Integrating Problems
Mrs. A was recently diagnosed with Motor Neuron Disease (Amyotrophic Lateral Sclerosis). ALS is a progressive spinal disorder that causes the myelin sheath of the neurons to disintegrate and harden (sclerosis) which in turn inhibits the synapses of nerve impulses across the body. Mrs. A initially started having difficulties grasping objects and performing simple tasks such as buttoning her shirts. She then decided to visit her physician.
The Biological aspects:
The main functions of the myelin sheaths are to insulate the axon as well as increase the speed at which action potentials are propagated between successive nodes of Ranvier, of the axon. The Nodes of Ranvier contain voltage-gated channels that assist in boosting
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The myelinated neuron is electrically modeled by Figure 2 and clearly shows the increase resistance. This prevents the loss of current and when the signal reached the node of Ranvier the signal is strong enough to trigger a regeneration of the impulse by the influx of Na+ ions through the voltage gates. When the neuron goes through demyelination the resistance around the axon decreases and electrical current is lost to the conducting extracellular fluid. This causes the membrane voltage to decrease because voltage and current are directly proportional according to Ohms Law (V= I/R). The electrical model of an unmeylinated axon is shown in Figure 3 and as the current travels along the axon the current decreases due to leakage which, in turn, decreases the membrane voltage. This results in the signal becoming weaker and weaker until it is too weak to carry the impulse to the effector. A graphical comparison of the conduction (for one internode) found in both the myelinated and unmyelinated neurons is shown is Figure 4 where we can see how signal strength is lost in the unmyelinated neuron.
As we can see the integration of these three subjects greatly increases our understanding the ALS
Following the diagnosis of ALS the main goal of treatment is focused at relieving the symptoms. The most important matter in managing ALS patients is that they continually sustain the highest amount of movement and activity as possible and remain comfortable while doing so. Although a cure for ALS has not yet been developed, effective treatments do exist to assist the patients in their fight with the disease. Physicians urge the patients to carry on with their everyday lives doing all the activities and physically exerting themselves as they would normally do. But, they also recommend the patients to limit their amount of physical exertion
There is also a recently developed SOD1 scan, the gene now thought to be the cause for ALS, especially familial ALS. Only 20%, however, of patients with familial ALS show positive on the SOD1 scan. Progress of ALS Until
Myelin, found only in vertebrate nervous systems, is a fatty substance that surrounds the axons and long dendrites of nerves in the brain and spinal cord tissue (4). By lowering the rate at which the axonal membrane absorbs nerve impulses, myelin acts as an insulator, allowing NS potentials to travel rapidly through the nervous system and maintain communication between the brain and the rest of the body (2). This communication between the brain and the rest of the CNS and peripheral nerves is a central
The nervous system is responsible for registering extrinsic and intrinsic factors and communicating them with the entirety of the body. Dendrites acquire these signals from other neurons and then the signal is transmitted through the cell body and down the axon. Graded potentials create an electrical charge that eventually leads to threshold being met and action potentials being generated. These action potentials send the signals down the axon. Signals will sometimes struggle to hike up stream due to threshold recently being met. This absolute refractory state inhibits signals from traveling up the axon unless myelin is present to increase signaling. It takes longer to conduct a signal down an un-myelinated axon because voltage-gated ion channels
Many people don’t know much about ALS; ALS is better known as “Lou Gehrig’s disease”.” ALS is a progressive disease that affects nerve cells in the brain and spinal cord and the muscles throughout the body”. Musculr Dystrophy Assocation. "Stages of ALS Amyotrophic Lateral Sclerosis." Muscular Dystrophy Association. N.p., 2014. Web. 01 Nov. There is no clear-cut time frame for how long somebody has after they get diagnosed with this disease. Some peoples symptoms gradually grow over time, others occur rapidly, and then plateau. With this disease there is much care needed. Although there isn’t a cure there are treatments to help slow
The brain is a complex organ that controls all of our bodies’ functions. In a normal functioning brain, there are thousands of neurons that lead to the muscles so they can control movement and feeling throughout the body. A rare disease called amyotrophic lateral sclerosis (ALS) affects these neurons in the brain, specifically in the medulla oblongata and the spinal cord. ALS is a neurodegenerative disease that leads to the inability of the brain to signal muscle movements, which cause the person to be unable to walk, speak, eat or breathe, and ultimately leads to paralysis and death.
Myelin is an isolating covering consisting in repeating layer around the axon. It is high in lipids and low in proteins, thus its white color and isolating power. Because many axons are myelinated, electric impulse is able to undergo salutatory movement which accelerates its transmission. Myelination is completed by oligodendrocytes in CNS and neurolemmocytes in the PNS.
Amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease, is a neurodegenerative disease. Originally acknowledged by Jean-Marie Charcot in 1874, this disease kills over 140,000 people worldwide every year. The disease causes progressive paralysis and muscle weakness due to destruction of both upper and lower motor neurons. Due to its insidious nature and the irreversible negative effects on skeletal muscle innervation, ALS has a survival duration time of only 3 to 5 years (Rowland).
Amyotrophic lateral sclerosis is commonly referred to as ALS. This disease is also known to many as Lou Gehrig’s disease. ALS is a disease characterized by the slow death of certain nerve cells in the brain and spine. As described by research done through the Mayo Clinic, nerve cells called motor neurons control the muscles that allow you to move muscles of your body. ALS effects these particular neurons and produce serious neurological effects that can start as muscle weakness that eventually leads to disability and then to death. For some people diagnosed with ALS they may find it difficult to walk, talk, eat food, swallow, and even breathe. These problems can, and will lead to injury, illness, and eventually death. For most people who suffer with ALS, death will occur within three to five years after the first symptoms begin; some people may live for many years beyond that.
The first signs of symptoms came in 2012, for Michael Tsun’s patient. Yet Michael’s patient had no idea that ALS is currently affecting his body, and during the first 6 months since the first sign of symptoms he has experienced slurred speech, stiff muscles and much more. Dr. Tsun could not do anything but watch the disease slowly take over his patient's life. Dr. Tsun felt so heartbroken that, “Tsun retreated into a shell of denial” (Duffy), according to Johns Hopkins Medicine. His family were the ones that “coaxed him out of the shell” (Duffy) and later Dr.Tsun went to a neurologist to see if the prognosis was true, the diagnosis was right on mark. Now Dr.Tsun cannot do anything for his patient but watch him slowly die. You can now see where
Amyotrophic Lateral Sclerosis is an insidiously developing, adult-onset, progressive anterior horn cell degeneration with associated degeneration of descending motor pathways. Despite increasing clinical and research interest, its cause remains obscure. Although many theories as to its cause have been proposed, no intervention has yet been shown to modify biologically determined motor system degeneration.
Amyotrophic Lateral Sclerosis, also known as ALS or sometimes called Lou Gehrig’s Disease, is a progressive neurological disease affecting the nerves that control voluntary muscle movement. It is the most common type of motor neuron disease. The body isn’t able to operate as it normally does due to the muscles weakening overtime. As the disease progresses, it starts to damage nerves of other vital areas of the body. When the body isn’t able to breath the way it should causing respiratory failure and other things start to go wrong, the body has no choice, but to shut down and die. (ALS Association)
Myelin forms around fibers along the axons that are a part of neurons. The Velocity of conducting action potentials can be increased by the myelination process which implies that oligodendrocytes in the central nervous system as well and Schwann cells wrap the axon in myelin. In this process there are layers of glial membrane which an insulator where myelin speeds up action potential conduction. Another way to look at Myelination is that it increases membrane resistance. When the membrane resistance increases this means depolarizing current cannot navigate through the membrane and goes through the low resistance interior. We learned that Node of Ranvier are gaps within the myelin axon which are crucial in producing currents and exchanging
Neurodegenerative diseases is an umbrella term used to describe diseases of the nervous systems that result in the degradation of neurons in the brain and or spinal cord (CITATION). In the case of Amyotrophic Lateral Sclerosis (ALS) the body’s motor neurons die in the motor cortex, brain steam and spinal cord because the brain can no longer send signals from the brain to the muscles. Leaving the individuals on a rapid progressive decline in motor function, which ultimately ends in death as a result of the loss of the muscles (paralysis) that preform breathing and coughing and or other respiratory complications that result in death (Redler & Dokholyan, 2012). ALS was first noted in 1869 by a French neurologist named Jean –Martin Charcot
A distinctive characteristic of ALS is that although the motor neurons die, the brain, cognitive functions and sensory neurons stay intact (Porth & Matfin, 2009). This makes the disease especially devastating because patients become trapped inside their dying body, with a fully alert mind, but are unable to move. It is not known what causes the exact death of the motor neurons in the body, but “five percent to 10% of cases are familial; the others are believed to be sporadic” (Porth & Matfin, 2009, p.