Pick’s disease is a rare neurodegenerative disorder that causes atrophy of the frontal and temporal lobes of the brain. It is characterized by the destruction of nerve cells and the accumulation of tau proteins. There are three different manifestations of frontotemporal lobar degeneration: frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia.
Pick’s disease is a rare neurodegenerative disorder that causes atrophy, or gradual wasting, of the frontal and temporal lobes of the brain, responsible for higher cognition, language processing and vision, and long-term memory. It is characterized by the destruction of nerve cells in the brain and the accumulation of tau proteins, a protein normally found in neurons of the central nervous system, in concentrations known as “pickup bodies”. Pick’s disease is named after the German neurologist and psychiatrist Arnold Pick, who discovered the condition in 1892. It is not known what causes Pick’s disease, but no genetic basis has been identified.
Pick disease is one of many disorders that can cause frontotemporal lobar degeneration. There are three different manifestations of frontotemporal lobar degeneration: frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia. Semantic dementia is less associated with Pick’s disease than the other subtypes.
Frontotemporal dementia causes two types of symptoms: behavioral symptoms and loss of executive function. Behavioral symptoms may include personality changes, apathy, and extreme lethargy or inappropriate behavior due to complete disinhibition. A patient may become unable to care for himself or may engage in risky and socially unacceptable behavior such as overtly sexual comments or stealing. Loss of executive function is characterized by difficulty performing tasks that involve complex planning and often manifests through impairment of language.
Progressive non-fluent aphasia is a type of speech impairment in which the patient exhibits difficulty speaking. This impairment can take many forms. The patient may have apraxia or difficulty forming speech sounds or may develop a stutter. Other possible forms include anomie, the inability to remember names or nouns; agrammatism, or the inability to speak in normal word order and tenses; and phonemic paraphasia, in which the patient uses the wrong consonant or vowel sounds in his speech. A patient with progressive nonfluent aphasia may have one or more of these symptoms, and the impairment worsens over time.
Semantic dementia was first described by Arnold Pick in 1904, but is not caused by Pick’s disease as often as the other two forms of frontotemporal lobar degeneration. Semantic dementia is characterized by the patient’s inability to remember the meanings of words and visual cues. A patient with semantic dementia may have anomia and impaired understanding of the speech of others. He may also be unable to match semantically related images or often call things by the wrong name.
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