Treatment Adrenal replacement therapy prolongs life and occasionally effects a partial neurologic remission. A diet enriched with monounsaturated fatty acids and devoid of long-chain fatty acids has been said to slow the progress of the disease when administered before the age of 6. Bone marrow transplantation, to date performed in over 50 children, has been the only treatment shown to stabilize the disease and reverse some of the MRI changes. The reviews by van Geel and colleagues, and the ones by Moser, the leading worker in the eld, summarizing diagnosis and treatment, are recommended. Juvenile and Adult Metachromatic Leukodystrophy (MLD) This form of leukodystrophy has already been described in relation to the inherited metabolic disorders of late infancy and early childhood in an earlier section. It is mentioned here again to emphasize the point that the disease may have its onset at almost any age. Juvenile forms may begin between 4 and 12 years and adult forms between the midteens and the seventh decade. The mutations giving rise to these various forms are described in the earlier section on MLD. Some of the sporadic cases reported in the adult have probably been examples of cerebral multiple sclerosis, but we have seen, as have others, cases of MLD appearing as late as middle adult life. In almost all cases, the clinical picture, like those Turpin and Baumann, was one of slowly evolving intellectual decline or behavioral abnormality, followed by spastic weakness, hyperre exia, Babinski signs, and stiff, short-stepped gait, with or without a polyneuropathy. Without manifest neurologic signs, misdiagnosis of psychiatric disease is common. As the disease progresses over a period of 3 to 5 years there may be a loss of vision and speech, then of hearing, and nally a state of virtual decerebration. In some of these cases it is impossible to distinguish the white matter disease from that of Pelizaeus-Merzbacher and of Cockayne, described in the preceding section. Orthochromatic Leukodystrophy This refers to a heterogeneous group of disorders, also called nonmetachromatic leukodystrophy, in which no enzyme defect or special staining characteristic of degenerated white matter has been identi ed. Furthermore, most of them have been sporadic, obscuring the nosology of the process. One type is associated with cerebellar ataxia and dementia; other adult cases have been described in which epilepsy was associated with a frontal lobe type of dementia. A surprising feature, such as in the cases of Letournel and colleagues, has been normal MRI, even with the more sensitive FLAIR sequences. Cerebrotendinous Xanthomatosis This rare disease is probably transmitted by an autosomal recessive gene. It usually begins in late childhood, with cataracts and xanthomas of tendon sheaths and lungs. As it progresses, dif culty in learning, impairment of retentive memory, and de cits in attention and visuospatial perception (the earliest neurologic manifestations) give way to dementia, ataxic or ataxic-spastic gait, dysarthria and dysphagia, and polyneuropathy. In the late stages (after 5 to 15 years), the patient becomes bedfast and helpless; death occurs at 20 to 30 years of age. In other cases the clinical course is much more benign. Neuropathologic examination shows masses of crystalline cholesterol in the brainstem and cerebellum and sometimes in the spinal cord, with symmetrical destruction of myelin in the involved areas. The lesions are visible by CT scanning and MRI. The basic defect is in the synthesis of primary bile acids, leading to an increased hepatic production of cholesterol and choles-
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HAVING A SICK CHILD IS AN UPSETTING AND WORRISOME experience for all parents. Because very young children can t talk, parents often have to resort to guessing what s wrong. Is he teething Does she have an earache Is he cold or wet or simply lonely In this story, Sophia, long before she could say the word hot, was able to tell her mother exactly what she was feeling. The signs Sophia and hundreds of thousands of other babies around the world are using are based on both extensive child development research and oldfashioned common sense about how babies communicate. All babies learn to wave a hand for bye-bye, shake the head back and forth for no, and nod the head
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likely to occur or prior to a major grain or livestock report in commodities trading. The key to success in straddles is not getting caught in a volatility trap in which implied volatility drops rapidly after an announcement or event. This ensures that your probabilities match your pro tability. If your probability analysis shows that the underlying has 25 percent or less chance of reaching outside the V shaped range of the straddle model in the intermediate time frame, then it is possible that you may be buying at too high a volatility or that the underlying may not show the range necessary to build value in a straddle. Long straddles and other delta-neutral strategies can be complicated in risk control because they are limited risk. Just because the risk is limited on the spread does not necessarily mean low or no risk. The underlying asset can move in either direction and reverse back and forth through the straddle thus eroding time value and possibly reducing volatility. The dif culty with a long straddle is your exit
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This industry magazine provides case studies, how-to information, and advice from experts on a variety of topics.
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Keep in mind that there are laws independent of man s consent, ruling over reality, over nature, over man, too, whether he be willing to recognize them or not to which we must bow, unless we think we can rule ourselves independently of the rest of nature. Egoism, in other words, must be defeated in self. The egoist is never happy.
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Bones. The human body contains 206 bones that, collectively, compose the skeleton. Muscles, as we have seen, are attached to bones by tendons and assist us in moving from one position to another.
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