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Pathologic examination reveals eosinophilic spheroids of swollen axoplasm in the posterior columns and nuclei of Goll and Burdach and in Clarke s column, substantia nigra, subthalamic nuclei, central nuclei of brainstem, and cerebral cortex There is cerebellar atrophy, affecting the granule cell layer predominantly, and increased iron-containing pigment in the basal ganglia (like that observed in Hallervorden-Spatz disease) The CT scans and CSF are normal, and there are no biochemical or blood cell abnormalities After the age of 2 years, however, the EEG shows characteristic high-amplitude fast rhythms (16 to 22 Hz) Evoked responses may be abnormal Nerve conduction velocities are normal despite EMG evidence of denervation The diagnosis can be reliably established during life by electron microscopic examination of skin and conjunctival nerves, which show the characteristic spheroids within axons.

Line positions are usually management jobs dealing with the elements of school systems that support and make possible the instructional program for example, compliance with school board, state, and federal regulations and mandates; personnel decisions; payroll; supplies; transportation; and food services Staff positions are leadership roles in instruction that have a teaching avor; the duties are neither as precise nor as controlling as those of line personnel The functions of personnel in staff positions are to consult and advise on personnel matters, curriculum, teaching, materials, media, and resources In other words, staff people generally serve as guides and supporters, whereas line personnel have administrative authority and responsibility Some staff personnel do, of course, have line responsibilities For example, when departments become large, as happens when two or more school.

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The Exadata Storage Servers include some flash storage as a hardware component, as will be described in the next chapter on Exadata hardware. However, there is a significant amount of intelligence in the software that utilizes this hardware component, which is covered in this section on Exadata Smart Flash Cache.

There is a later-onset form of the disease in which the course is more protracted and the neurologic manifestations (rigidity and spasticity, cerebellar ataxia, and myoclonus) are more pronounced In these cases the mental regression is slow Vision may be retained, but retinal degeneration has been documented Some of the late-onset cases are indistinguishable from Hallervorden-Spatz disease (page 832) The primary mutation in most cases is unknown In early infantile forms there is a mutation in a lysosomal hydrolase..

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However, the conversion of lactate to glucose requires the expenditure of 6 ATP equivalents by the liver: 2 lactate 2NAD 2 pyruvate 2NADH 2 pyruvate 2ATP 2CO2 2 oxaloacetate 2ADP 2Pi 2 oxaloacetate 2GTP 2PEP 2GDP 2Pi 2CO2 2PEP 2ATP 2NADH glucose 2ADP 2Pi 2NAD Net: 2 lactate 4ATP 2GTP 4ADP 2GDP 6Pi Each mole of glucose that goes through the Cori cycle costs the liver 6 ATP equivalents. If the Cori cycle were perfect and there were an endless supply of ATP, liver could supply glucose equivalents forever just using the same carbon atoms (6 in as lactate, 6 out as glucose). However, some of the lactate (pyruvate) in the nonliver tissues is burned to CO2 by the TCA cycle in the muscle and other tissues. It s this pool that must be replaced by fresh glucose from liver.

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Beginning at the feet of Orion and winding its way to the southern horizon and thence into unknown realms is a string of relatively dim stars. This

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