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Hyperhidrosis results from overactivity of sudomotor nerve bers under a variety of conditions It may occur as an initial excitatory phase of certain peripheral neuropathies (eg, due to arsenic or thallium) and be followed by anhidrosis; it is one aspect of the sympathetic re ex dystrophy pain syndromes (see pages 121 and 189) Disorders of small nerve bers, which enhance adrenergic responses, may also be associated with excessive sweating This is also observed as a localized effect in painful mononeuropathies (causalgia) and diffusely in a number of painful polyneuropathies ( burning foot syndrome) A type of nonthermoregulatory hyperhidrosis may occur in spinal paraplegics (page 1052 and as mentioned above) Loss of sweating in one part of the body may require a compensatory increase in normal parts for example, the excessive facial and upper truncal sweating that occurs in patients with transection of the high thoracic cord Localized hyperhidrosis may be a troublesome complaint in some patients One variety, presumably of congenital origin, affects the palms The social embarrassment of a succulent hand or a dripping paw is often intolerable It is taken to be a sign of nervousness, though many persons with this condition disclaim all other neurotic symptoms Cold, clammy hands are common in individuals with anxiety, and indeed this has been a useful sign in distinguishing an anxiety state from hyperthyroidism, in which the hands are also moist but warm Extirpation of T2 and T3 sympathetic ganglia relieves the more severe cases of palmar sweating; a Horner syndrome does not develop if the T1 ganglion is left intact In other cases, the hyperhidrosis affects mainly the feet Anhidrosis in restricted skin areas is a frequent and useful
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DISORDERS OF THE AUTONOMIC NERVOUS SYSTEM, RESPIRATION, AND SWALLOWING 467
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nding in peripheral nerve disease It is due to the interruption of the postganglionic sympathetic bers, and its boundaries can be mapped by means of the sweat tests described earlier in the chapter The loss of sweating corresponds to the area of sensory loss In contrast, sweating is not affected in spinal root disease because there is much intersegmental mixing of the preganglionic axons once they enter the sympathetic chain A postinfectious anhidrosis syndrome has been described, sometimes accompanied by mild orthostatic hypotension The process is probably a limited form of the pure pandysautonomia described earlier Corticosteroids are said to be bene cial, but we have not found them so Raynaud Syndrome This disorder, characterized by episodic, painful blanching of the ngers and presumably caused by digital artery spasm, was rst described by Raynaud in 1862 The classic appearance is of a triphasic sequence of color change of pallor, cyanosis, and subsequent rubor of the affected ngers or toes but about one-third of such patients have no cyanosis The episodes are brought on by cold or emotional stress and are usually followed by redness on rewarming Numbness, paresthesias, and burning often acompany the color changes It is a disease of early onset, the mean age in idiopathic cases being 14, and it occurs in a number of clinical settings Although most cases are idiopathic, in about half there is an associated connective tissue disease, scleroderma being the main one (Porter et al) In these patients, mostly women with the onset of digital symptoms after age 30, the Raynaud syndrome may antedate the emergence of scleroderma or another rheumatologic autoimmune disorder by many years; such disease usually develops within 2 years In a small group, predominantly men, the syndrome is induced by local trauma, such as prolonged sculling on a cold day, and particularly by vibratory injury incurred by the sustained use of a pneumatic drill or hammer (a syndrome well known in quarry workers) Obstructive arterial disease as might occur with the thoracic outlet syndrome, vasospasm due to drugs (ergot, cytotoxic agents, cocaine), previous cold injury (frostbite), and circulating cryoglobulins is a less common cause Still, in 64 of 219 patients studied by Porter and coworkers, the Raynaud syndrome was classi ed as idiopathic, and most of our cases have been of this type Formerly, the idiopathic form was called Raynaud disease; the type with associated disease was known as Raynaud phenomenon The presence of distorted and proliferative capillaries in the nail bed, visible with an ophthalmoscope, has been used as a bedside aid to reveal cases of connective tissue disease Other processes seen by neurologists, foremost among them carpal tunnel syndrome, also cause cold sensitivity in the ngers Attacks of digital pain and color change from vasculitis, atherosclerotic vascular occlusion, and other causes of occlusive vascular disease only super cially resemble the Raynaud phenomenon; a search for cryoprecipitable proteins in the blood is appropriate Irrespective of the associated disease, one of two mechanisms seems to be operative in the pathogenesis either an arterial constriction or a decrease in the intraluminal pressure The former, in purest form, is observed in young women on exposure to cold and aggravated by emotional stress; a decrease in intraluminal pressure is associated with arterial obstruction Treatment is directed to the associated conditions and prevention of precipitating factors Cervicothoracic sympathectomy has not proved to be an effective measure Lafferty and colleagues have pointed out that, in contrast to
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many areas of the body, the cutaneous nerves of the hands and feet can induce solely vasoconstrictive effects when stimulated, and dilatation is accomplished by chemical mediators Although the sympathetic vasoconstrictive mechanism is intact, sympathectomy is usually ineffective because vasodilatation is under control of these chemical mediators such as histamine The vasodilating properties of the various prostaglandins have been exploited to good effect in some cases A role for nitric oxide in causing digital vasoconstriction has been hypothesized; therefore antagonists of this substance, such as L-arginine, have been tried as therapy with some success, but other mediators of vasoconstriction, such as the peptide endothelins, have also been postulated to play a role Treatment Avoidance of cold exposure is an obvious strategy, as almost all affected patients have discovered by the time they see the physician Drugs that cause vasoconstriction are interdicted (ergots, sympathomimetics, clonidine, and serotonin receptor agonists) Calcium channel blockers are most effective, nifedipine being the most widely used, in doses of 30 to 60 mg per day Other treatments are summarized in the review by Wigley Erythromelalgia, rst described by Weir Mitchell, is a condition of unknown cause and mechanism in which the feet and lower extremities become red and painful on exposure to warm temperatures for prolonged periods (see page 189)
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