delusions increase with severity Appetite Carbohydrate craving Weight loss, anorexia Frontal lobe and/or anterior temporal lobe atrophy, either bilateral or asymmetric. In early cases the scan may seem normal. Recent techniques such as magnetic resonance spectroscopy, functional imaging and cortical thickness measurements offer an earlier diagnosis. FDG-PET scans classically show frontal and/or anterior temporal hypometabolism, which helps differentiate the disease from Alzheimer's disease. The PET scan in Alzheimer's disease classically shows biparietal hypometabolism.   The ventromedial prefrontal cortex is a major locus of dysfunction early in the course of bvFTD. NEUROIMAGING Pathology and Inheritance: Intracellular accumulations of abnormal forms of protein tau (40%), TDP-43 (50%) and FUS (10%).[3,4] Majority (50-80%) of FTDs are sporadic where as only few (20-50%) are genetically inherited. INTRODUCTION TREATMENT Frontotemporal dementia (FTD) consists of progressive damage to the temporal and/or frontal lobes. The hallmark is a gradual, progressive decline in behavior and/or language with young age at onset (< 60s). It represents an estimated 10%-20% of all dementia cases and is recognized as one of the most common dementias affecting a younger population. Pharmacological Selective Serotonin Reuptake Inhibitors (SSRIs) Atypical antipsychotics Lithium + SSRIs Acetyl cholinesterase inhibitors Memantine Antioxidants, Modafinil Non-Pharmacological Psycho education Behavioral management strategies Specific behavioral restrictions Genetic counseling Caregiver counseling Support Groups REFERENCES Boxer AL, Miller BL. Clinical features of frontotemporal dementia. Alzheimer Dis Assoc Disord. 2005;19 S1:S3-6 Muangpaisan W. Geriat Aging. 2007; McKhann MG et al. Arch Neurol 2001; Muangpaisan W et al. Neuro J Thai 2003 LM Shaw LM, Korecka M, Clark CM, Lee VMY, Troganowski. Biomarkers of neurodeneration for diagnosis and monitoring therapeutics Nature Reviews Drug Discovery. 2007;6:295-303. Seelar H, Rohrer LD, Pijnenburg YAL, Fox NC, can Swieten JC. Clinical, genetic and pathological heterogeneity of FTD: A review. J Neurol Neurosurg Psychiatry 2010.