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Peripheral Neuropathy with Secondary Orthostatic Hypotension
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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-
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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
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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
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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)
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