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Paired Helical Filament
Tauopathies are a class of neurodegenerative diseases characterized by the aggregation of abnormal tau protein. Hyperphosphorylation of tau proteins causes them to dissociate from microtubules and form insoluble aggregates called neurofibrillary tangles. Various neuropathologic phenotypes have been described based on the anatomical regions and cell types involved as well as the unique tau isoforms making up these deposits. The designation 'primary tauopathy' is assigned to disorders where the predominant feature is the deposition of tau protein. Alternatively, diseases exhibiting tau pathologies attributed to different and varied underlying causes are termed 'secondary tauopathies'. Some neuropathologic phenotypes involving tau protein are Alzheimer's disease, frontotemporal dementia, progressive supranuclear palsy, and corticobasal degeneration. Tau protein Tau protein, also called tubulin associated unit or microtubule-associated protein tau (MAPT), is a microtubule-associated pr ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Microtubule
Microtubules are polymers of tubulin that form part of the cytoskeleton and provide structure and shape to eukaryotic cells. Microtubules can be as long as 50 micrometres, as wide as 23 to 27 nanometer, nm and have an inner diameter between 11 and 15 nm. They are formed by the polymerization of a Protein dimer, dimer of two globular proteins, Tubulin#Eukaryotic, alpha and beta tubulin into #Structure, protofilaments that can then associate laterally to form a hollow tube, the microtubule. The most common form of a microtubule consists of 13 protofilaments in the tubular arrangement. Microtubules play an important role in a number of cellular processes. They are involved in maintaining the structure of the cell and, together with microfilaments and intermediate filaments, they form the cytoskeleton. They also make up the internal structure of cilia and flagella. They provide platforms for intracellular transport and are involved in a variety of cellular processes, in ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Frontotemporal Lobar Degeneration
Frontotemporal lobar degeneration (FTLD) is a pathological process that occurs in frontotemporal dementia. It is characterized by atrophy in the frontal lobe and temporal lobe of the brain, with sparing of the parietal and occipital lobes. Common proteinopathies that are found in FTLD include the accumulation of tau proteins and TAR DNA-binding protein 43 (TDP-43). Mutations in the '' C9orf72'' gene have been established as a major genetic contribution of FTLD, although defects in the granulin (GRN) and microtubule-associated proteins (MAPs) are also associated with it. Classification There are 3 main histological subtypes found at post-mortem: * FTLD-tau is characterised by tau positive inclusion bodies often referred to as Pick-bodies. Examples of FTLD-tau include; Pick's disease, corticobasal degeneration, progressive supranuclear palsy. * FTLD-TDP (or FTLD-U ) is characterised by ubiquitin and TDP-43 positive, tau negative, FUS negative inclusion bodies. The pat ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Tuberous Sclerosis
Tuberous sclerosis complex (TSC) is a rare multisystem autosomal dominant genetic disease that causes non-cancerous tumours to grow in the brain and on other vital organs such as the kidneys, heart, liver, eyes, lungs and skin. A combination of symptoms may include seizures, intellectual disability, developmental delay, behavioral problems, skin abnormalities, lung disease, and kidney disease. TSC is caused by a mutation of either of two genes, '' TSC1'' and '' TSC2'', which code for the proteins hamartin and tuberin, respectively, with ''TSC2'' mutations accounting for the majority and tending to cause more severe symptoms. These proteins act as tumor growth suppressors, agents that regulate cell proliferation and differentiation. Prognosis is highly variable and depends on the symptoms, but life expectancy is normal for many. The prevalence of the disease is estimated to be 7 to 12 in 100,000. The disease is often abbreviated to tuberous sclerosis, which refers to t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lead Poisoning
Lead poisoning, also known as plumbism and saturnism, is a type of metal poisoning caused by lead in the body. Symptoms may include abdominal pain, constipation, headaches, irritability, memory problems, infertility, numbness and paresthesia, tingling in the hands and feet. It causes almost 10% of intellectual disability of otherwise unknown cause and can result in behavioral problems. Some of the effects are permanent. In severe cases, anemia, seizures, coma, or death may occur. Exposure to lead can occur by contaminated air, water, dust, food, or consumer products. Lead poisoning poses a significantly increased risk to children and pets as they are far more likely to ingest lead indirectly by chewing on toys or other objects that are coated in lead paint. Additionally, children absorb greater quantities of lead from ingested sources than adults. Exposure at work is a common cause of lead poisoning in adults with certain occupations at particular risk. Diagnosis is typically b ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Subacute Sclerosing Panencephalitis
Subacute sclerosing panencephalitis (SSPE), also known as Dawson disease, is a rare form of progressive brain inflammation caused by a persistent infection with the measles virus. The condition primarily affects children, teens, and young adults. It has been estimated that about 2 in 10,000 people who get measles will eventually develop SSPE. However, a 2016 study estimated that the rate for unvaccinated infants under 15 months was as high as 1 in 609. No cure for SSPE exists, and the condition is almost always fatal. SSPE should not be confused with acute disseminated encephalomyelitis, which can also be caused by the measles virus, but has a very different timing and course. SSPE is caused by the wild-type virus, not by vaccine strains. Signs and symptoms SSPE is characterized by a history of primary measles infection, followed by an asymptomatic period that lasts 7 years on average but can range from 1 month to 27 years. After the asymptomatic period, progressive neurologica ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Meningioangiomatosis
Meningioangiomatosis is a rare disease and tauopathy of the brain. It is characterized by a benign lesion of the leptomeninges usually involving the cerebral cortex, and by leptomeningeal and meningovascular proliferation. Often the patient will present with seizures. The disease may be either sporadic or associated with neurofibromatosis type 2. The lesion is usually focused in one place, though extremely rare multifocal cases have been reported in both adults and children. Biopsy is usually necessary for diagnosis Diagnosis (: diagnoses) is the identification of the nature and cause of a certain phenomenon. Diagnosis is used in a lot of different academic discipline, disciplines, with variations in the use of logic, analytics, and experience, to determine " .... Treatment conventionally involves surgical removal of the lesion. References Further reading * * * Brain disorders {{nervoussystem-disease-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Gangliocytoma
A ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults. They are mixed cell tumors containing both neural ganglionic cells and neural glial cell components. This should not be confused with a gangliocytoma (ganglion cell tumor) which is composed of neurons of variable sizes but contains no glial cells. Classification Gangliogliomas are generally benign WHO grade I tumors; the presence of anaplastic changes in the glial component is considered to represent WHO grade III (anaplastic ganglioglioma). Criteria for WHO grade II have been suggested, but are not established. Malignant transformation of spinal ganglioglioma has been seen in only a select few cases. Poor prognostic factors for adults with gangliogliomas include older age at diagnosis, male sex, and malignant histologic features. Histopathology Histologically, ganglioglioma is composed of both neoplastic glial and ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lytico-bodig Disease
Lytico-Bodig (also Lytigo-bodig) disease, Guam disease, amyotrophic lateral sclerosis-parkinsonism-dementia complex (ALS-PDC), and Western Pacific amyotrophic lateral sclerosis-parkinsonism-dementia complex is a rare, terminal neurodegenerative disease of uncertain etiology endemic to the Chamorro people of the island of Guam in Micronesia. ''Lytigo'' and ''bodig'' are Chamorro language words for two different manifestations of the same condition. ''ALS-PDC'', a term coined by Asao Hirano and colleagues in 1961, reflects its resemblance to amyotrophic lateral sclerosis (ALS), Parkinson's disease, and Alzheimer's disease. First reports of the disease surfaced in three death certificates on Guam in 1904 which made some mention of paralysis. The frequency of cases grew amongst the Chamorro until it was the leading cause of adult death between 1945 and 1956. The incidence rate was 200 per 100,000 per year and it was 100 times more prevalent than in the rest of the world. Neurologist ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Frontotemporal Dementia And Parkinsonism Linked To Chromosome 17
Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) is an autosomal dominant neurodegenerative tauopathy and Parkinson plus syndrome. FTDP-17 is caused by mutations in the MAPT (microtubule associated protein tau) gene located on the q arm of chromosome 17, and has three cardinal features: behavioral and personality changes, cognitive impairment, and motor symptoms. FTDP-17 was defined during the ''International Consensus Conference in Ann Arbor'', Michigan, in 1996. Signs and symptoms FTDP-17 usually appears gradually. Individuals who have reached the fully developed stage of the disease exhibit an array of symptoms that include at least two of the three cardinal features of FTDP-17, which include behavioral and personality disturbances, cognitive deficits, and motor dysfunction. FTDP-17 clinical features differ significantly among affected individuals, regardless of whether they inherit the same or distinct mutations. Even members of the same family ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Chronic Traumatic Encephalopathy
Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease linked to repeated trauma to the head. The encephalopathy symptoms can include behavioral problems, mood problems, and problems with thinking. The disease often gets worse over time and can result in dementia. Most documented cases have occurred in athletes involved in striking-based combat sports, such as boxing, kickboxing, mixed martial arts, and contact sports such as rugby union, rugby league, American football, Australian rules football, professional wrestling, and ice hockey. It is also an issue in association football (soccer), but largely as a result of heading the ball rather than player contact. Other risk factors include being in the military ( combat arms), prior domestic violence, and repeated banging of the head. The exact amount of trauma required for the condition to occur is unknown, and as of 2025 definitive diagnosis can only occur at autopsy. The disease is classified as a tauop ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Amyloid Plaques
Amyloid plaques (also known as neuritic plaques, amyloid beta plaques or senile plaques) are extracellular deposits of amyloid beta (Aβ) protein that present mainly in the grey matter of the brain. Degeneration (medical), Degenerative neuronal elements and an abundance of microglia and astrocytes can be associated with amyloid plaques. Some plaques occur in the brain as a result of Aging brain, aging, but large numbers of plaques and neurofibrillary tangles are characteristic features of Alzheimer's disease. The plaques are highly variable in shape and size; in tissue sections immunohistochemistry, immunostained for Aβ, they comprise a log-normal size distribution curve, with an average plaque area of 400–450 square micrometers (μm2). The smallest plaques (less than 200 μm2), which often consist of diffuse deposits of Aβ, are particularly numerous. Plaques form when Aβ misfolds and aggregates into oligomers and longer polymers, the latter of which are characteristic ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
