rdlc data matrix

rdlc data matrix

rdlc data matrix

rdlc data matrix

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that the signs of postganglionic denervation were uncommon in patients classi ed as having the central type In the postganglionic type of autonomic failure, plasma levels of NE are subnormal while the patient is recumbent because of failure of the damaged nerve terminals to synthesize or release catecholamines When the patient stands, the NE levels do not rise, as they do in a normal person Also, in this type, there is denervation hypersensitivity to infused NE In the central preganglionic (Shy-Drager) type, the resting NE levels in the plasma are normal, but again, on standing, there is no rise, and the response to exogenously administered NE is normal In both types, the plasma levels of dopamine -hydroxylase, the enzyme that converts dopamine to NE, are subnormal (Ziegler et al) The use of these neurochemical tests in clinical practice is dif cult and the data in the literature are inconsistent Low s monograph should be consulted for procedural details Pathologic studies have disclosed the central type of autonomic failure to be somewhat heterogeneous Oppenheimer, who collected all the reported central cases with complete autopsies, found that they fell into two groups: (1) that which was designated by Adams as striatonigral degeneration or, later, Shy-Drager syndrome, where autonomic failure was associated with a parkinsonian syndrome and often with the presence of cytoplasmic inclusions in sympathetic neurons, and (2) another with involvement of the striatum, cerebellum, pons, and medulla but without inclusions, formerly designated olivopontocerebellar degeneration (there are now reported to be glial and neuronal cytoplasmic inclusions in all these cases) Both conditions are now loosely referred to as multiple system atrophy, as discussed in Chap 39 In both groups, the autonomic failure is attributable to degeneration of lateral horn cells of the thoracic cord There is also a degeneration of nerve cells in the vagal nuclei as well as nuclei of the tractus solitarius, locus ceruleus, and sacral autonomic nuclei, accounting for laryngeal abductor weakness (laryngeal paralysis and stridor are features in some cases), incontinence, and impotence NE and dopamine are depleted in the hypothalamus (Spokes et al) The sympathetic ganglia have been normal, an exception being the case of Rajput and Rozdilsky, in which most of the ganglion cells had degenerated (In Parkinson disease, where fainting is sometimes a problem, Lewy bodies are found in degenerating sympathetic ganglion cells) Treatment of orthostatic hypotension consists of having the patient sleep with the head of the bed elevated, administering mineralocorticoid medications such as udrocortisone acetate (Florinef ) 01 mg twice daily, or midodrine starting at 25 mg q 4 h, slowly raising the dose to 5 mg q 4 h, and using elastic stockings that compress the veins of the legs and lower abdomen.

Impairment of autonomic function, of which orthostatic hypotension is the most serious feature, may occur as part of the more common acute or chronic peripheral neuropathies (eg, diabetic, alcoholic-nutritional, amyloid, Guillain-Barre, heavy metal toxic, and porphyric) Disease of the peripheral nervous system may affect the circulation in two ways: the nerves from baroreceptors may be affected, interrupting normal homeostatic re exes on the afferent side, or postganglionic efferent sympathetic bers may be involved in the spinal nerves The severity of the autonomic failure need not parallel the degree of motor weakness An additional feature of the acute dysautonomias is a tendency to develop hypona-

tremia, presumably as a result of dysfunction of afferent bers from aortic arch volume receptors, which elicits a release of vasopressin These same stretch baroreceptors are implicated in the intermittent hypertension that sometimes complicates these acute neuropathies Of particular importance is the autonomic disorder that accompanies diabetic neuropathy It presents as impotence, constipation, or diarrhea (especially at night), hypotonia of the bladder, gastroparesis, and orthostatic hypotension, in some combination There are invariably signs of a sensory polyneuropathy, consisting of a distal loss of vibratory and thermal-pain sensation and reduced or lost ankle re exes; but again, the severity of affection of the two systems of nerve bers may not be parallel The pupils are often small and the amplitude of constriction to light is reduced (ArgyllRobertson pupils); this has been attributed to involvement of the ciliary ganglia The pathologic basis of the other features has been dif cult to assess because of the frequency of artifact in the sympathetic ganglia in autopsy material Duchen and coworkers attributed the autonomic disorder to vacuolization of sympathetic ganglionic neurons, cell necrosis and in ammation, loss of myelinated bers in the vagi and white rami communicantes, and loss of lateral horn cells in the spinal cord They believe the latter changes to be secondary Another polyneuropathy with unusually prominent dysautonomia is that due to amyloidosis Extensive loss of pain and thermal sensation is usually present; other forms of sensation may also be reduced to a lesser degree Motor function is much less altered Sympathetic function is more affected than parasympathetic Iridoplegia (pupillary paralysis) and disturbances of other smooth muscle and glandular functions are variable Diabetic and amyloid polyneuropathy are further described in Chap 46 Both the primary and secondary types of orthostatic hypotension are also discussed in connection with syncope in Chap 18

activities The frequency of a characteristic is displayed in a histogram (for example, the sales revenue for a product group) The frequencies are divided into classes, where each class corresponds to a column in the histogram In a histogram, categories (classes) are

dominant pattern of inheritance (Robinson and colleagues) Stabbing pains in the feet were increased by bending, crouching, and kneeling There was no sweat response to intradermal injection of 1% ACh and no autonomic bers were found in punch biopsies of the skin Fabry disease was excluded

Orthostatic hypotension is prevalent in the elderly, so much so that norms of blood pressure and pulse changes have been dif cult to establish Caird and coworkers reported that among individuals who were more than 65 years of age and living at home, 24 percent had a fall of systolic blood pressure on standing of 20 mmHg; 9 percent had a fall of 30 mmHg; and 5 percent, a fall of 40 mmHg An increased frequency of thermoregulatory impairment has been documented as well The elderly are also more liable to develop hypothermia and, when exposed to high ambient temperature, to hyperthermia Loss of sweating of the lower parts of the body and increased sweating of the head and arms probably re ect a senile neuropathy or neuronopathy The number of sensory ganglion cells decreases with age (Castro) Impotence and incontinence also increase with age, but these, of course, may be due to a number of processes besides autonomic failure It is of interest that the idiopathic type of small ber neuropathy that occurs predominantly in elderly women ( burning hands and feet syndrome) has no associated autonomic features (see Chap 46)