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Susac's Syndrome
Susac's syndrome (retinocochleocerebral vasculopathy) is a very rare form of microangiopathy characterized by encephalopathy, branch retinal artery occlusions and hearing loss. The cause is unknown but it is theorized that antibodies are produced against endothelial cells in tiny arteries which leads to damage and the symptoms related to the illness. Despite this being an extremely rare disease, there are 4 registries collecting data on the illness; two are the United States, one in Germany, and one in Portugal. Presentation Susac's syndrome is named for Dr. John Susac (1940–2012), of Winter Haven, Florida, who first described it in 1979. Susac's syndrome is a very rare disease, of unknown cause, and many persons who experience it do not display the bizarre symptoms named here. Their speech can be affected, such as the case of a female of late teens who suffered speech issues and hearing problems, and many experience unrelenting and intense headaches and migraines, some form ...
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Microangiopathy
Microangiopathy (also known as microvascular disease, small vessel disease (SVD) or microvascular dysfunction) is a disease of the microvessels, small blood vessels in the microcirculation. It can be contrasted to coronary heart disease, an angiopathy that affects the larger vessels. Type Cerebral small vessel disease refers to a group of diseases that affect the small arteries, arterioles, venules, and capillaries of the brain. Age-related and hypertension-related small vessel diseases and cerebral amyloid angiopathy are the most common forms. Coronary small vessel disease is a type of coronary heart disease that affects the arterioles and capillaries of the heart. Coronary small vessel disease is also known as microvascular angina, microvascular dysfunction, non-obstructive coronary disease, or previously ''cardiac syndrome X''. Pathophysiology One cause of microangiopathy is long-term diabetes mellitus. In this case, high blood glucose levels cause the endothelial cells linin ...
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Tinnitus
Tinnitus is the perception of sound when no corresponding external sound is present. Nearly everyone experiences a faint "normal tinnitus" in a completely quiet room; but it is of concern only if it is bothersome, interferes with normal hearing, or is associated with other problems. While often described as a ringing, it may also sound like a clicking, buzzing, hissing or roaring. It may be soft or loud, low- or high- pitched, and may seem to come from one or both ears or from the head itself. In some people, it may interfere with concentration, and in some cases is associated with anxiety and depression. Tinnitus is usually associated with a degree of hearing loss and decreased comprehension of speech in noisy environments. It is common, affecting about 10–15% of people. Most, however, tolerate it well, and it is a significant problem in only 1–2% of all people. It can trigger a fight-or-flight response, as the brain may perceive it as dangerous and important. The word ' ...
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Corticosteroid
Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are involved in a wide range of physiological processes, including stress response, immune response, and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte levels, and behavior. Some common naturally occurring steroid hormones are cortisol (), corticosterone (), cortisone () and aldosterone (). (Note that cortisone and aldosterone are isomers.) The main corticosteroids produced by the adrenal cortex are cortisol and aldosterone. Classes * Glucocorticoids such as cortisol affect carbohydrate, fat, and protein metabolism, and have anti-inflammatory, immunosuppressive, anti-proliferative, and vasoconstrictive effects. Anti-inflammatory effects are mediated by blocking the action of inflammatory medi ...
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Immunosuppressive
Immunosuppression is a reduction of the activation or efficacy of the immune system. Some portions of the immune system itself have immunosuppressive effects on other parts of the immune system, and immunosuppression may occur as an adverse reaction to treatment of other conditions. In general, deliberately induced immunosuppression is performed to prevent the body from rejecting an organ transplant. Additionally, it is used for treating graft-versus-host disease after a bone marrow transplant, or for the treatment of auto-immune diseases such as systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome, or Crohn's disease. This is typically done using medications, but may involve surgery (splenectomy), plasmapheresis, or radiation. A person who is undergoing immunosuppression, or whose immune system is weak for some other reasons (such as chemotherapy or HIV), is said to be ''immunocompromised''. Deliberately induced Administration of immunosuppressive medica ...
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Juvenile Dermatomyositis
Juvenile dermatomyositis (JDM) is an idiopathic inflammatory myopathy (IMM) of presumed autoimmune dysfunction resulting in muscle weakness among other complications. It manifests itself in children; it is the pediatric counterpart of dermatomyositis. In JDM, the body's immune system attacks blood vessels throughout the body, causing inflammation called vasculitis. In the United States, the incidence rate of JDMS is approximately 2-3 cases per million children per year. The UK incidence is believed to be between 2-3 per million children per year, with some difference between ethnic groups. The sex ratio (Female : Male) is approximately 2:1. Other Idiopathic inflammatory myopathies include; juvenile polymyositis (PM), which is rare and not as common in children as in adults. Signs and symptoms The vasculitis caused by JDMS manifests itself predominantly in two ways: One is a distinctive rash. The rash often affects the face, eyelids, and hands, and sometimes the skin above joi ...
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Gray Matter
Grey matter is a major component of the central nervous system, consisting of neuronal cell bodies, neuropil (dendrites and unmyelinated axons), glial cells (astrocytes and oligodendrocytes), synapses, and capillaries. Grey matter is distinguished from white matter in that it contains numerous cell bodies and relatively few myelinated axons, while white matter contains relatively few cell bodies and is composed chiefly of long-range myelinated axons. The colour difference arises mainly from the whiteness of myelin. In living tissue, grey matter actually has a very light grey colour with yellowish or pinkish hues, which come from capillary blood vessels and neuronal cell bodies. Structure Grey matter refers to unmyelinated neurons and other cells of the central nervous system. It is present in the brain, brainstem and cerebellum, and present throughout the spinal cord. Grey matter is distributed at the surface of the cerebral hemispheres (cerebral cortex) and of the cerebellum ...
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Acute Disseminated Encephalomyelitis
Acute disseminated encephalomyelitis (ADEM), or acute Demyelinating disease, demyelinating encephalomyelitis, is a rare autoimmune disease marked by a sudden, widespread attack of inflammation in the brain and spinal cord. As well as causing the brain and spinal cord to become inflamed, ADEM also attacks the nerves of the central nervous system and damages their myelin insulation, which, as a result, destroys the white matter. It is often triggered by a virus (biology), viral infection or vaccinations. ADEM's symptoms resemble the symptoms of multiple sclerosis (MS), so the disease itself is sorted into the classification of the multiple sclerosis borderline diseases. However, ADEM has several features that distinguish it from MS. Unlike MS, ADEM occurs usually in children and is marked with rapid fever, although adolescents and adults can get the disease too. ADEM consists of a single flare-up whereas MS is marked with several flare-ups (or relapses), over a long period of time. ...
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Multiple Sclerosis
Multiple (cerebral) sclerosis (MS), also known as encephalomyelitis disseminata or disseminated sclerosis, is the most common demyelinating disease, in which the insulating covers of nerve cells in the brain and spinal cord are damaged. This damage disrupts the ability of parts of the nervous system to transmit signals, resulting in a range of signs and symptoms, including physical, mental, and sometimes psychiatric problems. Specific symptoms can include double vision, blindness in one eye, muscle weakness, and trouble with sensation or coordination. MS takes several forms, with new symptoms either occurring in isolated attacks (relapsing forms) or building up over time (progressive forms). In the relapsing forms of MS, between attacks, symptoms may disappear completely, although some permanent neurological problems often remain, especially as the disease advances. While the cause is unclear, the underlying mechanism is thought to be either destruction by the immune system ...
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Meninges
In anatomy, the meninges (, ''singular:'' meninx ( or ), ) are the three membranes that envelop the brain and spinal cord. In mammals, the meninges are the dura mater, the arachnoid mater, and the pia mater. Cerebrospinal fluid is located in the subarachnoid space between the arachnoid mater and the pia mater. The primary function of the meninges is to protect the central nervous system. Structure Dura mater The dura mater ( la, tough mother) (also rarely called ''meninx fibrosa'' or ''pachymeninx'') is a thick, durable membrane, closest to the Human skull, skull and vertebrae. The dura mater, the outermost part, is a loosely arranged, fibroelastic layer of cells, characterized by multiple interdigitating cell processes, no extracellular collagen, and significant extracellular spaces. The middle region is a mostly fibrous portion. It consists of two layers: the endosteal layer, which lies closest to the Calvaria (skull), skull, and the inner meningeal layer, which lies closer ...
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Corpus Callosum
The corpus callosum (Latin for "tough body"), also callosal commissure, is a wide, thick nerve tract, consisting of a flat bundle of commissural fibers, beneath the cerebral cortex in the brain. The corpus callosum is only found in placental mammals. It spans part of the longitudinal fissure, connecting the left and right cerebral hemispheres, enabling communication between them. It is the largest white matter structure in the human brain, about in length and consisting of 200–300 million axonal projections. A number of separate nerve tracts, classed as subregions of the corpus callosum, connect different parts of the hemispheres. The main ones are known as the genu, the rostrum, the trunk or body, and the splenium. Structure The corpus callosum forms the floor of the longitudinal fissure that separates the two cerebral hemispheres. Part of the corpus callosum forms the roof of the lateral ventricles. The corpus callosum has four main parts – individual nerve tracts ...
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Fluorescein Angiography
Fluorescein angiography (FA), fluorescent angiography (FAG), or fundus fluorescein angiography (FFA) is a technique for examining the circulation of the retina and choroid (parts of the fundus) using a fluorescent dye and a specialized camera. Sodium fluorescein is added into the systemic circulation, the retina is illuminated with blue light at a wavelength of 490 nanometers, and an angiogram is obtained by photographing the fluorescent green light that is emitted by the dye. The fluorescein is administered intravenously in intravenous fluorescein angiography (IVFA) and orally in oral fluorescein angiography (OFA). The test is a dye tracing method. The fluorescein dye also reappears in the patient urine, causing the urine to appear darker, and sometimes orange. It can also cause discolouration of the saliva. Fluorescein angiography is one of several health care applications of this dye, all of which have a risk of severe adverse effects. See fluorescein safety in health care a ...
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Pathognomonic
Pathognomonic (rare synonym ''pathognomic'') is a term, often used in medicine, that means "characteristic for a particular disease". A pathognomonic sign is a particular sign whose presence means that a particular disease is present beyond any doubt. Labelling a sign or symptom "pathognomonic" represents a marked intensification of a "diagnostic" sign or symptom. The word is an adjective of Greek origin derived from πάθος ''pathos'' "disease" and γνώμων ''gnomon'' "indicator" (from γιγνώσκω ''gignosko'' "I know, I recognize"). Practical use While some findings may be classic, typical or highly suggestive in a certain condition, they may not occur ''uniquely'' in this condition and therefore may not directly imply a specific diagnosis. A pathognomonic sign or symptom has very high positive predictive value but does not need to have high sensitivity: for example it can sometimes be absent in a certain disease, since the term only implies that, when it is prese ...
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