Frontotemporal Dementia Disorders

by Dementia and AlzheimersWhile Alzheimer’s disease accounts for approximately 70% of dementia cases, frontotemporal dementia is another type of dementia that affects approximately 50,000-60,000 Americans. FTD is an umbrella term that includes several related disorders, including the following: Behavioral Variant FTD (bvFTD)More than 50% of FTD cases are of the behavioral variant type. The most common symptoms of bvFTD are behavioral or emotional challenges, such as overeating compulsively, an emotionally-detached demeanor, socially inappropriate responses, hyper-sexuality in conversation and thought, apathy and selfishness.Some individuals with bvFTD also experience movement difficulties similar to Parkinson’s disease, including decreased facial expressions, muscle stiffness, weakness and rigidity. Memory loss does not usually develop until the disease has significantly progressed. Diagnosis is often delayed because initially, it may be thought that the individual is just being rude, selfish, depressed or acting out of character.The prognosis for bvFTD is poor. Average life expectancy from onset of symptoms is approximately nine years. Treatment may consist of attempting to use non-drug behavior strategies to curb specific behaviors. Some physicians prescribe antidepressants called selective serotonin reuptake inhibitors (SSRIs), which may help with some of the obsessive-compulsive behaviors such as hoarding or overeating.Primary Progressive Aphasia (PPA)Individuals with PPA can often perform intricate tasks but have difficulty with speech or language. For example, they may be able to build a complicated house but not be able to express themselves well verbally or understand what others are trying to communicate to them. Initial symptoms of PPA include difficulty recalling a specific word or substituting a closely related word such as “take” for “tack.”Other symptoms develop as the disease progresses, such as increasing difficulty in speaking and not understanding words that are read or spoken to them. Individuals often become completely mute.On average, about five years after these initial symptoms involving language, PPA begins to affect memory and other cognitive functions. Interestingly, more men than women experience PPA.PPA can be subdivided into three categories:Semantic PPA: Individuals lose the ability to say certain words, and their ability to recognize others may decline.Agrammatic PPA: Individuals have difficulty forming complete sentences. For example, they may be able to speak using nouns and verbs but not be able to connect them with words like “to” and “from.” As agrammatic PPA progresses, individuals may struggle with forming any words and have trouble with swallowing and muscle control.Logopenic PPA: Individuals may experience difficulty locating the correct words to speak but retain the ability to understand what others are saying to them.Progressive Supranuclear Palsy (PSP)PSP was identified in 1964 as a specific disease and is sometimes referred to as Steele-Richardson-Olszewski Syndrome, named after the scientists who identified it. PSP’s initial symptoms include poor balance, unsteady gait in walking and unexplained falls. Memory problems, emotional outbursts, depression, apathy and irritability are other early symptoms.Eye movement problems are one of the hallmark symptoms of PSP. Because of the lesions that develop in the brain, it becomes difficult to aim the eyes correctly. For example, a person may not be able to maintain eye contact during a conversation. An accurate diagnosis of PSP often is not reached until it progresses to the stage when the eyes begin to function poorly. Until then, it is often misdiagnosed as Parkinson’s.PSP is different from Parkinson’s in several ways. One significant difference is that individuals with PSP often stand straight up or even lean back (causing the risk for falling backwards), while those with Parkinson’s often lean forward and bend over.Treatment options for PSP are limited. Some patients experience temporary improvement in their symptoms with the use of medications like Sinemet, a prescription drug traditionally prescribed to treat Parkinson’s. There also has been some improvement in motor and movement symptoms with Elavil, an antidepressant, though some patients report decreased balance when taking this drug.The prognosis for PSP is poor. Life expectancy may be up to 12 years after initial symptoms, and death usually results from a complication of PSP such as pneumonia. It is estimated that approximately 20,000 Americans have PSP.Corticobasal Degeneration (CBD)CBD, also known as corticobasal ganglionic degeneration, is a rare neurological disorder that shares several symptoms with Parkinson’s. Early symptoms include slow or clumsy movements, tremors or shakiness, muscle weakness and stiffness. The individual with CBD often initially has symptoms only on one side of the body. Later, as the disease progresses, both sides are affected.CBD also affects the ability to speak and understand what others are saying, and can affect short-term memory and the ability to do mathematical calculations. Behavioral components such as compulsions, socially inappropriate behaviors and repetitive actions may also be present.As with the other disorders in the frontotemporal dementia group, treatment of CBD is limited. Medications that may help relieve some of the symptoms include cognitive enhancers usually prescribed for Alzheimer’s patients, such as cholinesterase inhibitors. Other individuals show some improvement in their physical abilities with medications like Sinemet. Physical, occupational and speech therapy may also be ordered by the physician.CBD is a progressive disease that often renders its victims immobile after about five years; within 10 years, the person may succumb to complications such as pneumonia or another infection.