Progressive Muscular Atrophy
Progressive muscular atrophy (PMA), also called Duchenne–Aran disease and Duchenne–Aran muscular atrophy, is a disorder characterised by the degeneration of lower motor neurons, resulting in generalised, progressive loss of muscle function. PMA is classified among motor neuron diseases (MND) where it is thought to account for around 4% of all MND cases. PMA affects only the lower motor neurons, in contrast to amyotrophic lateral sclerosis (ALS), the most common MND, which affects both the upper and lower motor neurons, or primary lateral sclerosis, another MND, which affects only the upper motor neurons. The distinction is important because PMA is associated with a better prognosis than ALS. Signs and symptoms As a result of lower motor neuron degeneration, the symptoms of PMA include: * muscle weakness * muscle atrophy * fasciculations Some patients have symptoms restricted only to the arms or legs (or in some cases just one of either). These cases are referred to as flai ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Neurology
Neurology (from , "string, nerve" and the suffix wikt:-logia, -logia, "study of") is the branch of specialty (medicine) , medicine dealing with the diagnosis and treatment of all categories of conditions and disease involving the nervous system, which comprises the Human brain, brain, the spinal cord and the peripheral nervous system , peripheral nerves. Neurological practice relies heavily on the field of neuroscience, the scientific study of the nervous system, using various techniques of neurotherapy. IEEE Brain (2019). "Neurotherapy: Treating Disorders by Retraining the Brain". ''The Future Neural Therapeutics White Paper''. Retrieved 23.01.2025 from: https://brain.ieee.org/topics/neurotherapy-treating-disorders-by-retraining-the-brain/#:~:text=Neurotherapy%20trains%20a%20patient's%20brain,wave%20activity%20through%20positive%20reinforcement International Neuromodulation Society, Retrieved 23 January 2025 from: https://www.neuromodulation.com/ Val Danilov I (2023). "The O ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Babinski's Sign
The plantar reflex is a reflex elicited when the sole of the foot is stimulated with a blunt instrument. The reflex can take one of two forms. In healthy adults, the plantar reflex causes a downward response of the hallux (flexion). An upward response (extension) of the hallux is known as the Babinski response or Babinski sign, named after the neurologist Joseph Babinski. The presence of the Babinski sign can identify disease of the spinal cord and brain in adults, and also exists as a primitive reflex in infants. While first described in the medical literature by Babinski in 1896, the reflex has been identified in art at least as early as Botticelli's '' Virgin and Child with an Angel'', painted in the mid-15th century. Methods The lateral side of the sole of the foot is rubbed with a blunt instrument or device, so as not to cause pain, discomfort, or injury to the skin; the instrument is run from the heel along a curve to the toes (metatarsal pads). Many reflex hammers ta ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Progressive Bulbar Palsy
Progressive bulbar palsy (PBP) is a medical condition. It belongs to a group of disorders known as motor neuron diseases. Lapiedra 2002 PBP is a disease that attacks the nerves supplying the bulbar muscles. These disorders are characterized by the degeneration of motor neurons in the cerebral cortex, spinal cord, brain stem, and pyramidal tracts. This specifically involves the glossopharyngeal nerve (IX), vagus nerve (X), and hypoglossal nerve (XII). Hughes 1998 This disorder should not be confused with pseudobulbar palsy or progressive spinal muscular atrophy. The term infantile progressive bulbar palsy is used to describe progressive bulbar palsy in children. The ICD-11 lists progressive bulbar palsy as a variant of amyotrophic lateral sclerosis (ALS). Signs and symptoms Prognosis for PBP patients is poor. Progressive bulbar palsy symptoms can include progressive difficulty with talking and swallowing. Merck 2005 Patients can also exhibit reduced gag reflexes, weak palatal mo ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Primary Lateral Sclerosis
Primary lateral sclerosis (PLS) is a very rare neuromuscular disease characterized by progressive muscle weakness in the voluntary muscles. PLS belongs to a group of disorders known as motor neuron diseases. Motor neuron diseases develop when the nerve cells that control voluntary muscle movement degenerate and die, causing weakness in the muscles they control. PLS only affects upper motor neurons. There is no evidence of the degeneration of spinal motor neurons or muscle wasting ( amyotrophy) that occurs in amyotrophic lateral sclerosis (ALS). Signs and symptoms Onset of PLS usually occurs spontaneously after age 50 and progresses gradually over a number of years, or even decades. The disorder usually begins in the legs, but it may start in the tongue or the hands. Symptoms may include difficulty with balance, weakness and stiffness in the legs, and clumsiness. Other common symptoms are spasticity (involuntary muscle contraction due to the stretching of muscle, which depends ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease (MND) or—in the United States—Lou Gehrig's disease (LGD), is a rare, Terminal illness, terminal neurodegenerative disease, neurodegenerative disorder that results in the progressive loss of both upper and lower motor neurons that normally control Skeletal muscle, voluntary muscle contraction. ALS is the most common form of the motor neuron diseases. ALS often presents in its early stages with gradual muscle Spasticity, stiffness, Fasciculation, twitches, Muscle weakness, weakness, and Muscle atrophy, wasting. Motor neuron loss typically continues until the abilities to eat, speak, move, and, lastly, breathe are all lost. While only 15% of people with ALS also fully develop frontotemporal dementia, an estimated 50% face at least some minor difficulties with cognitive disorder, thinking and behavior. Depending on which of the aforementioned symptoms develops first, ALS is classified as ''limb-onset'' (b ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Scientific Literature
Scientific literature encompasses a vast body of academic papers that spans various disciplines within the natural and social sciences. It primarily consists of academic papers that present original empirical research and theoretical contributions. These papers serve as essential sources of knowledge and are commonly referred to simply as "the literature" within specific research fields. The process of academic publishing involves disseminating research findings to a wider audience. Researchers submit their work to reputable journals or conferences, where it undergoes rigorous evaluation by experts in the field. This evaluation, known as peer review, ensures the quality, validity, and reliability of the research before it becomes part of the scientific literature. Peer-reviewed publications contribute significantly to advancing our understanding of the world and shaping future research endeavors. Original scientific research first published in scientific journals co ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Spinal Muscular Atrophies
Spinal muscular atrophies (SMAs) are a genetically and clinically heterogeneous group of rare debilitating disorders characterised by the degeneration of lower motor neurons (neuronal cells situated in the anterior horn of the spinal cord) and subsequent atrophy (wasting) of various muscle groups in the body. While some SMAs lead to early infant death, other diseases of this group permit normal adult life with only mild weakness. Classification Based on the type of muscles affected, spinal muscular atrophies can be divided into: * ''Proximal spinal muscular atrophies'', i.e., conditions that affect primarily proximal muscles; * ''Distal spinal muscular atrophies'' (which significantly overlap with distal hereditary motor neuronopathies) where they affect primarily distal muscles. When taking into account prevalence, spinal muscular atrophies are traditionally divided into: * '' Autosomal recessive proximal spinal muscular atrophy'', responsible for 90-95% of cases and usu ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Duchenne De Boulogne
Guillaume-Benjamin-Amand Duchenne (de Boulogne) (September 17, 1806, in Boulogne-sur-Mer – September 15, 1875, in Paris) was a French neurologist who revived Luigi Galvani's research and greatly advanced the science of electrophysiology. The era of modern neurology developed from Duchenne's understanding of neural pathways and his diagnostic innovations including deep tissue biopsy, nerve conduction tests ( NCS), and clinical photography. This extraordinary range of activities (mostly in the Salpêtrière) was achieved against the background of a troubled personal life and a generally indifferent medical and scientific establishment. Neurology did not exist in France before Duchenne and although many medical historians regard Jean-Martin Charcot as the father of the discipline, Charcot owed much to Duchenne, often acknowledging him as "''mon maître en neurologie''" (my master in neurology). The American neurologist Joseph Collins (1866–1950) wrote that Duchenne found neuro ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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François-Amilcar Aran
François-Amilcar Aran (12 July 1817, in Bordeaux – 22 February 1861, in Paris) was a French physician. He studied medicine in Bordeaux and received his doctorate in Paris with a thesis on heart palpitations. In Paris he subsequently became a hospital physician and a professor agrégé. He worked as a deputy physician to Léon Louis Rostan at the Hôtel-Dieu, where he held popular clinical lectures. He also distinguished himself in his work performed at the Hôpital Saint-Antoine.François-Amilcar Aran at Who Named It
''Whonamedit?'' is an online English-language dictionary of medical eponyms and the people associated with their identification. Though it is a dictionary, many eponyms an ...
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Babinski Sign
The plantar reflex is a reflex elicited when the sole of the foot is stimulated with a blunt instrument. The reflex can take one of two forms. In healthy adults, the plantar reflex causes a downward response of the hallux (flexion). An upward response ( extension) of the hallux is known as the Babinski response or Babinski sign, named after the neurologist Joseph Babinski. The presence of the Babinski sign can identify disease of the spinal cord and brain in adults, and also exists as a primitive reflex in infants. While first described in the medical literature by Babinski in 1896, the reflex has been identified in art at least as early as Botticelli's '' Virgin and Child with an Angel'', painted in the mid-15th century. Methods The lateral side of the sole of the foot is rubbed with a blunt instrument or device, so as not to cause pain, discomfort, or injury to the skin; the instrument is run from the heel along a curve to the toes (metatarsal pads). Many reflex hamme ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Motor Neuron
A motor neuron (or motoneuron), also known as efferent neuron is a neuron whose cell body is located in the motor cortex, brainstem or the spinal cord, and whose axon (fiber) projects to the spinal cord or outside of the spinal cord to directly or indirectly control effector organs, mainly muscles and glands. There are two types of motor neuron – upper motor neurons and lower motor neurons. Axons from upper motor neurons synapse onto interneurons in the spinal cord and occasionally directly onto lower motor neurons. The axons from the lower motor neurons are efferent nerve fibers that carry signals from the spinal cord to the effectors. Types of lower motor neurons are alpha motor neurons, beta motor neurons, and gamma motor neurons. A single motor neuron may innervate many muscle fibres and a muscle fibre can undergo many action potentials in the time taken for a single muscle twitch. Innervation takes place at a neuromuscular junction and twitches can become superimpo ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |
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Insurance Contract
In insurance, the insurance policy is a contract (generally a standard form contract) between the insurer and the policyholder, which determines the claims which the insurer is legally required to pay. In exchange for an initial payment, known as the premium, the insurer promises to pay for loss caused by perils covered under the policy language. Insurance contracts are designed to meet specific needs and thus have many features not found in many other types of contracts. Since insurance policies are standard forms, they feature boilerplate language which is similar across a wide variety of different types of insurance policies. Available through HeinOnline. The insurance policy is generally an integrated contract, meaning that it includes all forms associated with the agreement between the insured and insurer.Wollner KS. (1999). How to Draft and Interpret Insurance Policies. Casualty Risk Publishing LLC. In some cases, however, supplementary writings such as letters sent after ... [...More Info...]       [...Related Items...]     OR:     [Wikipedia]   [Google]   [Baidu]   |