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Charcot–Marie–Tooth disease
Charcot–Marie–Tooth disease
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Charcot-Marie-Tooth Disease, Adult
Charcot-Marie-Tooth disease (CMT) is a group of nervous system diseases that cause gradual loss of strength and feeling in the arms and legs. The condition usually develops in late childhood or early adolescence. Over time, the arm and leg muscles may shrink (atrophy).
CMT affects the nerves outside the brain and spinal cord (peripheral nerves). There are several types of CMT, depending on the type of gene mutation you have. Symptoms of CMT can range from mild to severe.
CAUSES
This condition is usually passed down through families (inherited). CMT is caused by defects (mutations) in the genes that affect the peripheral nerves. Over time, these mutations cause the nerves to break down. The nerves lose
warm) in the left upper and lower extremities; decreased strength and movement of the right upper and lower extremities and of the left abdominal muscles; lack of triceps and biceps reflexes in the right upper extremity; atypical response of patellar, Achilles (hyper) reflexes in the right lower extremity; abnormal cremasteric reflex in the right groin; fracture in cervical vertebrae #7; and significant swelling in the C7-T12 region of the spinal canal (Signs and symptoms, n.d.). The objective complaint of a severe headache could also be consistent with a spinal cord injury (Headache, nausea, and vomiting,
Also evident are molluscoid pseudotumors (fleshy lesions associated with scars) frequently found over pressure points (e.g. elbows) and subcutaneous spheroids, which are commonly mobile and palpable on the forearms and shins. Complications of joint hypermobility include sprains, dislocation are common in the shoulder, patella and temporomandibular joints Muscle hypotonia and slower gross motor development also can occur It is inherited in an autosomal dominant manner (Clarke, D., Skrocki-Czerpak, K., Neumann-Potash, L.). In the Hypermobile type of EDS, the joints of the body experience Hypermobility, which is the dominant clinical manifestation. General joint hypermobility affects large (elbows, knees) and small (fingers and toes) joints. Skin is hyperextensible, smooth/velvety, and bruising occurs easily as well.
T/D comes in a dry form only. There is a “small bites” version available for smaller dogs and a regular kibble size for medium to large do...
Peripheral and central mechanisms involving nerve lesions and their input are substantial when perceiving phantom pain. Due to the impairment of peripheral nerves in the process of amputation, regenerative sprouting of damaged axons occurs and the activity rate of inflamed C-fibres and demyelinated A-fibres spontaneously increases (Flor, 2002). As a consequence of this nerve injury, a neuroma, which is a mass of pruned and tangled axons, may form in the residual limb producing abnormal (ectopic) activity (Katz, 1992). Flor, Nikolajsen and Jenson (2006) proposed that ectopic discharge from a neuroma in the stump illustrates abnormal afferent input to the spinal cord, which is a possible mechanism for unpro...
Flaccid dysarthria results from damage to the lower motor neurons (LMN) or the peripheral nervous system (Hageman, 1997). The characteristics of flaccid dysarthria generally reflect damage to cranial nerves with motor speech functions (e.g., cranial nerves IX, X, XI and XII) (Seikel, King & Drumright, 2010). Lower motor neurons connect the central nervous system to the muscle fibers; from the brainstem to the cranial nerves with motor function, or from the anterior horns of grey matter to the spinal nerves (Murdoch, 1998). If there are lesions to spinal nerves and the cranial nerves with motor speech functions, it is indicative of a lower motor neuron lesion and flaccid dysarthria. Damage to lower motor neurons that supply the speech muscles is also known as bulbar palsy (Pena-Brooks & Hedge, 2007). Potential etiologies of flaccid dysarthria include spinal cord injury, cerebrovascular accidents, tumors or traumatic brain injury (Pena-Brooks & Hedge, 2007). Possible congenital etiologies of flaccid dysarthria include Moebius syndrome and cerebral palsy. Flaccid dysarthria can also arise from infections such as polio, herpes zoster, and secondary infections to AIDS (Pena-Brooks & Hedge, 2007). Additionally, demyelinating diseases such as Guilian-Barre syndrome and myotonic muscular dystrophy can also lead to flaccid dysarthria (Pena-Brookes & Hedge, 2007). The lower motor neuron lesion results in loss of voluntary muscle control, and an inability to maintain muscle tone. Fasciculations, or twitching movements, may occur if the cell body is involved in the lesion (Seikel et. al., 2010). The primary speech characteristics of flaccid dysarthria include imprecise consonant production, hypernasal resonance, breathiness, and harsh voice (...
The effects include paralysis of a limb or one side of the body and disturbances of speech and vision. The nature and extent of damage depends on the size and location of the affected blood vessels. The main causes are cerebral infarction (approx. 85%) and spontaneous intracranial haemorrhage (15%) (Waugh & Grant, 2010).
... damaged neurons. (Mayo clinic, 2014). This is called neuroplasticity, the ability for the nerves to compensate for damage caused by some outside force. Because of neuroplasticity physical training works to cure some of the paralysis left by the virus and allows us to walk again after the legs or another appendage is deformed or damaged.
The most common types of topographical types are diplegia, hemiplegia, double hemiplegia, and quadriplegia. The basal ganglia are part of the extrapyramidal system and work in conjunction with the motor cortex in providing movement and serve as the relay center. Damage to this area results in Athetoid Cerebral Palsy, the second most common form of cerebral palsy. Involuntary, purposeless movements, particularly in the arms, hands, and facial muscles, characterize athetosis. In addition, the individual can become “stuck” in abnormal positions or postures and require specific positioning to maintain normal tone and movement.
The walking dead does in fact exist. However, it is not the flesh eating zombie that many think about when hearing the term ‘walking dead.’ Instead, it is a rare and serious mental disorder also known as Cotard’s Syndrome. “In 1880, Jules Cotard (1840-1889) described the syndrome that bears his name as a constellation of false nihilistic beliefs, often in the form of self-negation.” (Ramirez-Bermudez, Aguilar-Venegas, Crail-Melendez, Espinola-Nadurille, Nente & Mendez, 2010) Throughout time there has been many controversies regarding what causes this disease. Cotard’s Syndrome was finally divided into three groups in 1995 as psychotic depression, Cotard’s Syndrome Type I, and Cotard’s Syndrome Type II.
Multiple sclerosis is an immune mediated disease, although many researchers argue it is an autoimmune disease. MS causes the body to create an abnormal immune response to the Central Nervous System (CNS). The CNS is the body’s processing center. It consists of the brain, spinal cord and peripheral nerves. The axon of these nerve cells are coated with a myelin sheath, a fatty substance that surrounds the nerve endings. This sheath protects and insulates the axons allowing electrical impulses to pass freely from one nerve cell to the next. MS causes the body’s immune system to produce T-cells that pass from the bloodstream and into the central nervous system. The T-cells directly attack the nerve cells as if they were a foreign substance. They destroy the myelin coating around nerve fibers. When any part of the myelin is damaged scar tissue forms around the nerve ending. This demyelination and scaring causes lesions on the nerves. The lesions prevent impulses from traveling throughout the body and hinders the body’s response to afferent and efferent signals being sent to and from the brain.
Multiple sclerosis is a disease that affects the central nervous system, attacking the brain and the spinal cord. MS attacks myelin, the fatty material that acts as a protective coating to the body's nerves. (1) The inflammation of the nerve tissues covering the nerves can affect any part of the nervous system and varies from person to person. (7) Normal nerve function decreases with the onset of MS because MS causes scars to form on the covering of the nerve. Multiple Sclerosis acquires this term because it literally means scars. (1,7) The covering of the nerve with myelin is very important so that the nerve can transmit signals rapidly and efficiently. Demylelination enables the nerve to carry impulses properly by either blocking or slowing transmission and this is why the various symptoms of MS occur. (1)
When a person begins to suffer from Guillain- Barre Syndrome their myelin sheath of their nervous system is being attacked and destroyed by the immune system (NINDS, 2011). The myelin sheath begins to lose its ability to transmit signals rapidly and affectively. Since signals are not getting transmitted to the brain fast enough, a person begins to notice fewer sensory responses from the rest of the body (NINDS, 2011). A person wouldn’t be able to tell right away or at all if an item they are touching is hot, cold, or causing pain. There also wouldn’t be good signal transmission from the brain to the rest of the body (NINDS, 2011). There would be signs of the muscles being unable to respond to the weakened or distraught signals they were receiving. Since the myelin sheath is responsible for transmitting the signals from a long distance, the upper and lower extremities would be the first to show signs of muscle dysfunction.
Charcot Marie Tooth disease encompasses a group of inherited disorders that affect motor and sensory peripheral nerves. It is a type of neuropathy characterized by damage to myelin sheaths and nerve axon structure that results in impaired ability of the peripheral nervous system to send signals or relay sensory information. CMT typically presents with distal predominance of limb-muscle wasting, weakness, and sensory loss , . Symptoms start in the feet, which commonly have high arches, hammer toes, intrinsic muscle weakness, and wasting. The disease then begins to affect the legs and the lower thighs, which results in distal atrophy of the lower limbs. The hands become affected, followed by the forearms. Sensory loss also
Charcot Marie Tooth Disease is a neurological disorder named after the three physicians who first described it in 1886, Jean Martin Charcot and Pierre Marie from France and Howard Henry Tooth from the United Kingdom. Charcot Marie Tooth Disease is a group of hereditary disorders that damage the nerves in the arms and legs. When the parts of the nerves, the axons and the myelin, become damaged the messages that run along the nerves move more slowly or have a weak signal. Charcot Marie Tooth Disease is a disease of the peripheral nerves that controls the muscles. It is found in men and women, and slowly increases causing loss of normal use of the lower legs, feet, arms, and hands. Charcot Marie Tooth Disease is also known as hereditary sensory