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Before long, the triad of atrophic weakness of the hands and forearms, slight spasticity of the arms and legs, and generalized hyperre exia all in the absence of sensory change leaves little doubt as to the diagnosis Muscle strength and bulk diminish in parallel or there is a relative preservation of power early in the illness Despite the amyotrophy, the tendon re exes are notable for their liveliness Babinski and Hoffmann signs are variably present Surprisingly, they may not appear even as the illness progresses Abductors, adductors, and extensors of ngers and thumb tend to become weak before the long exors, on which the handgrip depends, and the dorsal interosseous spaces become hollowed, giving rise to the cadaveric or skeletal hand The muscles of the upper arm and shoulder girdles are typically involved later.

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' movement subroutines forward: high high high high enable_left forward_left enable_right forward_right

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2. Terminate the SCSI chain. Perform this step only if the new device is at the end of the chain. If the device has an on-board terminator, select the proper jumper setting to enable it. If not, place a terminator plug in the unused port on the device. 3. Set the SCSI ID. If this is a Plug-and-Play device, the ID will be assigned automatically. Simply restart the computer and skip the remaining steps. If the ID must be configured manually and electronically, restart the computer and run the device s Setup program (this will also load the driver, so you can skip the next step). To set the ID manually using a jumper, consult the manufacturer s documentation and make the proper jumper setting. 4. Load the device s driver. Restart the computer and insert the floppy disk or CD that came with the device. Run the Setup or Install program.

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When an arm is the rst limb affected, all this occurs while the thigh and leg muscles seem relatively normal, and there may come a time in some cases when the patient walks about with useless, dangling arms Later the atrophic weakness spreads to the neck, tongue, pharyngeal, and laryngeal muscles, and eventually those in the trunk and lower extremities yield to the onslaught of the disease The affected parts may ache and feel cold, but true paresthesias, except from poor positioning and pressure on nerves, do not occur or are minor Sphincteric control is well maintained even after both legs have become weak and spastic, but many patients acquire urinary and sometimes fecal urgency in the advanced stages of the disease The abdominal re exes may be elicitable even when the plantar re exes are extensor Extreme spasticity is rarely seen.

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When there is no substrate present ([S] 0), there is no velocity so far, so good. As the substrate concentration [S] is increased, the reaction goes faster as the enzyme finds it easier and easier to locate the substrate in solution. At low substrate concentrations ([S] Km), doubling the concentration of substrate causes the velocity to double.

Coarse fasciculations are usually evident in the weakened muscles but may not be noticed by the patient until the physician calls attention to them Fasciculations are almost never the sole presenting feature of ALS a clinical truism with which one can reassure physicians and medical students who fear, on the basis of persistent focal muscle twitching, that they are developing the disease The course of this illness, irrespective of its particular mode of onset and pattern of evolution, is progressive There may be periods lasting weeks or months during which the patient observes no advance in symptoms but clinical changes can nonetheless be detected Half the patients succumb within 3 years of onset and 90 percent within 6 years (Mulder et al) The clinical variants of motor neuron disease that occur with regularity and have distinguishing clinical features are described below.

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That takes care of not only one, but two of the elements we wanted to turn into 0. But there s a problem starting to take shape. We want the third entry in the third row to end up as a nonzero element. We won t be able to do that without making both the first and the second elements in that row nonzero as well. We can go further and add the first two rows in the above matrix together, replacing the first row with the sum. Then we get

Other Patterns of Evolution In addition to the special con gurations discussed further on, there are many patterns of neuromuscular involvement other than the one just described A leg may be affected before the hands A foot drop with weakness and wasting of the pretibial muscles may be incorrectly attributed to peroneal nerve compression until weakness of the gastrocnemius and other muscles betrays a more widespread involvement of lumbosacral.

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