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Corticobasal Syndrome
Corticobasal syndrome (CBS) is a rare, progressive disease, progressive Parkinson plus syndrome, atypical Parkinsonism syndrome and is a tauopathy related to frontotemporal dementia. CBS is typically caused by the deposit of tau proteins forming in different areas of the brain. Classification CBS is the most common type of corticobasal degeneration (CBD) although the terms CBD and CBS have been used interchangeably in the past. The other three phenotypes of CBD are: * frontal-behavioral dysexecutive-spatial syndrome (FBS) * nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and * progressive supranuclear palsy syndrome (PSPS). Symptoms and signs Symptoms of CBS include apraxia, alien limb phenomenon, frontal deficits, and Extrapyramidal symptoms, extrapyramidal motor symptoms such as myoclonus or rigidity. Movement deficits often begin on one side and progress to the other. Pathophysiology CBD is the pathology underlying approximately 50% of CBS cases. Diagnosis ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Progressive Disease
Progressive disease or progressive illness is a disease or physical ailment whose course in most cases is the worsening, growth, or spread of the disease. This may happen until death, serious debility, or organ failure occurs. Some progressive diseases can be halted and reversed by treatment (surgical, dietary, or lifestyle interventions). Many can be slowed by medical therapy. Some cannot be altered by current treatments. Though the time distinctions are imprecise, diseases can be ''rapidly progressive'' (typically days to weeks) or ''slowly progressive'' (months to years). The time course of a disease affects whether it is considered acute or chronic. By definition, virtually all slowly progressive diseases are also chronic diseases. Biologically, many of these are also referred to as degenerative diseases due to the cellular changes. Not all chronic diseases are progressive: a chronic, non-progressive disease may be referred to as a ''static'' condition. ''Progressive dise ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Multiple System Atrophy
Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction, tremors, slow movement, muscle rigidity, and postural instability (collectively known as parkinsonism) and ataxia. This is caused by progressive degeneration of neurons in several parts of the brain including the basal ganglia, inferior olivary nucleus, and cerebellum. Many people affected by MSA experience dysfunction of the autonomic nervous system, which commonly manifests as orthostatic hypotension, impotence, loss of sweating, dry mouth and urinary retention and incontinence. Palsy of the vocal cords is an important and sometimes initial clinical manifestation of the disorder. A modified form of the alpha-synuclein protein within affected neurons may cause MSA. About 55% of MSA cases occur in men, with those affected first showing symptoms at the age of 50–60 years. MSA often presents with some of the same symptoms as Parkinson's disease. However, those with MSA gen ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cognitive Disorders
Cognitive disorders (CDs), also known as neurocognitive disorders (NCDs), are a category of mental health disorders that primarily affect cognitive abilities including learning, memory, perception, and problem solving. Neurocognitive disorders include delirium, mild neurocognitive disorders, and major neurocognitive disorder (previously known as dementia). They are defined by deficits in cognitive ability that are acquired (as opposed to developmental), typically represent decline, and may have an underlying brain pathology. The DSM-5 defines six key domains of cognitive function: executive function, learning and memory, perceptual-motor function, language, complex attention, and social cognition. Although Alzheimer's disease accounts for the majority of cases of neurocognitive disorders, there are various medical conditions that affect mental functions such as memory, thinking, and the ability to reason, including frontotemporal degeneration, Huntington's disease, dementia with L ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Corticobasal Syndrome
Corticobasal syndrome (CBS) is a rare, progressive disease, progressive Parkinson plus syndrome, atypical Parkinsonism syndrome and is a tauopathy related to frontotemporal dementia. CBS is typically caused by the deposit of tau proteins forming in different areas of the brain. Classification CBS is the most common type of corticobasal degeneration (CBD) although the terms CBD and CBS have been used interchangeably in the past. The other three phenotypes of CBD are: * frontal-behavioral dysexecutive-spatial syndrome (FBS) * nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and * progressive supranuclear palsy syndrome (PSPS). Symptoms and signs Symptoms of CBS include apraxia, alien limb phenomenon, frontal deficits, and Extrapyramidal symptoms, extrapyramidal motor symptoms such as myoclonus or rigidity. Movement deficits often begin on one side and progress to the other. Pathophysiology CBD is the pathology underlying approximately 50% of CBS cases. Diagnosis ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Executive Functions
In cognitive science and neuropsychology, executive functions (collectively referred to as executive function and cognitive control) are a set of cognitive processes that are necessary for the cognitive control of behavior: selecting and successfully monitoring behaviors that facilitate the attainment of chosen goals. Executive functions include basic cognitive processes such as attentional control, cognitive inhibition, inhibitory control, working memory, and cognitive flexibility. Higher-order executive functions require the simultaneous use of multiple basic executive functions and include planning and fluid intelligence (e.g., reasoning and problem-solving). Executive functions gradually develop and change across the lifespan of an individual and can be improved at any time over the course of a person's life. Similarly, these cognitive processes can be adversely affected by a variety of events which affect an individual. Both neuropsychological tests (e.g., the Stroop test ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Progranulin
Granulin is a protein that in humans is encoded by the ''GRN'' gene. Each granulin protein is cleaved from the precursor progranulin, a 593 amino-acid-long and 68.5 kDa protein. While the function of progranulin and granulin have yet to be determined, both forms of the protein have been implicated in development, inflammation, cell proliferation and protein homeostasis. The 2006 discovery of the GRN mutation in a population of patients with frontotemporal dementia has spurred much research in uncovering the function and involvement in disease of progranulin in the body. While there is a growing body of research on progranulin's role in the body, studies on specific granulin residues are still limited. Progranulin Progranulin is the precursor protein for granulin. Cleavage of progranulin produces a variety of active 6 kDa granulin peptides. These smaller cleavage products are named granulin A, granulin B, granulin C, etc. Epithelins 1 and 2 are synonymous with granulins A and B, ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lewy Body Dementia
Lewy body dementias are two similar and common subtypes of dementia—dementia with Lewy bodies (DLB) and Parkinson's disease dementia (PDD). Both are characterized by changes in thinking, movement, behavior, and mood. The two conditions have similar features and may have similar causes, and are believed to belong on a spectrum of Lewy body disease that includes Parkinson's disease. As of 2014, they were more often misdiagnosed than any other common dementia. The exact cause is unknown, but involves widespread deposits of abnormal clumps of protein that form in neurons of the diseased brain. Known as Lewy bodies (discovered in 1912 by Frederic Lewy) and Lewy neurites, these clumps affect both the central nervous system and the autonomic nervous system. The fifth revision of the ''Diagnostic and Statistical Manual of Mental Disorders'' (DSM-5) gives Lewy body disease as the causative subtype of dementia with Lewy bodies, and Parkinson's disease as the causative subtype of Parkins ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pick's Disease
Frontotemporal dementia (FTD), or frontotemporal degeneration disease, or frontotemporal neurocognitive disorder, encompasses several types of dementia involving the progressive degeneration of frontal and temporal lobes. FTDs broadly present as behavioral or language disorders with gradual onsets. The three main subtypes or variant syndromes are a behavioral variant (bvFTD) previously known as ''Pick's disease'', and two variants of primary progressive aphasia – semantic variant (svPPA), and nonfluent variant (nfvPPA). Two rare distinct subtypes of FTD are neuronal intermediate filament inclusion disease (NIFID), and basophilic inclusion body disease. Other related disorders include corticobasal syndrome and FTD with amyotrophic lateral sclerosis (ALS) ''FTD-ALS'' also called ''FTD- MND''. Frontotemporal dementias are mostly early-onset syndromes that are linked to frontotemporal lobar degeneration (FTLD), which is characterized by progressive neuronal loss predominantly i ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) or Lou Gehrig's disease, is a neurodegenerative disease that results in the progressive loss of motor neurons that control voluntary muscles. ALS is the most common type of motor neuron diseases. Early symptoms of ALS include stiff muscles, muscle twitches, and gradual increasing weakness and muscle wasting. ''Limb-onset ALS'' begins with weakness in the arms or legs, while ''bulbar-onset ALS'' begins with difficulty speaking or swallowing. Half of the people with ALS develop at least mild difficulties with thinking and behavior, and about 15% develop frontotemporal dementia. Most people experience pain. The affected muscles are responsible for chewing food, speaking, and walking. Motor neuron loss continues until the ability to eat, speak, move, and finally the ability to breathe is lost. ALS eventually causes paralysis and early death, usually from respiratory failure. Most cases of ALS (a ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Alzheimer's Disease
Alzheimer's disease (AD) is a neurodegeneration, neurodegenerative disease that usually starts slowly and progressively worsens. It is the cause of 60–70% of cases of dementia. The most common early symptom is difficulty in short-term memory, remembering recent events. As the disease advances, symptoms can include primary progressive aphasia, problems with language, Orientation (mental), disorientation (including easily getting lost), mood swings, loss of motivation, self-neglect, and challenging behaviour, behavioral issues. As a person's condition declines, they often withdraw from family and society. Gradually, bodily functions are lost, ultimately leading to death. Although the speed of progression can vary, the typical life expectancy following diagnosis is three to nine years. The cause of Alzheimer's disease is poorly understood. There are many environmental and genetic risk factors associated with its development. The strongest genetic risk factor is from an alle ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lewy Body
Lewy bodies are the inclusion bodies – abnormal aggregations of protein – that develop inside nerve cells affected by Parkinson's disease (PD), the Lewy body dementias (Parkinson's disease dementia and dementia with Lewy bodies (DLB)), and some other disorders. They are also seen in cases of multiple system atrophy, particularly the parkinsonian variant (MSA-P). Lewy bodies appear as spherical masses in the cytoplasm that displace other cell components. For instance, some Lewy bodies tend to displace the nucleus to one side of the cell. There are two main kinds of Lewy bodies: classical and cortical. Lewy bodies may be found in the midbrain (within the substantia nigra) or within the cortex. A classical Lewy body is an eosinophilic cytoplasmic inclusion consisting of a dense core surrounded by a halo of 10-nm-wide radiating fibrils, the primary structural component of which is alpha-synuclein. History In 1910, Fritz Heinrich Lewy was studying in Berlin for his doctor ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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