1. 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:
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.