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   Journal ListNeuropsychiatr Dis Treatv.10; 2014PMC4062551   
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   Neuropsychiatr Dis Treat. 2014; 10: 1045–1055.   
   Published online 2014 Jun 12. doi:  10.2147/NDT.S38821   
   PMCID: PMC4062551   
      
      
   Frontotemporal dementia and primary progressive aphasia, a review   
      
   Howard S Kirshner   
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   Abstract   
   Frontotemporal dementias are neurodegenerative diseases in which symptoms of   
   frontal and/or temporal lobe disease are the first signs of the illness, and   
   as the diseases progress, they resemble a focal left hemisphere process such   
   as stroke or traumatic    
   brain injury, even more than a neurodegenerative disease. Over time, some   
   patients develop a more generalized dementia. Four clinical subtypes   
   characterize the predominant presentations of this illness: behavioral or   
   frontal variant FTD, progressive    
   nonfluent aphasia, semantic dementia, and logopenic primary progressive   
   aphasia. These clinical variants correlate with regional patterns of atrophy   
   on brain imaging studies such as MRI and PET scanning, as well as with   
   biochemical and molecular genetic    
   variants of the disorder. The treatment is as yet only symptomatic, but   
   advances in molecular genetics promise new therapies.   
      
   Keywords: FTD, behavior variant or frontal variant FTD, pick’s disease, PPA,   
   progressive nonfluent aphasia   
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   Introduction   
   Frontotemporal dementia (FTD) is a neurodegenerative disease, one of a series   
   of disorders in which specific populations of neurons die off prematurely.   
   Dementias are defined as syndromes of deterioration of cognitive function,   
   sufficient to cause    
   disability or affect activities of daily living. FTD refers to a family of   
   disorders in which both the clinical syndromes and the regional pathology   
   involve populations of neurons in the frontal and/or temporal lobes on one or   
   both sides of the brain.    
   The hallmark feature of FTD is the presentation with “focalâ€� syndromes   
   such as progressive language dysfunction, or aphasia, or behavioral changes   
   characteristic of frontal lobe disorders. These syndromes mimic focal   
   pathological states such as    
   stroke, brain tumor, or traumatic brain injury. This family of focal, cortical   
   disorders historically has been contrasted with the presentation of   
   Alzheimer’s disease (AD), the most common dementing illness, and often   
   considered to be a “generalized†  
   � dementia. AD most typically presents with a global short-term memory   
   deficit, but this presentation is variable, and AD also affects specific   
   populations of neurons in the cortical association areas, hippocampi, and   
   medial temporal structures, and also    
   the basal forebrain nuclei, such as the nucleus of Meynert. FTD is less common   
   than AD, but it disproportionately affects younger patients, such that it is a   
   major psychological and economic burden for patients and families still in the   
   prime of life. In    
   patients younger than 60 years, FTD is equal to or greater than AD in   
   incidence.   
      
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   Historical background   
   The concept of a “focalâ€� dementia is not new. Arnold Pick, a German   
   physician, published a case report in 1892, describing a focal disorder   
   involving aphasia, as well as psychiatric manifestations.1 In Pick’s words,   
   â€œsimple progressive brain    
   atrophy can lead to symptoms of local disturbance through local accentuation   
   of the diffuse processâ€�. In other words, a neuropathological disorder has to   
   start somewhere in the brain, and then it may spread to other areas and   
   develop into a    
   recognizable dementing illness. This concept of the progression of a   
   neurodegenerative disease from one region of the brain, or one neuron to   
   another, remains central to current thinking on the evolution of dementing   
   diseases in current neuroscience.   
      
   From the 1890s until the 1980s, the field advanced principally via case   
   reports of unusual progressive aphasia and frontal neurobehavioral syndromes.   
   In the 1980s, two patterns of publications appeared, describing    
   eurodegenenerative disorders that    
   presented with either aphasia or frontal lobe syndromes. In the US, the   
   emphasis was on progressive language deterioration, or aphasia. Marsel   
   Mesulam2 reported six patients with gradually progressive aphasia without a   
   more generalized dementia. This    
   disorder, called “primary progressive aphasiaâ€� (PPA), achieved syndrome   
   status in the US and was supported by numerous other case reports and series.   
   Mesulam’s group later defined PPA as a focal disease process, limited to   
   progressive language    
   deterioration and without general cognitive impairment or dementia, for at   
   least 2 years.3–6 Over time, however, some patients with PPA evolve into a   
   more generalized dementia.7,8 Cases of isolated right frontal or temporal   
   degeneration have also been    
   reported, although they have been less frequently recognized than those with   
   either progressive aphasia or frontal lobe behavioral syndromes.9,10 For   
   example, patients have been reported whose initial symptoms involved inability   
   to recognize family    
   members (prosopagnosia) or failure to remember common routes, others with   
   traits such as religiosity, obsessive–compulsive tendencies, uninhibited   
   behavior, and alterations in eating.9 In addition, some have increased   
   artistic tendencies.9   
      
      
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