From: hounddog23x@gmail.com   
      
   Rare dementia ravages people in midlife   
      
      
   Stephanie Groves, DHI Media Staff Writer   
   Thursday, January 22, 2015 12:04 AM   
      
   DELPHOS -- Frontotemporal dementia (FTD) is a form of irreversible dementia   
   which affects 50,000-60,000 nationwide, accounts for 2-5 percent of all   
   dementia cases and affects more men than women.   
   The Association for Frontotemporal Degeneration's (AFTD) Executive Director   
   Susan Dickinson said FTD is a neuro-degenerative brain disease that does not   
   affect the memory as Alzheimer's does; instead, it affects parts of the brain   
   that controls behavior,    
   language and movement.   
      
   "People with FTD are affected at a much younger age," she explained. "The   
   average age is 57, 10 years younger than an Alzheimer's diagnosis."   
      
   She said people diagnosed with FTD at this age are still working and parenting   
   kids and teens at home. The onset of the disease often occurs in people   
   between the ages of 50 and 60, but has been seen as early as age 21 and as   
   late as age 80. Roughly 60    
   percent of cases occur in people 45-64 years old.   
      
   "It's an uncommon disease that not many people understand," Dickinson   
   detailed. "Many physicians are unaware of the disease which results in   
   misdiagnoses or delayed diagnosis."   
      
   She said some FTD patients are misdiagnosed with psychiatric disorders,   
   including Alzheimer's and depression. No diagnosis or a delay in diagnosis   
   occurs frequently since the symptoms presented are not memory-based.   
      
   "It's that many more years the patient and their family has less together than   
   if there would have been a diagnosis on the onset," she said. "It affects   
   their personalities and behaviors which can do a lot of harm to relationships."   
      
   FTD is caused by progressive cell degeneration due to protein-filled   
   structures (Pick bodies) that clump together and become toxic to brain cells   
   causing them to die in the frontal lobes (the areas behind the forehead)   
   and/or temporal lobes (the regions    
   behind the ears). Brain cell death leads to tissue shrinkage and reduced   
   function in both lobes which control planning and judgment, emotions, speaking   
   and understanding speech and certain types of movement.   
      
   "The temporal lobes of the brain control language and the frontal lobes affect   
   personality," Dickinson clarified.   
      
   Research from the University of California in San Francisco revealed 60   
   percent of people develop FTD with no known cause and the other 40 percent   
   have a family history of the condition.   
      
   According to AFTD, the majority of FTD cases are not being inherited within   
   the family; however, people with both inherited and sporadic (not inherited)   
   FTD exhibit the same clinical symptoms, which makes evaluation of the family   
   history the most    
   sensitive tool for determining the likelihood of a genetic cause.   
      
   "In 15-20 percent of the cases, there is clearly a hereditary link, a vertical   
   pattern including a grandparent, parent and their offspring. Familial cases   
   where there is no clear hereditary pattern -- but includes at least one other   
   relative who has or    
   had a neurodegenerative disease -- occur 30-35 percent of the time. Perhaps   
   there's a smattering of Parkinson's or another inheritable disease that   
   elevates the risk of FTD," Dickinson said. "The other 50 percent of FTD cases   
   are sporadic (not hereditary)   
    and there is no understood cause."   
      
   Alzheimer's.org reports FTDs can be grouped into three types -- as defined by   
   the earliest symptoms physicians identify when they examine patients -- and   
   includes personality/behavior decline, progressive language decline and   
   progressive motor decline.   
      
   Personality/Behavior decline behavioral variant FTD (bvFTD) is characterized   
   by changes in personality, behavior, and judgement -- which can result in   
   embarrassing social situations -- and a numbing of emotions.   
      
   "It isn't that the patient does not love their family any longer, they just   
   can't show it," Dickinson said. "This disease can rip families apart."   
      
   The progressive language decline Primary Progressive Aphasia (PPA) is marked   
   by early changes in language ability, including speaking, understanding,   
   reading and writing. There are three identified variants.   
      
   * Semantic PPA (semantic dementia) causes a person to slowly lose the ability   
   to understand single words and sometimes the ability to recognize the faces of   
   familiar people and common objects;   
      
   * Agrammatic PPA (progressive nonfluent aphasia) results in a person having   
   more and more trouble producing speech; and   
      
   * Logopenic PPA symptoms includes a person having trouble finding the right   
   words during conversation but they can understand words and sentences. The   
   person does not have problems with grammar.   
      
   Progressive motor decline Corticobasal syndrome (CBS), progressive   
   supranuclear palsy (PSP) and amyotrophis lateral sclerosis (ALS) are   
   characterized by difficulties with physical movement, including the use of one   
   or more limbs, shaking, difficulty    
   walking, frequent falls and poor coordination.   
      
   * CBS can be caused by a gradual atrophy and loss of nerve cells in specific   
   parts of the brain causing progressive loss of the ability to control   
   movement, typically beginning around age 60. The most prominent symptom may be   
   the inability to use the    
   hands or arms to perform a movement despite normal strength (called apraxia);   
      
   * PSP inflicts problems with balance and walking and causes people to move   
   slowly, experience unexplained falls, lose facial expression, and have body   
   stiffness, especially in the neck and upper body -- symptoms similar to those   
   of Parkinson's disease. A    
   hallmark sign of PSP is trouble with eye movements, particularly looking down;   
   and   
      
   * FTD-ALS is a combination of bvFTD and ALS, commonly called Lou Gehrig's   
   disease. Symptoms include the behavioral and/or language changes seen in bvFTD   
   as well as the progressive muscle weakness seen in ALS. Symptoms of either   
   disease may appear first,    
   with other symptoms developing over time.   
      
   "People with FTD typically live 7-8 years and the wide ranging prognosis of   
   2-20 years is the best clinical experts can give," Dickinson said. " There is   
   one exception, the prognosis for a patient with FTD with an ALS overlap is   
   currently 2 years."   
      
   Key differences between FTD and Alzheimer's includes:   
      
   * Most people with FTD are diagnosed in their 50s and early 60s and only about   
   10 percent are diagnosed after age 70. Alzheimer's grows more common with   
   increasing age;   
      
      
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    * Origin: you cannot sedate... all the things you hate (1:229/2)