A syndrome of hyperhidrosis, hypothermia, and bradycardia possibly due to central monoaminergic dysfunction.
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A 62-year-old woman presented with episodic sweating and shivering with reduced core temperature. Brain MRI demonstrated a basal forebrain malformation. Physiologic testing included EEG, SPECT, heat challenge, and autonomic testing. Glycopyrrolate aborted spells and raised core temperature. Hypothalamic dysregulation is likely the primary pathophysiology in the setting of other forebrain anomalies. These findings expand the structural abnormalities and treatment options within the temperature dysregulating conditions of Shapiro's syndrome and "diencephalic epilepsy."
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High upper thoracic sympathectomy using microsurgical techniques aided by electrical stimulation of the sympathetic chain is described. Use of the microscope facilitates identification and dissection of the sympathetic chain and minimizes the risk of pleural damage. Electrical stimulation of the sympathetic chain establishes the correct functional level for surgical excision. At the correct level, the threshold for piloerection, sudomotor response, and decrease in blood flow of the ipsilateral hand to electrical stimulation was minimal, and a six-fold increase in stimulus current causing current spread was required to dilate the ipsilateral pupil. After identification of the proper level, surgical excision can be done without risking postoperative Horner's syndrome. Excision of the appropriate ganglia and intervening sympathetic chain with placement of surgical clips on the proximal and distal nerve stumps provides tissue for histological analysis, decreases the opportunity for regeneration, and facilitates localization on postoperative x-rays.
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Axillary hyperhidrotics is reviewed from the standpoint of anatomical factors, physiological mechanisms and the history of methods of control. Anhydrous aluminum chloride and anhydrous zirconium tetrachloride are shown to be superior topical agents for partial control of axillary sweating when applied as a powder or in anhydrous nonreactive vehicles. Complete anhidrosis as demonstrated by sustained garment armpit dryness could be achieved in hyperhidrotics within 48 hours by the following trinary antiperspirant system: (1) a saturated solution of aluminum chloride hexahydrate or zirconyl chloride in absolute ethanol or isopropyl alcohol, (2) application to the dry axilla at times of sleep or other prolonged non-sweating period, (3) water vapor occlusion of area for 6 to 8 hours by means of Saran wrap. The hypothesis is presented that metallic antiperspirants act by reflux entrance into the terminal intraepidermal eccrine duct, slowly combining with the intraductal keratin, to produce a fibrillar contraction (super contraction) of keratin and hence functional closure, not histologically evident. This altered keratin is shed weeks later, with the consequent return of ductal patency and sweating.
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The identification of the T-2 ganglion through a narrow operative viewfield is the greatest challenge in performing endoscopic transaxillary T-2 sympathectomy, especially for a surgeon who is unfamiliar with the technique. The authors describe a simple anatomical method for identifying the T-2 ganglion during the operation, based on a study of 17 adult cadavers. First, a similar clinical procedure was performed along the anterior or middle axillary line via the second to fourth intercostal spaces to measure the aiming angles and intrathoracic depth needed. Second, the regional anatomical structures and their relationship to bilateral T-2 ganglia were delineated. It was discovered that the superior intercostal artery, a branch of the subclavian artery, was an accessible landmark. This small vessel existed in 87.5% of the cadavers studied. It consistently runs lateral to the parallel sympathetic chain at an average distance of 10 mm. Most important is that it can be easily distinguished where it runs across the inner part of the second rib. The authors emphasize that the superior intercostal artery should be a very beneficial landmark for surgical orientation.
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