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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 ...
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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 ...
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Emotional Lability
In medicine and psychology, emotional lability is a Medical sign, sign or symptom typified by exaggerated changes in mood or affect (psychology), affect in quick succession. Sometimes the emotions expressed outwardly are very different from how the person feels on the inside. These strong emotions can be a disproportionate response to something that happened, but other times there might be no trigger at all. The person experiencing emotional lability usually feels like they do not have control over their emotions. For example, someone might cry uncontrollably in response to any strong emotion even if they do not feel sad or unhappy. Emotional lability is seen or reported in various conditions including borderline personality disorder, histrionic personality disorder, post-traumatic stress disorder, hypomanic or manic episodes of bipolar disorder, and neurological disorders or brain injury (where it is termed pseudobulbar affect), such as after a stroke. It has sometimes been found ...
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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 ...
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Assistive Cane
An assistive cane is a walking stick used as a crutch or mobility aid. A cane can help redistribute weight from a lower leg that is weak or painful, improve stability by increasing the base of support, and provide tactile information about the ground to improve balance. In the US, ten percent of adults older than 65 years use a cane, and 4.6 percent use walkers. In contrast to crutches, canes are generally lighter, but, because they transfer the load through the user's unsupported wrist, are unable to offload equal loads from the legs. Another type of crutch is the walker, a frame held in front of the user and which the user leans on during movement. Walkers are more stable due to their increased area of ground contact, but are larger and less wieldy and, like canes, pass the full load through the user's wrists in most cases. Parts of medical canes The basic cane has four parts. These parts vary depending on the design of the cane and the needs of the user. * Handle. The ...
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Wheelchairs
A wheelchair is a mobilized form of chair using two or more wheels, a footrest, and an armrest usually cushioned. It is used when walking is difficult or impossible to do due to illnesses, injury, disabilities, or age-related health conditions. Wheelchairs provide mobility, postural support, and freedom to those who cannot walk or have difficulty walking, enabling them to move around, participate in everyday activities, and live life on their own terms. Wheelchairs come in a wide variety of formats to meet the specific needs of their users. They may include specialized seating adaptions, and individualized controls, and may be specific to particular activities, as with sports wheelchairs and beach wheelchairs. The most widely recognized distinction is between motorized wheelchairs, where propulsion is provided by batteries and electric motors, and manual wheelchairs, where the propulsive force is provided either by the wheelchair user or occupant pushing the wheelchair by hand ...
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Cerebrospinal Fluid
Cerebrospinal fluid (CSF) is a clear, colorless Extracellular fluid#Transcellular fluid, transcellular body fluid found within the meninges, meningeal tissue that surrounds the vertebrate brain and spinal cord, and in the ventricular system, ventricles of the brain. CSF is mostly produced by specialized Ependyma, ependymal cells in the choroid plexuses of the ventricles of the brain, and absorbed in the arachnoid granulations. It is also produced by ependymal cells in the lining of the ventricles. In humans, there is about 125 mL of CSF at any one time, and about 500 mL is generated every day. CSF acts as a shock absorber, cushion or buffer, providing basic mechanical and immune system, immunological protection to the brain inside the Human skull, skull. CSF also serves a vital function in the cerebral autoregulation of cerebral blood flow. CSF occupies the subarachnoid space (between the arachnoid mater and the pia mater) and the ventricular system around and inside t ...
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Phenytoin
Phenytoin (PHT), sold under the brand name Dilantin among others, is an anticonvulsant, anti-seizure medication. It is useful for the prevention of tonic-clonic seizures (also known as grand mal seizures) and focal seizures, but not absence seizures. The intravenous form, fosphenytoin, is used for status epilepticus that does not improve with benzodiazepines. It may also be used for certain heart arrhythmias or neuropathic pain. It can be taken intravenously or by mouth. The intravenous form generally begins working within 30 minutes and is effective for roughly 24 hours. Blood levels can be measured to determine the proper dose. Common side effects include nausea, stomach pain, loss of appetite, poor coordination, hypertrichosis, increased hair growth, and Gingival hyperplasia, enlargement of the gums. Potentially serious side effects include sleepiness, self harm, liver problems, bone marrow suppression, hypotension, low blood pressure, toxic epidermal necrolysis, and atrop ...
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Quinine
Quinine is a medication used to treat malaria and babesiosis. This includes the treatment of malaria due to ''Plasmodium falciparum'' that is resistant to chloroquine when artesunate is not available. While sometimes used for nocturnal leg cramps, quinine is not recommended for this purpose due to the risk of serious side effects. It can be taken by mouth or intravenously. Malaria resistance to quinine occurs in certain areas of the world. Quinine is also used as an ingredient in tonic water and other beverages to impart a bitter taste. Common side effects include headache, tinnitus, ringing in the ears, vision issues, and sweating. More severe side effects include deafness, thrombocytopenia, low blood platelets, and an irregular heartbeat. Use can make one more prone to sunburn. While it is unclear if use during pregnancy carries potential for fetal harm, treating malaria during pregnancy with quinine when appropriate is still recommended. Quinine is an alkaloid, a natural ...
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Tizanidine
Tizanidine, sold under the brand name Zanaflex among others, is an alpha-2 (α2) adrenergic receptor agonist, similar to clonidine, that is used to treat muscle spasticity due to spinal cord injury, multiple sclerosis, and spastic cerebral palsy. Effectiveness appears similar to baclofen or diazepam. It is taken by mouth. Common side effects of tizanidine include dry mouth, sleepiness, weakness, and dizziness. Serious side effects may include low blood pressure, liver problems, psychosis, and QT prolongation. It is unclear if use in pregnancy and breastfeeding is safe. It is an α2-adrenergic agonist, but how it works is not entirely clear. Tizanidine was approved for medical use in the United States in 1996. It is available as a generic medication. In 2022, it was the 94th most commonly prescribed medication in the United States, with more than 6million prescriptions. Medical uses Tizanidine has been found to be as effective as other antispasmodic drugs and i ...
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Baclofen
Baclofen, sold under the brand name Lioresal among others, is a medication used to treat muscle spasticity, such as from a spinal cord injury or multiple sclerosis. It may also be used for hiccups and muscle spasms near the end of life, and off-label to treat alcohol use disorder or opioid withdrawal symptoms. It is taken orally or by intrathecal pump (delivered into the spinal canal via an implantable pump device). It is sometimes used transdermally (applied topically to the skin) in combination with gabapentin and clonidine prepared at a compounding pharmacy. It is believed to work by decreasing levels of certain neurotransmitters. Baclofen should be avoided in the setting of chronic kidney disease and end stage renal disease as even small doses can cause excessive toxicity. Common side effects include sleepiness, weakness, and dizziness. Serious side effects, such as seizures and rhabdomyolysis, may occur if use of baclofen is stopped abruptly. Use during pregn ...
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Mayo Clinic
Mayo Clinic () is a Nonprofit organization, private American Academic health science centre, academic Medical centers in the United States, medical center focused on integrated health care, healthcare, Mayo Clinic College of Medicine and Science, education, and research. It maintains three major campuses in Rochester, Minnesota; Jacksonville, Florida; and Phoenix, Arizona, Phoenix/Scottsdale, Arizona. Mayo Clinic employs over 7,300 physicians and scientists, along with another 66,000 administrative and allied health staff. The practice specializes in treating difficult cases through Health care#Tertiary care, tertiary care and Medical tourism#United States, destination medicine. It is home to the top-15 ranked Mayo Clinic Alix School of Medicine in addition to many of the highest regarded residency education programs in the United States. It spends over $660 million a year on research and has more than 3,000 full-time research personnel. William Worrall Mayo settled his family i ...
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ALS2
Alsin is a protein that in humans is encoded by the ''ALS2'' gene. ''ALS2'' orthologs have been identified in all mammals for which complete genome data are available. See also * Juvenile primary lateral sclerosis * 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 i ... References Further reading * * * * * * * * * * * * * * * * * External links GeneReviews/NCBI/NIH/UW entry on ALS2-Related Disorders OMIM entries on ALS2-Related Disorders Genetics Home Reference- US National Library of Medicine
* {{gene-2-stub ...
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