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In the face of such clinical information, one should obtain appropriate biochemical analyses of blood, urine, and cerebrospinal uid (CSF); magnetic resonance imaging (MRI) of the brain; and, in certain instances, chromosome-genetic studies. In addition to the investigation of symptomatic individuals, the array of available genetic and biochemical tests has made practical the mass screening of newborns for inborn metabolic defects. Innovative tests have also led to the discovery of a number of previously unknown diseases and have clari ed the basic biochemistry of old ones. As a consequence, the neurologist s role is changing. No longer must he wait until a disease of the nervous system has declared itself by conventional symptoms and signs, by which time the underlying lesion may have become irreversible. Now it is possible to nd patients who, though asymptomatic, are at risk and to introduce dietary and other measures that may prevent injury to the nervous system. This is especially important to families who have already had an affected infant. To assume this new responsibility intelligently requires some knowledge of genetics, biochemical screening methods, and public health measures. The many clinical syndromes by which these inborn errors of metabolism declare themselves vary in accordance with the nature of the biochemical defect and the stage of maturation of the nervous system at which these metabolic alterations become apparent. In phenylketonuria, for example, there is a speci c effect on the cerebral white matter, mainly during the period of active myelination; once the stages of myelinogenesis are complete, the biochemical abnormality becomes relatively harmless. Even more important from the neurologist s point of view is the level of function that has been achieved by the developing nervous system when the disease strikes. A derangement of function in a neonate or infant,

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( 19.8)

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Pain, it has been said, is one of nature s earliest signs of morbidity, and it stands pre-eminent among all the sensory experiences by which humans judge the existence of disease within themselves Indeed, pain is the most common symptom of disease Relatively few diseases do not have a painful phase, and in many, pain is a characteristic without which diagnosis must be in doubt The painful experiences of the sick pose manifold problems in virtually every eld of medicine; physicians must therefore learn something of these problems and their management if they are to practice effectively They must be prepared to recognize disease in patients who have felt only the rst rumblings of discomfort, before other symptoms and signs have appeared.

82. What is the interval notation for x ! 4 a 4; 1 c 1; 4 83. A salesman earns $12,000 annual base salary plus 8% commission on sales. If he wants an annual salary of at least $45,000, what should his annual sales be (a) At least $712,500 (c) At least $412,500 9 84. p 2 (b) At most $712,500 (d) At most $412,500

Fig. 19-12

Even more problematic are patients who seek treatment for pain that appears to have little or no structural basis; further inquiry may disclose that fear of serious disease, worry, or depression has aggravated some relatively minor ache or that the complaint of pain has become the means of seeking drugs or monetary compensation They must also cope with the dif cult pain cases in which no amount of investigation brings to light either medical or psychiatric illness Finally, the physician must be prepared to manage patients who demand relief from intractable pain caused by established and incurable disease To deal intelligently with such pain problems requires familiarity with the anatomy of sensory pathways and the sensory supply of body segments as well as insight into the psychologic factors that in uence the perception of and reaction to pain.

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