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Technically, a variable is a named storage location (that is, stored in memory) with a type. After the final line of code in the previous example, the value 12 is stored in the named location myVariable. Example 3-1 illustrates the use of variables. To test this program, open Visual Studio .NET and create a console application, just as you did with Hello World in 1. Type in the code shown in bold in Example 3-1.

B Dejerine-Sottas Disease (HMSN Type III)

The disorder may occur on a sporadic, autosomal dominant or, less commonly, autosomal recessive basis Onset in infancy or childhood leads to a progressive motor and sensory polyneuropathy with weakness, ataxia, sensory loss, and depressed or absent tendon reflexes The peripheral nerves may be palpably enlarged and are characterized pathologically by segmental demyelination, Schwann cell hyperplasia, and thin myelin sheaths Electrophysiologically, there is slowing of conduction, and sensory action potentials may be unrecordable

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Patients generally present in childhood or early adult life with this autosomal recessive disorder, which has been related to an unstable mutation of the X25 gene on chromosome 9q13 q211 The gait becomes atactic, the hands become clumsy, and other signs of cerebellar dysfunction develop accompanied by weakness of the legs and extensor plantar responses Involvement of peripheral sensory fibers leads to sensory disturbances in the limbs and depressed tendon reflexes There is bilateral pes cavus Pathologically, there is a marked loss of cells in the posterior root ganglia and degeneration of peripheral sensory fibers In the central nervous system, changes are conspicuous in the posterior and lateral columns of the cord Electrophysiologically, conduction velocity in motor fibers is normal or only mildly reduced, but sensory action potentials are small or absent

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ical cases, but gross slowing of conduction is uncommon A similar distal sensorimotor polyneuropathy is a well-recognized feature of beriberi (thiamine deficiency) In vitamin B12 deficiency, distal sensory polyneuropathy may develop but is usually overshadowed by central nervous system manifestations (eg, myelopathy, optic neuropathy, or intellectual changes)

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D Refsum s Disease (HMSN Type IV)

This autosomal recessive disorder is due to a disturbance in phytanic acid metabolism Clinically, pigmentary retinal degeneration is accompanied by progressive sensorimotor polyneuropathy and cerebellar signs Auditory dysfunction, cardiomyopathy, and cutaneous manifestations may also occur Motor and sensory conduction velocity are reduced, often markedly, and there may be electromyographic evidence of denervation in affected muscles Dietary restriction of phytanic acid and its precursors may be helpful therapeutically Plasmapheresis to reduce stored phytanic acid may help at the initiation of treatment

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A symmetric sensorimotor polyneuropathy that is gradual in onset, progressive in course, and often accompanied by pain and dysesthesias in the limbs may occur in patients (especially men) with multiple myeloma The neuropathy is of the axonal type in classic lytic myeloma, but segmental demyelination (primary or secondary) and axonal loss may occur in sclerotic myeloma and lead to predominantly motor clinical manifestations Both demyelinating and axonal neuropathies are also observed in patients with paraproteinemias without myeloma A small fraction will develop myeloma if serially followed The demyelinating neuropathy in these patients may be due to the monoclonal protein s reacting to a component of the nerve myelin The neuropathy of classic multiple myeloma is poorly responsive to therapy The polyneuropathy of benign monoclonal gammopathy may respond to immunosuppressant drugs and plasmapheresis Polyneuropathy may also occur in association with macroglobulinemia and cryoglobulinemia and sometimes responds to plasmapheresis Entrapment neuropathy, such as carpal tunnel syndrome, is more common than polyneuropathy in patients with (nonhereditary) generalized amyloidosis With polyneuropathy due to amyloidosis, sensory and autonomic symptoms are especially conspicuous, whereas distal wasting and weakness occur later; there is no specific treatment

Peripheral nerve involvement may occur during acute attacks in both variegate porphyria and acute intermittent porphyria Motor symptoms usually occur first, and weakness is often most marked proximally and in the upper limbs rather than the lower Sensory symptoms and signs may be proximal or distal in distribution Autonomic involvement is sometimes pronounced The electrophysiologic findings are in keeping with the results of neuropathologic studies suggesting that the neuropathy is axonal in type Hematin (4 mg/kg intravenously over 15 minutes once or twice daily) may lead to rapid improvement A high-carbohydrate diet and, in severe cases, intravenous glucose or levulose may also be helpful Propranolol (up to 100 mg every 4 hours) may control tachycardia and hypertension in acute attacks

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