Cerebral Amyloid Angiopathy
Cerebral Amyloid Angiopathy, Familial
Amyloid beta-Peptides
Amyloid
Cerebral Hemorrhage
Alzheimer Disease
Amyloidosis
Brain
Vasculitis, Central Nervous System
Amyloid beta-Protein Precursor
Congo Red
Cystatins
Siderosis
Intracranial Hemorrhages
Apolipoprotein E4
Neurofibrillary Tangles
Presenilin-1
Cerebral Arterial Diseases
Apolipoproteins E
Meninges
Blood Vessels
Cystatin C
Cerebrospinal Fluid Proteins
Mice, Transgenic
Cerebrovascular Disorders
Magnetic Resonance Imaging
Dementia
Dementia, Vascular
Alkenes
Apolipoprotein E2
Peptide Fragments
Serum Amyloid A Protein
Cerebral Cortex
Brain Diseases
Blood-Brain Barrier
Aging
Fatal Outcome
Immunohistochemistry
Disease Models, Animal
Cerebral amyloid angiopathy is a pathogenic lesion in Alzheimer's disease due to a novel presenilin 1 mutation. (1/15)
The dense-cored plaques are considered the pathogenic type of amyloid deposition in Alzheimer's disease brains because of their predominant association with dystrophic neurites. Nevertheless, in > 90% of cases of Alzheimer's disease amyloid is also deposited in cerebral blood vessel walls (congophilic amyloid angiopathy; CAA) but its role in Alzheimer's disease pathogenesis remains enigmatic. Here, we report a family (family GB) in which early-onset Alzheimer's disease was caused by a novel presenilin 1 mutation (L282V). This was unusually severe CAA reminiscent of the Flemish amyloid precursor protein (A692G) mutation we reported previously, which causes Alzheimer's disease and/or cerebral haemorrhages. In family GB, however, the disease presented as typical progressive Alzheimer's disease in the absence of strokes or stroke-like episodes. Similarly, neuroimaging studies and neuropathological examination favoured a degenerative over a vascular dementia. Interestingly, an immunohistochemical study revealed that, similar to causing dense-cored amyloid plaques, CAA also appeared capable of instigating a strong local dystrophic and inflammatory reaction. This was suggested by the observed neuronal loss, the presence of tau- and ubiquitin-positive neurites, micro- and astrogliosis, and complement activation. Together, these data suggest that, like the dense-cored neuritic plaques, CAA might represent a pathogenic lesion that contributes significantly to the progressive neurodegeneration that occurs in Alzheimer's disease. (+info)Vitamin E but not 17beta-estradiol protects against vascular toxicity induced by beta-amyloid wild type and the Dutch amyloid variant. (2/15)
Amyloid beta-peptide (Abeta) fibril deposition on cerebral vessels produces cerebral amyloid angiopathy that appears in the majority of Alzheimer's disease patients. An early onset of a cerebral amyloid angiopathy variant called hereditary cerebral hemorrhage with amyloidosis of the Dutch type is caused by a point mutation in Abeta yielding Abeta(Glu22-->Gln). The present study addresses the effect of amyloid fibrils from both wild-type and mutated Abeta on vascular cells, as well as the putative protective role of antioxidants on amyloid angiopathy. For this purpose, we studied the cytotoxicity induced by Abeta(1-40 Glu22-->Gln) and Abeta(1-40 wild-type) fibrils on human venule endothelial cells and rat aorta smooth muscle cells. We observed that Abeta(Glu22-->Gln) fibrils are more toxic for vascular cells than the wild-type fibrils. We also evaluated the cytotoxicity of Abeta fibrils bound with acetylcholinesterase (AChE), a common component of amyloid deposits. Abeta(1-40 wild-type)-AChE fibrillar complexes, similar to neuronal cells, resulted in an increased toxicity on vascular cells. Previous reports showing that antioxidants are able to reduce the toxicity of Abeta fibrils on neuronal cells prompted us to test the effect of vitamin E, vitamin C, and 17beta-estradiol on vascular damage induced by Abeta(wild-type) and Abeta(Glu22-->Gln). Our data indicate that vitamin E attenuated significantly the Abeta-mediated cytotoxicity on vascular cells, although 17beta-estradiol and vitamin C failed to inhibit the cytotoxicity induced by Abeta fibrils. (+info)RAGE (yin) versus LRP (yang) balance regulates alzheimer amyloid beta-peptide clearance through transport across the blood-brain barrier. (3/15)
Accumulation of amyloid beta-peptide (Abeta) in the central nervous system (CNS) may initiate pathogenic cascades mediating neurovascular and neuronal dysfunctions associated with the development of cerebral beta-amyloidosis and cognitive decline in patients with Alzheimer disease (AD) and with related familial cerebrovascular disorders. Whether Abeta-related pathology in the CNS is reversible or not and what key therapeutic targets are controlling Abeta/amyloid levels in the aging brain remain debatable. In this article, we summarize recent evidence why the receptor for advanced glycation end products and low-density lipoprotein receptor related protein 1 in the vascular CNS barriers are critical for regulation of Abeta homeostasis in the CNS and how altered activities in these 2 receptors at the blood-brain barrier may contribute to the CNS Abeta accumulation resulting in neuroinflammation, disconnect between the cerebral blood flow and metabolism, altered synaptic transmission, neuronal injury, and amyloid deposition into parenchymal and neurovascular lesions. We briefly discuss the potential of advanced glycation end products and low-density lipoprotein receptor related protein 1-based therapeutic strategies to control brain Abeta in animal models of AD and ultimately in patients with AD and related familial cerebrovascular beta-amyloidoses. (+info)Reducing cerebral microvascular amyloid-beta protein deposition diminishes regional neuroinflammation in vasculotropic mutant amyloid precursor protein transgenic mice. (4/15)
Cerebral microvascular amyloid-beta (Abeta) protein deposition is emerging as an important contributory factor to neuroinflammation and dementia in Alzheimer's disease and related familial cerebral amyloid angiopathy disorders. In particular, cerebral microvascular amyloid deposition, but not parenchymal amyloid, is more often correlated with dementia. Recently, we generated transgenic mice (Tg-SwDI) expressing the vasculotropic Dutch (E693Q)/Iowa (D694N) mutant human Abeta precursor protein in brain that accumulate abundant cerebral microvascular fibrillar amyloid deposits. In the present study, our aim was to assess how the presence or absence of fibrillar Abeta deposition in the cerebral microvasculature affects neuroinflammation in Tg-SwDI mice. Using Tg-SwDI mice bred onto an apolipoprotein E gene knock-out background, we found a strong reduction of fibrillar cerebral microvascular Abeta deposition, which was accompanied by a sharp decrease in microvascular-associated neuroinflammatory cells and interleukin-1beta levels. Quantitative immunochemical measurements showed that this reduction of the neuroinflammation occurred in the absence of lowering the levels of total Abeta40/Abeta42 or soluble Abeta oligomers in brain. These findings suggest that specifically reducing cerebral microvascular fibrillar Abeta deposition, in the absence of lowering either the total amount of Abeta or soluble Abeta oligomers in brain, may be sufficient to ameliorate microvascular amyloid-associated neuroinflammation. (+info)Cerebral microvascular amyloid beta protein deposition induces vascular degeneration and neuroinflammation in transgenic mice expressing human vasculotropic mutant amyloid beta precursor protein. (5/15)
Cerebral vascular amyloid beta-protein (Abeta) deposition, also known as cerebral amyloid angiopathy, is a common pathological feature of Alzheimer's disease. Additionally, several familial forms of cerebral amyloid angiopathy exist including the Dutch (E22Q) and Iowa (D23N) mutations of Abeta. Increasing evidence has associated cerebral microvascular amyloid deposition with neuroinflammation and dementia in these disorders. We recently established a transgenic mouse model (Tg-SwDI) that expresses human vasculotropic Dutch/Iowa mutant amyloid beta-protein precursor in brain. Tg-SwDI mice were shown to develop early-onset deposition of Abeta exhibiting high association with cerebral microvessels. Here we present quantitative temporal analysis showing robust and progressive accumulation of cerebral microvascular fibrillar Abeta accompanied by decreased cerebral vascular densities, the presence of apoptotic cerebral vascular cells, and cerebral vascular cell loss in Tg-SwDI mice. Abundant neuroinflammatory reactive astrocytes and activated microglia strongly associated with the cerebral microvascular fibrillar Abeta deposits. In addition, Tg-SwDI mouse brain exhibited elevated levels of the inflammatory cytokines interleukin-1beta and -6. Together, these studies identify the Tg-SwDI mouse as a unique model to investigate selective accumulation of cerebral microvascular amyloid and the associated neuroinflammation. (+info)Inhibition of familial cerebral amyloid angiopathy mutant amyloid beta-protein fibril assembly by myelin basic protein. (6/15)
Deposition of fibrillar amyloid beta-protein (Abeta) in the brain is a prominent pathological feature of Alzheimer disease and related disorders, including familial forms of cerebral amyloid angiopathy (CAA). Mutant forms of Abeta, including Dutch- and Iowa-type Abeta, which are responsible for familial CAA, deposit primarily as fibrillar amyloid along the cerebral vasculature and are either absent or present only as diffuse non-fibrillar plaques in the brain parenchyma. Despite the lack of parenchymal fibril formation in vivo, these CAA mutant Abeta peptides exhibit a markedly increased rate and extent of fibril formation in vitro compared with wild-type Abeta. Based on these conflicting observations, we sought to determine whether brain parenchymal factors that selectively interact with and modulate CAA mutant Abeta fibril assembly exist. Using a combination of immunoaffinity chromatography and mass spectrometry, we identified myelin basic protein (MBP) as a prominent brain parenchymal factor that preferentially binds to CAA mutant Abeta compared with wild-type Abeta. Surface plasmon resonance measurements confirmed that MBP bound more tightly to Dutch/Iowa CAA double mutant Abeta than to wild-type Abeta. Using a combination of biochemical and ultrastructural techniques, we found that MBP inhibited the fibril assembly of CAA mutant Abeta. Together, these findings suggest a possible role for MBP in regulating parenchymal fibrillar Abeta deposition in familial CAA. (+info)Lipoprotein receptor-related protein-1 mediates amyloid-beta-mediated cell death of cerebrovascular cells. (7/15)
Inefficient clearance of A beta, caused by impaired blood-brain barrier crossing into the circulation, seems to be a major cause of A beta accumulation in the brain of late-onset Alzheimer's disease patients and hereditary cerebral hemorrhage with amyloidosis Dutch type. We observed association of receptor for advanced glycation end products, CD36, and low-density lipoprotein receptor (LDLR) with cerebral amyloid angiopathy in both Alzheimer's disease and hereditary cerebral hemorrhage with amyloidosis Dutch type brains and increased low-density lipoprotein receptor-related protein-1 (LRP-1) expression by perivascular cells in cerebral amyloid angiopathy. We investigated if these A beta receptors are involved in A beta internalization and in A beta-mediated cell death of human cerebrovascular cells and astrocytes. Expression of both the LRP-1 and LDLR by human brain pericytes and leptomeningeal smooth muscle cells, but not by astrocytes, increased on incubation with A beta. Receptor-associated protein specifically inhibited A beta-mediated up-regulation of LRP-1, but not of LDLR, and receptor-associated protein also decreased A beta internalization and A beta-mediated cell death. We conclude that especially LRP-1 and, to a minor extent, LDLR are involved in A beta internalization by and A beta-mediated cell death of cerebral perivascular cells. Although perivascular cells may adapt their A beta internalization capacity to the levels of A beta present, saturated LRP-1/LDLR-mediated uptake of A beta results in degeneration of perivascular cells. (+info)E22Q-mutant Abeta peptide (AbetaDutch) increases vascular but reduces parenchymal Abeta deposition. (8/15)
(+info)The term "cerebral" refers to the brain, "amyloid" refers to the abnormal protein deposits, and "angiopathy" refers to the damage caused to the blood vessels. CAA is often associated with other conditions such as Alzheimer's disease, Down syndrome, and other forms of dementia.
CAA is a type of small vessel ischemic disease (SVID), which affects the smaller blood vessels in the brain. The exact cause of CAA is not yet fully understood, but it is thought to be related to a combination of genetic and environmental factors. There is currently no cure for CAA, but researchers are working to develop new treatments to slow its progression and manage its symptoms.
Some common symptoms of CAA include:
* Cognitive decline
* Seizures
* Stroke-like episodes
* Memory loss
* Confusion
* Difficulty with coordination and balance
If you suspect you or a loved one may be experiencing symptoms of CAA, it is important to speak with a healthcare professional for proper diagnosis and treatment. A thorough medical history and physical examination, along with imaging tests such as MRI or CT scans, can help confirm the presence of CAA.
While there is no cure for CAA, there are several treatment options available to manage its symptoms and slow its progression. These may include medications to control seizures, improve cognitive function, and reduce inflammation. In some cases, surgery or endovascular procedures may be necessary to repair or remove damaged blood vessels.
It is important to note that CAA is a complex condition, and its management requires a multidisciplinary approach involving neurologists, geriatricians, radiologists, and other healthcare professionals. With proper diagnosis and treatment, however, many individuals with CAA are able to lead active and fulfilling lives.
The term 'familial' refers to the fact that CAA is inherited in an autosomal dominant pattern, meaning that a single copy of the mutated gene is enough to cause the condition. This means that if one parent has the mutation, each child has a 50% chance of inheriting the mutated gene. The age of onset and severity of symptoms can vary widely among individuals with CAA, even within the same family.
There are several types of CAA, including:
1. Cerebral Amyloid Angiopathy, type 1 (CAA1): This is the most common form of CAA and is caused by mutations in the PROAPLIN1 gene.
2. Cerebral Amyloid Angiopathy, type 2 (CAA2): This form of CAA is less common and is caused by mutations in the PROAPLIN2 gene.
3. Cerebral Amyloid Angiopathy, type 3 (CAA3): This rare form of CAA is caused by mutations in the AGT gene.
There is currently no cure for CAA, but research is ongoing to develop new treatments and therapies to slow or halt the progression of the disease. Management of symptoms and prevention of complications are key components of care for individuals with CAA.
Symptoms of cerebral hemorrhage may include sudden severe headache, confusion, seizures, weakness or numbness in the face or limbs, and loss of consciousness. The condition is diagnosed through a combination of physical examination, imaging tests such as CT or MRI scans, and laboratory tests to determine the cause of the bleeding.
Treatment for cerebral hemorrhage depends on the location and severity of the bleeding, as well as the underlying cause. Medications may be used to control symptoms such as high blood pressure or seizures, while surgery may be necessary to repair the ruptured blood vessel or relieve pressure on the brain. In some cases, the condition may be fatal, and immediate medical attention is essential to prevent long-term damage or death.
Some of the most common complications associated with cerebral hemorrhage include:
1. Rebleeding: There is a risk of rebleeding after the initial hemorrhage, which can lead to further brain damage and increased risk of death.
2. Hydrocephalus: Excess cerebrospinal fluid can accumulate in the brain, leading to increased intracranial pressure and potentially life-threatening complications.
3. Brain edema: Swelling of the brain tissue can occur due to the bleeding, leading to increased intracranial pressure and potentially life-threatening complications.
4. Seizures: Cerebral hemorrhage can cause seizures, which can be a sign of a more severe injury.
5. Cognitive and motor deficits: Depending on the location and severity of the bleeding, cerebral hemorrhage can result in long-term cognitive and motor deficits.
6. Vision loss: Cerebral hemorrhage can cause vision loss or blindness due to damage to the visual cortex.
7. Communication difficulties: Cerebral hemorrhage can cause difficulty with speech and language processing, leading to communication difficulties.
8. Behavioral changes: Depending on the location and severity of the bleeding, cerebral hemorrhage can result in behavioral changes, such as irritability, agitation, or apathy.
9. Infection: Cerebral hemorrhage can increase the risk of infection, particularly if the hemorrhage is caused by a ruptured aneurysm or arteriovenous malformation (AVM).
10. Death: Cerebral hemorrhage can be fatal, particularly if the bleeding is severe or if there are underlying medical conditions that compromise the patient's ability to tolerate the injury.
The term "amyloid" refers specifically to the type of protein aggregate that forms these plaques, and is derived from the Greek word for "flour-like." Amyloidosis is the general term used to describe the condition of having amyloid deposits in the body, while Alzheimer's disease is a specific type of amyloidosis that is characterized by the accumulation of beta-amyloid peptides in the brain.
Plaques, amyloid play a central role in the pathogenesis of many neurodegenerative diseases, and understanding their formation and clearance is an area of ongoing research. In addition to their role in Alzheimer's disease, amyloid plaques have been implicated in other conditions such as cerebral amyloid angiopathy, primary lateral sclerosis, and progressive supranuclear palsy.
Plaques, amyloid are composed of a variety of proteins, including beta-amyloid peptides, tau protein, and apolipoprotein E (apoE). The composition and structure of these plaques can vary depending on the underlying disease, and their presence is often associated with inflammation and oxidative stress.
In addition to their role in neurodegeneration, amyloid plaques have been implicated in other diseases such as type 2 diabetes and cardiovascular disease. The accumulation of amyloid fibrils in these tissues can contribute to the development of insulin resistance and atherosclerosis, respectively.
Overall, plaques, amyloid are a complex and multifaceted area of research, with many open questions remaining about their formation, function, and clinical implications. Ongoing studies in this field may provide valuable insights into the pathogenesis of various diseases and ultimately lead to the development of novel therapeutic strategies for these conditions.
In conclusion, plaques, amyloid are a hallmark of several neurodegenerative diseases, including Alzheimer's disease, and have been associated with inflammation, oxidative stress, and neurodegeneration. The composition and structure of these plaques can vary depending on the underlying disease, and their presence is often linked to the progression of the condition. Furthermore, amyloid plaques have been implicated in other diseases such as type 2 diabetes and cardiovascular disease, highlighting their potential clinical significance beyond neurodegeneration. Ongoing research into the mechanisms of amyloid plaque formation and clearance may lead to the development of novel therapeutic strategies for these conditions.
The symptoms of Alzheimer's disease can vary from person to person and may progress slowly over time. Early symptoms may include memory loss, confusion, and difficulty with problem-solving. As the disease progresses, individuals may experience language difficulties, visual hallucinations, and changes in mood and behavior.
There is currently no cure for Alzheimer's disease, but there are several medications and therapies that can help manage its symptoms and slow its progression. These include cholinesterase inhibitors, memantine, and non-pharmacological interventions such as cognitive training and behavioral therapy.
Alzheimer's disease is a significant public health concern, affecting an estimated 5.8 million Americans in 2020. It is the sixth leading cause of death in the United States, and its prevalence is expected to continue to increase as the population ages.
There is ongoing research into the causes and potential treatments for Alzheimer's disease, including studies into the role of inflammation, oxidative stress, and the immune system. Other areas of research include the development of biomarkers for early detection and the use of advanced imaging techniques to monitor progression of the disease.
Overall, Alzheimer's disease is a complex and multifactorial disorder that poses significant challenges for individuals, families, and healthcare systems. However, with ongoing research and advances in medical technology, there is hope for improving diagnosis and treatment options in the future.
There are several types of amyloidosis, each with different causes and symptoms. The most common types include:
1. Primary amyloidosis: This type is caused by the production of abnormal proteins in the bone marrow. It mainly affects older adults and can lead to symptoms such as fatigue, weight loss, and numbness or tingling in the hands and feet.
2. Secondary amyloidosis: This type is caused by other conditions, such as rheumatoid arthritis, tuberculosis, or inflammatory bowel disease. It can also be caused by long-term use of certain medications, such as antibiotics or chemotherapy.
3. Familial amyloid polyneuropathy: This type is inherited and affects the nerves in the body, leading to symptoms such as muscle weakness, numbness, and pain.
4. Localized amyloidosis: This type affects a specific area of the body, such as the tongue or the skin.
The symptoms of amyloidosis can vary depending on the organs affected and the severity of the condition. Some common symptoms include:
1. Fatigue
2. Weakness
3. Pain
4. Numbness or tingling in the hands and feet
5. Swelling in the legs, ankles, and feet
6. Difficulty with speech or swallowing
7. Seizures
8. Heart problems
9. Kidney failure
10. Liver failure
The diagnosis of amyloidosis is based on a combination of physical examination, medical history, laboratory tests, and imaging studies. Laboratory tests may include blood tests to measure the levels of certain proteins in the body, as well as biopsies to examine tissue samples under a microscope. Imaging studies, such as X-rays, CT scans, and MRI scans, may be used to evaluate the organs affected by the condition.
There is no cure for amyloidosis, but treatment can help manage the symptoms and slow the progression of the disease. Treatment options may include:
1. Medications to control symptoms such as pain, swelling, and heart problems
2. Chemotherapy to reduce the production of abnormal proteins
3. Autologous stem cell transplantation to replace damaged cells with healthy ones
4. Dialysis to remove excess fluids and waste products from the body
5. Nutritional support to ensure adequate nutrition and hydration
6. Physical therapy to maintain muscle strength and mobility
7. Supportive care to manage pain, improve quality of life, and reduce stress on the family.
In conclusion, amyloidosis is a complex and rare group of diseases that can affect multiple organs and systems in the body. Early diagnosis and treatment are essential to managing the symptoms and slowing the progression of the disease. It is important for patients with suspected amyloidosis to seek medical attention from a specialist, such as a hematologist or nephrologist, for proper evaluation and treatment.
The exact cause of CNS vasculitis is not fully understood, but it is believed to be an autoimmune disorder, meaning that the immune system mistakenly attacks healthy tissues in the CNS. The condition can occur at any age, but it most commonly affects adults between the ages of 40 and 60.
Symptoms of CNS vasculitis can vary depending on the location and severity of the inflammation, but may include:
* Headaches
* Confusion
* Memory loss
* Seizures
* Weakness or numbness in the limbs
* Vision problems
* Speech difficulties
Diagnosis of CNS vasculitis typically involves a combination of physical examination, medical history, and diagnostic tests such as MRI or CT scans, lumbar puncture, and blood tests. Treatment options for CNS vasculitis vary depending on the severity of the condition and may include corticosteroids, immunosuppressive drugs, and plasmapheresis. In severe cases, surgery may be necessary to relieve pressure on the brain or spinal cord.
Overall, CNS vasculitis is a serious condition that can have significant neurological consequences if left untreated. Early diagnosis and aggressive treatment are critical to prevent long-term damage and improve outcomes for patients with this condition.
Here is a list of 10 diseases related to 'Siderosis':
1. Hemochromatosis - A genetic disorder that causes the body to absorb too much iron from food, leading to siderosis and damage to various organs.
2. Sickle Cell Disease - A group of inherited blood disorders that can cause anemia, pain, and a range of complications including siderosis.
3. Thalassemia - A genetic disorder that affects the production of hemoglobin, leading to anemia and other complications such as siderosis.
4. Chronic Blood Transfusions - Repeated blood transfusions can lead to an excessive accumulation of iron in the body, causing siderosis.
5. Iron Overload - Excessive intake of iron-rich foods or supplements can cause iron overload and siderosis.
6. Anemia of Chronic Disease - A type of anemia that occurs in people with chronic diseases such as rheumatoid arthritis, cancer, and HIV/AIDS, which can lead to siderosis.
7. Inherited Iron Overload Disorders - A group of rare genetic disorders that cause the body to absorb too much iron from food, leading to siderosis and other complications.
8. Acquired Iron Overload Disorders - Conditions such as chronic hepatitis C infection and hemodialysis can lead to excessive iron accumulation in the body, causing siderosis.
9. Chronic Inflammation - Prolonged inflammation can lead to an increase in iron absorption, causing siderosis.
10. Poor Nutrition - Consuming a diet low in iron can lead to siderosis over time.
It's important to note that siderosis is a rare condition and these causes are not exhaustive. If you suspect you or someone you know may have siderosis, it's important to consult with a healthcare professional for proper diagnosis and treatment.
There are several types of intracranial hemorrhage, including:
1. Cerebral hemorrhage: Bleeding within the cerebral tissue itself, which can cause damage to brain cells and lead to a variety of complications.
2. Subarachnoid hemorrhage: Bleeding between the brain and the thin membrane that covers it (the meninges), which can cause severe headaches and other symptoms.
3. Epidural hemorrhage: Bleeding between the dura mater, a protective layer of tissue surrounding the brain, and the skull.
4. Subdural hemorrhage: Bleeding between the dura mater and the arachnoid membrane, which can cause severe headaches and other symptoms.
The symptoms of intracranial hemorrhage can vary depending on the location and severity of the bleeding, but may include:
* Sudden, severe headache
* Nausea and vomiting
* Confusion and disorientation
* Weakness or numbness in the face, arm, or leg
* Seizures
* Loss of consciousness
Diagnosis is typically made through a combination of physical examination, imaging tests (such as CT or MRI scans), and laboratory tests to determine the cause of the hemorrhage. Treatment depends on the location and severity of the bleeding, but may include medications to control symptoms, surgery to repair the source of the bleeding, or other interventions as needed.
Types of cerebral arterial diseases include:
1. Cerebral vasospasm: A temporary constriction of the blood vessels in the brain, often seen after subarachnoid hemorrhage (bleeding in the space surrounding the brain).
2. Moyamoya disease: A rare condition caused by narrowing or blockage of the internal carotid artery and its branches, leading to decreased blood flow to the brain.
3. Cerebral amyloid angiopathy: A condition in which abnormal protein deposits accumulate in the walls of blood vessels supplying the brain, leading to inflammation and damage.
4. Cerebral infarction (stroke): The loss of brain tissue due to reduced blood flow or a blockage in an artery supplying the brain.
5. Cerebral hemorrhage: Bleeding in the brain, often due to rupture of a blood vessel or aneurysm.
Symptoms of cerebral arterial diseases can vary depending on the location and severity of the affected blood vessels, but may include headache, confusion, weakness or numbness in the face or limbs, difficulty speaking or understanding speech, and vision problems.
Diagnosis of cerebral arterial diseases typically involves a combination of physical examination, medical history, neuroimaging studies (such as CT or MRI scans), and angiography (a test that uses dye and X-rays to visualize the blood vessels in the brain).
Treatment options for cerebral arterial diseases depend on the underlying cause and severity of the condition, but may include medications to control blood pressure, cholesterol levels, or inflammation, as well as surgical interventions such as endarterectomy (removing plaque from the affected blood vessel) or aneurysm repair. In some cases, cerebral arterial diseases may be treated with a combination of medical and surgical therapies.
Complications of cerebral arterial diseases can include stroke, seizures, and cognitive decline. With prompt and appropriate treatment, however, many individuals with cerebral arterial diseases can experience significant improvement in symptoms and quality of life.
1. Stroke: A stroke occurs when the blood supply to the brain is interrupted, either due to a blockage or a rupture of the blood vessels. This can lead to cell death and permanent brain damage.
2. Cerebral vasospasm: Vasospasm is a temporary constriction of the blood vessels in the brain, which can occur after a subarachnoid hemorrhage (bleeding in the space surrounding the brain).
3. Moyamoya disease: This is a rare condition caused by narrowing or blockage of the internal carotid artery and its branches. It can lead to recurrent transient ischemic attacks (TIs) or stroke.
4. Cerebral amyloid angiopathy: This is a condition where abnormal protein deposits accumulate in the blood vessels of the brain, leading to inflammation and bleeding.
5. Cavernous malformations: These are abnormal collections of blood vessels in the brain that can cause seizures, headaches, and other symptoms.
6. Carotid artery disease: Atherosclerosis (hardening) of the carotid arteries can lead to a stroke or TIAs.
7. Vertebrobasilar insufficiency: This is a condition where the blood flow to the brain is reduced due to narrowing or blockage of the vertebral and basilar arteries.
8. Temporal lobe dementia: This is a type of dementia that affects the temporal lobe of the brain, leading to memory loss and other cognitive symptoms.
9. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL): This is a rare genetic disorder that affects the blood vessels in the brain, leading to recurrent stroke-like events.
10. Moyamoya disease: This is a rare condition caused by narrowing or blockage of the internal carotid artery and its branches, leading to decreased blood flow to the brain and increased risk of stroke.
It's important to note that this list is not exhaustive and there may be other causes of stroke and TIAs that are not included here. A proper diagnosis can only be made by a qualified medical professional after conducting a thorough examination and reviewing the individual's medical history.
There are several types of dementia, each with its own set of symptoms and characteristics. Some common types of dementia include:
* Alzheimer's disease: This is the most common form of dementia, accounting for 50-70% of all cases. It is a progressive disease that causes the death of brain cells, leading to memory loss and cognitive decline.
* Vascular dementia: This type of dementia is caused by problems with blood flow to the brain, often as a result of a stroke or small vessel disease. It can cause difficulty with communication, language, and visual-spatial skills.
* Lewy body dementia: This type of dementia is characterized by the presence of abnormal protein deposits called Lewy bodies in the brain. It can cause a range of symptoms, including memory loss, confusion, hallucinations, and difficulty with movement.
* Frontotemporal dementia: This is a group of diseases that affect the front and temporal lobes of the brain, leading to changes in personality, behavior, and language.
The symptoms of dementia can vary depending on the underlying cause, but common symptoms include:
* Memory loss: Difficulty remembering recent events or learning new information.
* Communication and language difficulties: Struggling to find the right words or understand what others are saying.
* Disorientation: Getting lost in familiar places or having difficulty understanding the time and date.
* Difficulty with problem-solving: Trouble with planning, organizing, and decision-making.
* Mood changes: Depression, anxiety, agitation, or aggression.
* Personality changes: Becoming passive, suspicious, or withdrawn.
* Difficulty with movement: Trouble with coordination, balance, or using utensils.
* Hallucinations: Seeing or hearing things that are not there.
* Sleep disturbances: Having trouble falling asleep or staying asleep.
The symptoms of dementia can be subtle at first and may progress slowly over time. In the early stages, they may be barely noticeable, but as the disease progresses, they can become more pronounced and interfere with daily life. It is important to seek medical advice if you or a loved one is experiencing any of these symptoms, as early diagnosis and treatment can help improve outcomes.
The symptoms of vascular dementia can vary depending on the location and severity of the damage to the brain, but common symptoms include:
* Memory loss, such as difficulty remembering recent events or learning new information
* Confusion and disorientation
* Difficulty with communication, including trouble finding the right words or understanding what others are saying
* Difficulty with problem-solving, decision-making, and judgment
* Mood changes, such as depression, anxiety, or agitation
* Personality changes, such as becoming more passive or suspicious
* Difficulty with coordination and movement, including trouble walking or balance
Vascular dementia can be caused by a variety of conditions that affect the blood vessels in the brain, including:
* Stroke or transient ischemic attack (TIA, or "mini-stroke")
* Small vessel disease, such as tiny strokes or changes in the blood vessels that occur over time
* Moyamoya disease, a rare condition caused by narrowing or blockage of the internal carotid artery and its branches
* Cerebral amyloid angiopathy, a condition in which abnormal protein deposits build up in the blood vessels of the brain
* Other conditions that can cause reduced blood flow to the brain, such as high blood pressure, diabetes, or cardiovascular disease
There is no cure for vascular dementia, but there are several treatment options available to help manage its symptoms and slow its progression. These may include medications to improve memory and cognitive function, physical therapy to maintain mobility and strength, and lifestyle changes such as a healthy diet and regular exercise. In some cases, surgery or endovascular procedures may be recommended to treat the underlying cause of the dementia, such as a stroke or blocked blood vessel.
It is important for individuals with vascular dementia to receive timely and accurate diagnosis and treatment, as well as ongoing support and care from healthcare professionals, family members, and caregivers. With appropriate management, many people with vascular dementia are able to maintain their independence and quality of life for as long as possible.
Some common types of brain diseases include:
1. Neurodegenerative diseases: These are progressive conditions that damage or kill brain cells over time, leading to memory loss, cognitive decline, and movement disorders. Examples include Alzheimer's disease, Parkinson's disease, Huntington's disease, and amyotrophic lateral sclerosis (ALS).
2. Stroke: This occurs when blood flow to the brain is interrupted, leading to cell death and potential long-term disability.
3. Traumatic brain injury (TBI): This refers to any type of head injury that causes damage to the brain, such as concussions, contusions, or penetrating wounds.
4. Infections: Viral, bacterial, and fungal infections can all affect the brain, leading to a range of symptoms including fever, seizures, and meningitis.
5. Tumors: Brain tumors can be benign or malignant and can cause a variety of symptoms depending on their location and size.
6. Cerebrovascular diseases: These conditions affect the blood vessels of the brain, leading to conditions such as aneurysms, arteriovenous malformations (AVMs), and Moyamoya disease.
7. Neurodevelopmental disorders: These are conditions that affect the development of the brain and nervous system, such as autism spectrum disorder, ADHD, and intellectual disability.
8. Sleep disorders: Conditions such as insomnia, narcolepsy, and sleep apnea can all have a significant impact on brain function.
9. Psychiatric disorders: Mental health conditions such as depression, anxiety, and schizophrenia can affect the brain and its functioning.
10. Neurodegenerative with brain iron accumulation: Conditions such as Parkinson's disease, Alzheimer's disease, and Huntington's disease are characterized by the accumulation of abnormal proteins and other substances in the brain, leading to progressive loss of brain function over time.
It is important to note that this is not an exhaustive list and there may be other conditions or factors that can affect the brain and its functioning. Additionally, many of these conditions can have a significant impact on a person's quality of life, and it is important to seek medical attention if symptoms persist or worsen over time.
1) They share similarities with humans: Many animal species share similar biological and physiological characteristics with humans, making them useful for studying human diseases. For example, mice and rats are often used to study diseases such as diabetes, heart disease, and cancer because they have similar metabolic and cardiovascular systems to humans.
2) They can be genetically manipulated: Animal disease models can be genetically engineered to develop specific diseases or to model human genetic disorders. This allows researchers to study the progression of the disease and test potential treatments in a controlled environment.
3) They can be used to test drugs and therapies: Before new drugs or therapies are tested in humans, they are often first tested in animal models of disease. This allows researchers to assess the safety and efficacy of the treatment before moving on to human clinical trials.
4) They can provide insights into disease mechanisms: Studying disease models in animals can provide valuable insights into the underlying mechanisms of a particular disease. This information can then be used to develop new treatments or improve existing ones.
5) Reduces the need for human testing: Using animal disease models reduces the need for human testing, which can be time-consuming, expensive, and ethically challenging. However, it is important to note that animal models are not perfect substitutes for human subjects, and results obtained from animal studies may not always translate to humans.
6) They can be used to study infectious diseases: Animal disease models can be used to study infectious diseases such as HIV, TB, and malaria. These models allow researchers to understand how the disease is transmitted, how it progresses, and how it responds to treatment.
7) They can be used to study complex diseases: Animal disease models can be used to study complex diseases such as cancer, diabetes, and heart disease. These models allow researchers to understand the underlying mechanisms of the disease and test potential treatments.
8) They are cost-effective: Animal disease models are often less expensive than human clinical trials, making them a cost-effective way to conduct research.
9) They can be used to study drug delivery: Animal disease models can be used to study drug delivery and pharmacokinetics, which is important for developing new drugs and drug delivery systems.
10) They can be used to study aging: Animal disease models can be used to study the aging process and age-related diseases such as Alzheimer's and Parkinson's. This allows researchers to understand how aging contributes to disease and develop potential treatments.
Cerebral amyloid angiopathy
Familial Danish dementia
Tubulin beta-4A chain
TUBA1B
Organ-limited amyloidosis
Amyloid beta
List of MeSH codes (C16)
List of MeSH codes (C10)
List of diseases (C)
Amyloid-beta precursor protein
List of OMIM disorder codes
Chromosome 21
List of MeSH codes (C18)
Alnylam Pharmaceuticals
List of MeSH codes (C14)
Early-onset Alzheimer's disease
Lipohyalinosis
Susceptibility weighted imaging
Cerebrovascular disease
Plasma gelsolin
Hereditary cerebral amyloid angiopathy: MedlinePlus Genetics
MeSH Browser
Bruce W. Chesebro, M.D. | NIH: National Institute of Allergy and Infectious Diseases
Lack of ApoE inhibits ADan amyloidosis in a mouse model of familial Danish dementia - PubMed
MESH TREE NUMBER CHANGES - 2012 MeSH. August 19, 2011
MedlinePlus: Genetic Conditions
DeCS
MeSH Browser
HuGE Navigator|Phenopedia|PHGKB
A Rare Case Report of Cerebral Amyloid Angiopathy Related Inflammation Presenting as Acute Aphasia | Insight Medical Publishing
Pesquisa | Biblioteca Virtual em Saúde - BRASIL
Alzheimer Disease in Down Syndrome: Overview, Pathophysiology/Risk Factors, Epidemiology
Bruce W. Chesebro, M.D. | Principal Investigators | NIH Intramural Research Program
September 24, 2020<...
Amyloidosis: MedlinePlus
Amyloid neuropathies. Medical search
ApoE isoforms differentially regulates cleavage and secretion of BDNF | Molecular Brain | Full Text
Amyloidosis: Definition of Amyloid and Amyloidosis, Classification Systems, Systemic Amyloidoses
MESH TREE NUMBER CHANGES - 2007 MeSH. September 14, 2006
MESH TREE NUMBER CHANGES - 2012 MeSH. August 19, 2011
MESH TREE NUMBER CHANGES - 2012 MeSH. August 19, 2011
Testy yet trying: Download/Print Free Speech Articulation Materials
Broadford Primary: Cancer Research UK Race for Life - 4 days to go!
Menkes Kinky Hair Syndrome (medical concept explorer)
Lysosomal Storage Diseases, Nervous System | Profiles RNS
Charting vascular contributions to white matter disease in familial Alzheimers disease and CADASIL | NIH
Find Your Program Officer-Decision Tree | National Institute of Neurological Disorders and Stroke
INCLUDE Project/Down Syndrome Research Plan | National Institutes of Health (NIH)
Insights on altered mitochondrial function and dynamics in the pathogenesis of neurodegeneration | Translational...
George Carlson | UCSF Profiles
Amyloidosis9
- Amyloidosis occurs when abnormal proteins called amyloids build up and form deposits. (nih.gov)
- Amyloidosis results from the accumulation of pathogenic amyloids-most of which are aggregates of misfolded proteins-in a variety of tissues. (medscape.com)
- Amyloidosis is a clinical disorder caused by extracellular and/or intracellular deposition of insoluble abnormal amyloid fibrils that alter the normal function of tissues. (medscape.com)
- [ 5 ] Only 10% of amyloidosis deposits consist of components such as glycosaminoglycans (GAGs), apolipoprotein-E (apoE), and serum amyloid P-component (SAP), while nearly 90% of the deposits consist of amyloid fibrils that are formed by the aggregation of misfolded proteins. (medscape.com)
- Some classification systems included myeloma-associated, familial, and localized amyloidosis. (medscape.com)
- The modern era of amyloidosis classification began in the late 1960s with the development of methods to solubilize amyloid fibrils. (medscape.com)
- Amyloid plaques and amyloid angiopathy also characterize the brains of individuals with Trisomy 21 (Down's Syndrome) and Hereditary Cerebral Hemorrhage with Amyloidosis of the Dutch-Type (HCHWA-D). (justia.com)
- In order to elucidate the pathogenesis ofAβ2M amyloidosis, we established an experimental system to study the mechanism of amyloid fibril formation or degradation in vitro. (sagepub.com)
- Chickens (Gallus gallus domesticus) can suffer from AA amyloidosis featuring the joints as major targets of amyloid accumulation. (sagepub.com)
Precursor protein7
- The APP gene provides instructions for making a protein called amyloid precursor protein. (medlineplus.gov)
- In the brain, the amyloid precursor protein plays a role in the development and maintenance of nerve cells (neurons). (medlineplus.gov)
- Additionally, the ITM2B protein may be involved in processing the amyloid precursor protein. (medlineplus.gov)
- The etiopathogenesis of caari is very closely related to alzhemiers disease which is due to deposition of amyloid precursor protein in perivascular area and thus causing cererbal edema. (imedpub.com)
- All recognized mutations for AD are associated with increased deposition of amyloid-beta (Abeta), a peptide fragment comprising 39-43 amino acids that derive from the catabolism of the amyloid precursor protein (APP) molecule. (medscape.com)
- It is cleaved by AMYLOID PRECURSOR PROTEIN SECRETASES to produce peptides of varying amino acid lengths. (lookformedical.com)
- Molecular biological and protein chemical analyses have shown that Aβ is a small fragment of a much larger precursor protein, referred to as the β-amyloid precursor protein (APP) (see, e.g. (justia.com)
Alzheimer's6
- This model replicates the prion/TSE agent and has extensive amyloid deposits in the brain with cerebral amyloid angiopathy similar to human brain amyloid diseases including Alzheimer's disease. (nih.gov)
- An amyloid fibrillar form of these peptides is the major component of amyloid plaques found in individuals with Alzheimer's disease and in aged individuals with trisomy 21 (DOWN SYNDROME). (lookformedical.com)
- The abnormal accumulation of amyloid-beta peptide is believed to cause malfunctioning of neurons in the Alzheimer's disease brain. (biomedcentral.com)
- Recent findings suggest that soluble amyloid-beta oligomers may represent the primary pathological species in Alzheimer's disease and the most toxic form that impairs synaptic and thus neuronal function. (biomedcentral.com)
- These data suggest that acyl peptide hydrolase is involved in the degradation of oligomeric amyloid-beta, an activity that, if induced, might present a new tool for therapy aimed at reducing neurodegeneration in the Alzheimer's brain. (biomedcentral.com)
- Alzheimer's disease (AD) is a neurodegenerative disorder resulting from the pathological processing of APP that leads to the accumulation of amyloid-beta (Aβ) peptide, neuronal loss, synaptic dysfunction, inappropriate levels of neurotransmitters, and finally, to irreversible and progressive memory loss. (biomedcentral.com)
Deposits in the blood ves1
- Hereditary cerebral amyloid angiopathy is a condition characterized by an abnormal buildup of protein clumps called amyloid deposits in the blood vessels in the brain, causing vascular disease (angiopathy). (medlineplus.gov)
Deposition6
- ApoE plays a critical role in amyloid-β (Aβ) accumulation in AD, and genetic deletion of the murine ApoE gene in mouse models results in a decrease or inhibition of Aβ deposition. (nih.gov)
- Familial Danish dementia (FDD) is an autosomal dominant neurodegenerative disease characterized by the presence of widespread parenchymal and vascular Danish amyloid (ADan) deposition and neurofibrillary tangles. (nih.gov)
- It is a form of amyloid vasculopathy in which there is pathological deposition of amyloid b precursor in tunica intima and media of small and medium sized arteries of brain parenchyma mainly involving cortical and subcortical white matter which leads to their increased fragility and thus more susceptible to rupture and cause microhemmorhage or sometimes even macrohemmohage or it may lead to occlusion of arteries causing infarct [ 2 ]. (imedpub.com)
- Disorders of the peripheral nervous system associated with the deposition of AMYLOID in nerve tissue. (lookformedical.com)
- A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. (lookformedical.com)
- Polymorphisms that slightly vary native peptides or inflammatory processes set the stage for abnormal protein folding and amyloid fibril deposition. (medscape.com)
Peptide8
- There is considerable overlap of pathogenesis of alzhemiers disease and cerebral amyloid angiopathy with the antibodies directed against vascular Amyloid β (Aβ)-which is the same 1-42 amino acid Aβ peptide found in Alzheimer 's Disease (AD) plaques. (imedpub.com)
- A 39-42 amino acid peptide, AMYLOID BETA-PEPTIDES is a principal component of the extracellular amyloid in SENILE PLAQUES. (lookformedical.com)
- Functional amyloids play a beneficial role in a variety of physiologic processes (eg, long-term memory formation, gradual release of stored peptide hormones). (medscape.com)
- The extracellular plaques result from elevated levels of an approximately 4.2 kilodalton (kD) protein of about 39-43 amino acids designated the β-amyloid peptide (Aβ) or sometimes βAP, AβP or β/A4 (see, e.g. (justia.com)
- We previously reported the isolation of a novel amyloid-beta-degrading enzyme, acyl peptide hydrolase, a serine protease that degrades amyloid-beta, and is different in structure and activity from other amyloid-beta-degrading enzymes. (biomedcentral.com)
- Acyl peptide hydrolase cleaves the amyloid-beta peptide at amino acids 13, 14 and 19. (biomedcentral.com)
- In addition, by real-time PCR we found elevated acyl peptide hydrolase expression in brain areas rich in amyloid plaques suggesting that this enzyme's levels are responsive to increases in amyloid-beta levels. (biomedcentral.com)
- Lastly, tissue culture experiments using transfected CHO cells expressing APP751 bearing the V717F mutation indicate that acyl peptide hydrolase preferentially degrades dimeric and trimeric forms of amyloid-beta. (biomedcentral.com)
FIBRILS6
- It is the circulating precusor of amyloid A protein, which is found deposited in AA type AMYLOID FIBRILS. (lookformedical.com)
- Accumulations of extracellularly deposited AMYLOID FIBRILS within tissues. (lookformedical.com)
- Islet amyloid polypeptide can fold into AMYLOID FIBRILS that have been found as a major constituent of pancreatic AMYLOID DEPOSITS. (lookformedical.com)
- Amyloid fibrils are protein polymers comprising identical monomer units (homopolymers). (medscape.com)
- [ 6 ] In humans, about 23 different unrelated proteins are known to form amyloid fibrils in vivo. (medscape.com)
- Amyloid-beta exists in different assembly forms in the aging mammalian brain including monomers, oligomers, and aggregates, and in senile plaques, fibrils. (biomedcentral.com)
Fibrillar1
- All types of amyloid consist of one major fibrillar protein that defines the type of amyloid. (medscape.com)
Protein5
- Figure shows protease-resistant prion protein (PrP-res) deposited as amyloid stained with Thioflavin S in the corpus callosum and cerebellum of an anchorless PrP transgenic mouse 194 days after infection with scrapie strain 22L. (nih.gov)
- This model mimics a new fatal familial prion disease in which patients express prion protein with a stop codon near the C-terminus, thus eliminating the GPI anchoring of prion protein. (nih.gov)
- Race B, Williams K, Striebel JF, Chesebro B. Prion-associated cerebral amyloid angiopathy is not exacerbated by human phosphorylated tau aggregates in scrapie-infected mice expressing anchorless prion protein . (nih.gov)
- The amyloidoses are referred to with a capital A (for amyloid) followed by an abbreviation for the fibril protein. (medscape.com)
- Two types of protein aggregates found in the brain are pathological hallmark of AD: intracellular neurofibrillary tangles and extracellular amyloid plaques (for a recent review see Wong et al. (justia.com)
Blood vessels2
- A familial disorder marked by AMYLOID deposits in the walls of small and medium sized blood vessels of CEREBRAL CORTEX and MENINGES . (nih.gov)
- The brains of individuals with AD exhibit characteristic lesions termed senile (or amyloid) plaques, amyloid angiopathy (amyloid deposits in blood vessels) and neurofibrillary tangles. (justia.com)
Autosomal dominant2
- Cerebral microbleeds (CMBs) detected on magnetic resonance imaging are common in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). (bvsalud.org)
- Some familial subtypes demonstrate an autosomal dominant pattern of inheritance. (lookformedical.com)
Proteins1
- The amyloid structure has also been found in a number of functional proteins that are unrelated to disease. (lookformedical.com)
Plaques1
- Deposits of amyloid in the form of AMYLOID PLAQUES are associated with a variety of degenerative diseases. (lookformedical.com)
MENINGES1
- Trastorno familiar marcado por depósitos de AMILOIDE en las paredes de los vasos sanguíneos de tamaño pequeño y medio de la CORTEZA CEREBRAL y de las MENINGES. (bvsalud.org)
CORTEX1
- His Hypothalamus and Cerebral cortex study in the realm of Endocrinology interacts with subjects such as Androgen receptor and Estrogen receptor alpha. (research.com)
ApoE3
- The association between the presence of ApoE and amyloid in amyloidoses suggests a more general role for ApoE in the fibrillogenesis process. (nih.gov)
- However, whether decreasing levels of ApoE would attenuate amyloid pathology in different amyloidoses has not been directly addressed. (nih.gov)
- The discovery of the apolipoprotein E (ApoE) 4 allele as a major risk factor for sporadic and late-onset familial AD has brought attention to the possible role of ApoE in neurodegenerative conditions. (biomedcentral.com)
Encephalopathy1
- Cerebral Amyloid Angiopathy Related Inflammation (CAARI) is a form of reversible encephalopathy presenting with acute or subacute onset of behavioural changes, seizures, and decline in cognition or neurological deficits. (imedpub.com)
Diseases1
- [ 2 ] Many classic eponymic diseases were later found to be related to a diverse array of misfolded polypeptides (amyloid) that contain the common beta-pleated sheet architecture. (medscape.com)
Dementia6
- People with hereditary cerebral amyloid angiopathy often have progressive loss of intellectual function (dementia), stroke, and other neurological problems starting in mid-adulthood. (medlineplus.gov)
- People with the Flemish and Italian types of hereditary cerebral amyloid angiopathy are prone to recurrent strokes and dementia. (medlineplus.gov)
- The first sign of the Icelandic type of hereditary cerebral amyloid angiopathy is typically a stroke followed by dementia. (medlineplus.gov)
- Strokes are rare in people with the Arctic type of hereditary cerebral amyloid angiopathy, in which the first sign is usually memory loss that then progresses to severe dementia. (medlineplus.gov)
- Two types of hereditary cerebral amyloid angiopathy, known as familial British dementia and familial Danish dementia, are characterized by dementia and movement problems. (medlineplus.gov)
- Familial British and Danish dementia are caused by variants in the ITM2B gene. (medlineplus.gov)
Degenerative1
- Cerebral amyloid angiopathy related inflammation is an acute to subacute progressive degenerative neurological disorder which is often mistaken as the clinical and radiological features overlap those of any other neuro proliferative or hemmorhagic conditions [ 1 ]. (imedpub.com)
Clinical1
- Various descriptive classification systems were proposed based on the organ distribution of amyloid deposits and clinical findings. (medscape.com)
Mutation1
- The study incorporates disciplines such as Neurotoxicity, Mutation, Mutant, Knockout mouse and Cerebral amyloid angiopathy in addition to Molecular biology. (research.com)
Type1
- The Dutch type of hereditary cerebral amyloid angiopathy is the most common form. (medlineplus.gov)
Findings1
- Recent findings, however, have implicated altered function of ER-mitochondria contact sites and amyloid beta- and/or tau-induced defects in mitochondrial function and dynamics in the pathogenesis of AD, suggesting that mitochondrial defects may act as key mediators in the pathogenesis of AD as well. (biomedcentral.com)
Liver1
- Nineteen patients, who had undergone liver transplantation for familial amyloidotic polyneuropathy, had answered a quality of life questionnaire including 61 questions on somatic and mental symptoms, social aspects of life, confidence and satisfaction before, one year, and two years after. (sagepub.com)
Secondary1
- Familial, primary (nonfamilial), and secondary forms have been described. (lookformedical.com)
Tissue1
- As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. (lookformedical.com)
Associated with increased1
- The presence of the ApoE4 allele is associated with increased risk of cerebral amyloid angiopathy and age-related cognitive decline during normal aging [ 2 ]. (biomedcentral.com)
Types1
- The various types of hereditary cerebral amyloid angiopathy are named after the regions where they were first diagnosed. (medlineplus.gov)
Common1
- Variants (also called mutations) in the APP gene are the most common cause of hereditary cerebral amyloid angiopathy. (medlineplus.gov)
Native1
- We compared the kinetics of Aβ2M amyloid fibril (fAβ2M) extension with native β2microglobulin (n-β2M) purified from the. (sagepub.com)
Normal1
- A variant of the normal extracellular cysteine protease inhibitor cystatin C (L68Q-cystatin C), is the amyloid precursor in hereditary cystatin C amyloid angiopathy (HCCAA). (sagepub.com)