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Susac 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 four 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 f ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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 macroangiopathies such as atherosclerosis, where large and medium-sized arteries (e.g., aorta, carotid and coronary arteries) are primarily affected. Small vessel diseases (SVDs) affect primarily organs that receive significant portions of cardiac output such as the brain, the kidney, and the retina. Thus, SVDs are a major etiologic cause in debilitating conditions such as renal failure, blindness, lacunar infarcts, and dementia. Types Microangiopathies are involved in a variety of different diseases including: Pathophysiology The main target of small vessel diseases is the endothelium, which plays a key role in vascular homeostasis. The pathogenesis of SVDs in various organs is characterized by endothelial dysfunction, capillary rarefaction, microthrombi and mi ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Inner Ear
The inner ear (internal ear, auris interna) is the innermost part of the vertebrate ear. In vertebrates, the inner ear is mainly responsible for sound detection and balance. In mammals, it consists of the bony labyrinth, a hollow cavity in the temporal bone of the skull with a system of passages comprising two main functional parts: * The cochlea, dedicated to hearing; converting sound pressure patterns from the outer ear into electrochemical impulses which are passed on to the brain via the auditory nerve. * The vestibular system, dedicated to balance (ability), balance. The inner ear is found in all vertebrates, with substantial variations in form and function. The inner ear is innervated by the eighth cranial nerve in all vertebrates. Structure The labyrinth can be divided by layer or by region. Bony and membranous labyrinths The bony labyrinth, or osseous labyrinth, is the network of passages with bony walls lined with periosteum. The three major parts of the bony labyrin ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Gray Matter
Grey matter, or gray matter in American English, 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 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Acute Disseminated Encephalomyelitis
Acute disseminated encephalomyelitis (ADEM), or acute 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. The cause is often a trigger such as from viral infection or, in extraordinarily rare cases, 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 per ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Multiple Sclerosis
Multiple sclerosis (MS) is an autoimmune disease resulting in damage to myelinthe insulating covers of nerve cellsin the brain and spinal cord. As a demyelinating disease, MS disrupts the nervous system's ability to Action potential, transmit signals, resulting in a range of signs and symptoms, including physical, cognitive disability, mental, and sometimes psychiatric problems. Symptoms include double vision, vision loss, eye pain, muscle weakness, and loss of Sensation (psychology), 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 relapsing forms of MS, symptoms may disappear completely between attacks, although some permanent neurological problems often remain, especially as the disease advances. In progressive forms of MS, bodily function slowly deteriorates once symptoms manifest and will steadily worsen if left untreated. While its cause is unclear, ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Meninges
In anatomy, the meninges (; meninx ; ) 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 (), 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 skull, and the inner meningeal layer, which lies closer to the brain. It contains larger blood vessels that split into the capillaries in the pia mater. It is composed ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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 nerv ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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-green 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 h ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pathognomonic
Pathognomonic (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. The absence of a pathognomonic sign does not rule out the disease. 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 and high specificity but does not need to have high sensitivity: for example it can som ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Arteriole
An arteriole is a small-diameter blood vessel in the microcirculation that extends and branches out from an artery and leads to capillary, capillaries. Arterioles have vascular smooth muscle, muscular walls (usually only one to two layers of smooth muscle cells) and are the primary site of vascular resistance. The greatest change in blood pressure and velocity of blood flow occurs at the transition of arterioles to capillaries. This function is extremely important because it prevents the thin, one-layer capillaries from exploding upon pressure. The arterioles achieve this decrease in pressure, as they are the site with the highest resistance (a large contributor to total peripheral resistance) which translates to a large decrease in the pressure. Structure In a healthy vascular system, the endothelium lines all blood-contacting surfaces, including arteries, arterioles, veins, venules, capillaries, and heart chambers. This healthy condition is promoted by the ample production of n ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Branch Retinal Artery Occlusion
Branch retinal artery occlusion (BRAO) is a rare retinal vascular disorder in which one of the branches of the central retinal artery is obstructed. Although often grouped together under one term, the condition consists of two distinct subtypes: permanent BRAO and transient BRAO. Signs and symptoms Sudden painless partial vision loss Causes Diagnosis Treatment No proven treatment exists for branch retinal artery occlusion. In the rare patient who has branch retinal artery obstruction accompanied by a systemic disorder, systemic anti-coagulation may prevent further events. Epidemiology See also * Central retinal artery occlusion Central retinal artery occlusion (CRAO) is a disease of the eye where the flow of blood through the central retinal artery is blocked (occluded). There are several different causes of this occlusion; the most common is carotid artery atheroscle ... * Central retinal vein occlusion * Branch retinal vein occlusion References E ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
