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Vulpian-Bernhardt Syndrome
Brachial amyotrophic diplegia, also called Vulpian-Bernhardt Syndrome (VBS), flail arm syndrome, or man-in-barrel syndrome, is a rare Motor neuron diseases, motor neuron disease, often considered to be a phenotype or regional variant of ALS, amyotrophic lateral sclerosis. In the first twelve to eighteen months, only a progressive weakness of one or both arms is observed. Within the spectrum of ALS, this subtype has a relatively longer survival time. Because of its distinct symptoms, it was suggested that VBS is a separate entity, different from ALS. History The disease was described first in 1886 by the French neurologist Alfred Vulpian and the German neuropathologist Martin Bernhardt as a progressive weakness of the upper limbs but Medulla oblongata, bulbar muscles unaffected. It is a rare and atypical disease that can only be diagnosed after a significant time has elapsed. A 2015 study found significant differences between ALS and VBS, most importantly the absence of signs of u ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Motor Neuron Diseases
An engine or motor is a machine designed to convert one or more forms of energy into mechanical energy. Available energy sources include potential energy (e.g. energy of the Earth's gravitational field as exploited in hydroelectric power generation), heat energy (e.g. geothermal), chemical energy, electric potential and nuclear energy (from nuclear fission or nuclear fusion). Many of these processes generate heat as an intermediate energy form; thus heat engines have special importance. Some natural processes, such as atmospheric convection cells convert environmental heat into motion (e.g. in the form of rising air currents). Mechanical energy is of particular importance in transportation, but also plays a role in many industrial processes such as cutting, grinding, crushing, and mixing. Mechanical heat engines convert heat into work via various thermodynamic processes. The internal combustion engine is perhaps the most common example of a mechanical heat engine in which heat ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Alfred Vulpian
Edmé Félix Alfred Vulpian (5 January 1826 – 18 May 1887) was a French physician and neurologist. He was the co-discoverer of Vulpian-Bernhardt spinal muscular atrophy and the Vulpian-Heidenhain-Sherrington phenomenon. Vulpian was born in Paris, France, in 1826. Among other noted discoveries and experiments, Vulpian discovered adrenaline in the adrenal medulla The adrenal medulla () is the inner part of the adrenal gland. It is located at the center of the gland, being surrounded by the adrenal cortex. It is the innermost part of the adrenal gland, consisting of chromaffin cells that secrete catecho .... He was the first to use the term " fibrillation" to describe a chaotic irregular rhythm of the heart.Cardioversion Past, Present and Future. Cakulev I, Efimov I and Waldo A. Circulation 2009; 120:1623–1632 Vulpian's monument in Paris A large marble statue has been erected to Vulpian, just at the end of Rue Antoine Dubois, a short distance from the Faculty of Medicin ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Martin Bernhardt
Martin Bernhardt (10 April 1844 – 17 March 1915) was a German neuropathologist. Bernhardt was a native of Potsdam. His family was Jewish.Andreas Killen, ''Berlin Electropolis: Shock, Nerves, and German Modernity'', University of California Press (2006), p. 64 In 1867 he received his medical doctorate at the University of Berlin, where he was a student of Rudolf Virchow (1821-1902) and Ludwig Traube (1818-1878). Subsequently, he became an assistant to Ernst Viktor von Leyden (1832-1910) at the university clinic at Königsberg, and afterwards worked at the Berlin-Charité under Karl Friedrich Otto Westphal (1833-1890). After military service in the Franco-Prussian War, he returned to Berlin as a specialist in neuropathology, and in 1882 attained the title of "professor extraordinarius". Bernhardt published several treatises on neurological diseases and electrotherapy, and in 1885 became editor-in-chief of the ''Centralblatt für die Medizinischen Wissenschaften''. With Russian ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Medulla Oblongata
The medulla oblongata or simply medulla is a long stem-like structure which makes up the lower part of the brainstem. It is anterior and partially inferior to the cerebellum. It is a cone-shaped neuronal mass responsible for autonomic (involuntary) functions, ranging from vomiting to sneezing. The medulla contains the cardiovascular center, the respiratory center, vomiting and vasomotor centers, responsible for the autonomic functions of breathing, heart rate and blood pressure as well as the sleep–wake cycle. "Medulla" is from Latin, ‘pith or marrow’. And "oblongata" is from Latin, ‘lengthened or longish or elongated'. During embryonic development, the medulla oblongata develops from the myelencephalon. The myelencephalon is a secondary brain vesicle which forms during the maturation of the rhombencephalon, also referred to as the hindbrain. The bulb is an archaic term for the medulla oblongata. In modern clinical usage, the word bulbar (as in bulbar palsy) is r ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Upper Motor Neuron
Upper motor neurons (UMNs) is a term introduced by William Gowers in 1886. They are found in the cerebral cortex and brainstem and carry information down to activate interneurons and lower motor neurons, which in turn directly signal muscles to contract or relax. UMNs represent the major origin point for voluntary somatic movement. Upper motor neurons represent the largest pyramidal cells in the motor regions of the cerebral cortex. The major cell type of the UMNs is the '' Betz cells'' residing in layer V of the primary motor cortex, located on the precentral gyrus in the posterior frontal lobe. The cell bodies of Betz cell neurons are the largest in the brain, approaching nearly 0.1 mm in diameter. The axons of the upper motor neurons project out of the precentral gyrus travelling through to the brainstem, where they will decussate (intersect) within the lower medulla oblongata to form the lateral corticospinal tract on each side of the spinal cord. The fibers that ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Paraneoplastic Syndrome
A paraneoplastic syndrome is a syndrome (a set of signs and symptoms) that is the consequence of a tumor in the body (usually a cancerous one). It is specifically due to the production of chemical signaling molecules (such as hormones or cytokines) by tumor cells or by an immune response against the tumor. Unlike a mass effect, it is not due to the local presence of cancer cells. Paraneoplastic syndromes are typical among middle-aged to older people, and they most commonly occur with cancers of the lung, breast, ovaries or lymphatic system (a lymphoma). Sometimes, the symptoms of paraneoplastic syndromes show before the diagnosis of a malignancy, which has been hypothesized to relate to the disease pathogenesis. In this paradigm, tumor cells express tissue-restricted antigens (e.g., neuronal proteins), triggering an anti-tumor immune response which may be partially or, rarely, completely effective in suppressing tumor growth and symptoms. Patients then come to clinical attention ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
SOD1
Superoxide dismutase u-Zn'' also known as superoxide dismutase 1 or hSod1 is an enzyme that in humans is encoded by the ''SOD1'' gene, located on chromosome 21. SOD1 is one of three human superoxide dismutases. It is implicated in apoptosis, familial amyotrophic lateral sclerosis and Parkinson's disease. Structure SOD1 is a 32 kDa homodimer which forms a beta barrel (β-barrel) and contains an intramolecular disulfide bond and a binuclear Cu/Zn site in each subunit. This Cu/Zn site holds the copper and a zinc ion and is responsible for catalyzing the disproportionation of superoxide to hydrogen peroxide and dioxygen. The maturation process of this protein is complex and not fully understood, involving the selective binding of copper and zinc ions, formation of the intra-subunit disulfide bond between Cys-57 and Cys-146, and dimerization of the two subunits. The copper chaperone for Sod1 (CCS) facilitates copper insertion and disulfide oxidation. Although SOD1 is synthesized in ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Neurodegenerative Disorders
A neurodegenerative disease is caused by the progressive loss of neurons, in the process known as neurodegeneration. Neuronal damage may also ultimately result in their death. Neurodegenerative diseases include amyotrophic lateral sclerosis, multiple sclerosis, Parkinson's disease, Alzheimer's disease, Huntington's disease, multiple system atrophy, tauopathies, and prion diseases. Neurodegeneration can be found in the brain at many different levels of neuronal circuitry, ranging from molecular to systemic. Because there is no known way to reverse the progressive degeneration of neurons, these diseases are considered to be incurable; however research has shown that the two major contributing factors to neurodegeneration are oxidative stress and inflammation. Biomedical research has revealed many similarities between these diseases at the subcellular level, including atypical protein assemblies (like proteinopathy) and induced cell death. These similarities suggest that therapeut ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
