Frontal temperal Dementia

SCA 17 is a rare, autosomal dominant disorder caused by TBP gene CAG/CAA repeat expansion. Ataxia and dementia are common. The presence of frontal dysfunction at outset of the disease may mimic frontotemporal dementia (FTD). Parkinsonism, chorea, dystonia, and pyramidal signs may occur. We report an Irish family with autosomal dominant partially penetrant frontal dementia with cerebellar atrophy due to SCA17 and present detailed neuropsychological assessment for the first time. A 44-year-old doctor presented with 18-month history of behavioral problems...

We retrospectively examined the clinical features and the neuroradiological findings on autopsy of 2 cases of young-onset dementia. The patient in case 1 was a 43-year-old woman who was unable to determine the time on the clock and who made frivolous remarks. Neuropsychological test batteries demonstrated memory impairment and frontal lobe dysfunction. T2-weighted magnetic resonance imaging (MRI) of the head revealed abnormal high-intensity signals around the lateral ventricles and thinning of the corpus callosum...

The aim of the Neuropsychology applied to the Alcohol Dependence field is the comprehension of the effects of brain dysfunction on cognition and human behavior. It investigates neurocognitive impairments and associates them to structural and functional neuroimaging findings (CT, MRI, PET and SPECT). Acute use of alcohol impairs attention, memory, executive functions and visuospatial skills, while chronic abuse causes neurocognitive deficits in memory, learning, visuospatial functions, psychomotor speed processing, executive functions and decision-making, and may lead to persistent amnesic disorder and alcoholic dementia...

OBJECTIVE: To evaluate the role of Gallyas silver staining in the diagnosis of neurodegenerative diseases. METHOD: Modified Gallyas-Braak staining method was used to investigate samples of the brain and spinal cord of 22 cases with neurodegenerative disease including Alzheimer's disease (AD), Parkinson's diseas (PD), Pick's disease, diffuse Lewy body disease (DLBD), progressive supranuclear palsy (PSP), diagnosed by clinical and routine pathologic method. 10 cases without clinical symptoms and pathologic abnormalities of the nervous system served as control...

A 51 year old male, presented with recurrent outbursts of aggression, hypersexuality, sexual indiscretion and hoarding litter following a head injury sustained in a road traffic accident. His premorbid personality was described as energetic but quick tempered. He had no delusions or perceptual disturbances and cognitive functions were intact. Physical and detailed neurological examination were essentially normal, with the exception of disuse atrophy of the lower limb muscles. He lacked insight into his problems...