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Postural orthostatic tachycardia syndrome (POTS) is characterized by excessive tachycardia during orthostasis. To test the hypothesis that patients with POTS have decreased sympathetic neural responses to baroreflex stimuli, we measured heart rate (HR) and muscle sympathetic nerve activity (MSNA) responses to three baroreflex stimuli including vasoactive drug boluses (modified Oxford technique), Valsalva maneuver, and head-up tilt (HUT) in POTS patients and healthy control subjects. The MSNA response to the Valsalva maneuver was significantly greater in the POTS group (controls, 26 +/- 7 vs. POTS, 48 +/- 6% of baseline MSNA/mmHg; P = 0.03). POTS patients also had an exaggerated MSNA response to 30 degrees HUT (controls, 123 +/- 24 vs. POTS, 208 +/- 30% of baseline MSNA; P = 0.03) and tended to have an exaggerated response to 45 degrees HUT (controls, 137 +/- 27 vs. POTS, 248 +/- 58% of baseline MSNA; P = 0.10). Sympathetic baroreflex sensitivity calculated during administration of the vasoactive drug boluses also tended to be greater in the POTS patients; however, this did not reach statistical significance (P = 0.15). Baseline MSNA values during supine rest were not different between the groups (controls, 23 +/- 4 vs. POTS, 16 +/- 5 bursts/100 heartbeats; P = 0.30); however, resting HR was significantly higher in the POTS group (controls, 58 +/- 3 vs. POTS, 82 +/- 4 beats/min; P = 0.0001). Our results suggest that POTS patients have exaggerated MSNA responses to baroreflex challenges compared with healthy control subjects, although resting supine MSNA values did not differ between the groups.

This paper presents some currently available neurophysiological tools that are helpful in the clinical setting to evaluate and document neuropathic disturbances that may be associated with pain. The specific tests described in this discussion are quantitative sensory tests (QSTs), autonomic tests (ATs), microneurography (MCNG), and laser evoked potentials (LEPs). Quantitative sensory testing of the nociceptive system includes the thermal stimulation (TST) and current perception threshold (CPT) tests. The ATs applicable to some patients with pain are sudomotor and vasomotor tests. The quantitative sudomotor axon reflex test (QSART), resting sweat output (RSO), and sympathetic skin response (SSR) are the tests for sudomotor involvement. The vasomotor system is tested by measuring skin temperature (surface thermistor or thermography) at rest and, in some cases, after provocative maneuvers. In addition, MCNG (intraneural recording of single nerve fibers or fascicles of nerves) allows examiners to look directly at muscle and skin sympathetic efferent output in normal subjects without pain or with experimental pain and in patients with neuropathic pain. This technique also provides a means of studying the physiology of primary afferent fibers in persons with neurogenic pain. Recent development of LEPs that incorporate the use of painful infrared laser-induced stimuli allow selective study of the nociceptive system, both the central and peripheral portions.

We describe a patient with bilateral orbital myositis, multiple cranial neuropathies, a sensory polyneuropathy, serum and cerebrospinal fluid paraproteins, and high-grade non-Hodgkin's lymphoma. Neurologic symptoms began more than 1 year before diagnosis of the lymphoma. Results of extraocular muscle biopsy showed extensive destruction of myofibers and granulomatous features, with no evidence of direct tumor involvement. The cranial neuropathies and orbital myositis improved with immunosuppressive therapy, while the patient's tumor progressed. We believe the orbital myositis and the multiple neurologic abnormalities were paraneoplastic effects of the lymphoma. To our knowledge, this is the first case of orbital myositis identified as a paraneoplastic syndrome.

Causalgia, reflex sympathetic dystrophy, and sympathetically maintained pain (SMP) are a complex group of disorders, with symptoms of spontaneous/stimulus-induced pain and vasomotor, sudomotor or skeletomotor dysfunction of the involved area. Sympatholysis has been recommended for diagnosis/classification and treatment of these patients. Lack of adequate placebo control makes the physiologic response to this intervention unclear. Sensitization of wide dynamic range (WDR) neurons in the central nociceptive pathway has been proposed as a key element in pathophysiologic mechanisms of these disorders. Low threshold mechanoreceptors and nociceptors have been implicated as the primary afferents transmitting signals to or maintaining sensitization of WDR neurons in SMP. Vasomotor disturbances may result from antidromic vasodilatation, vasoparalytic dilatation, normal somatosympathetic reflexes, and denervation supersensitivity. There is conflicting information regarding the use of phentolamine and clonidine in these pain syndromes. Treatment of these patients remains a challenge given the many potential underlying mechanisms.

1. Effects of rate of rise of temperature stimuli applied to skin on (i) unitary receptor threshold and frequency response often single C nociceptors, and (ii) on magnitude and reaction times of evoked pain were studied in fifteen healthy human volunteers. 2. Temperature ramps of 32 to 45 or 47 degrees C were applied at three consistent rates of rise to receptive fields of C nociceptors in dorsum of foot (n = 9) or hand (n = 1). For rates of rise of 0.3, 2.0 and 6.0 degrees C/s, mean receptor threshold for heat was remarkably uniform: 41.5 +/- 0.57, 41.5 +/- 0.61 and 41.9 +/- 0.71 degrees C respectively. 3. The mean discharge rate of the ten cutaneous C nociceptors increased with rate of rise of temperature stimuli: 1.22 +/- 0.13, 4.57 +/- 0.49 and 13.45 +/- 0.71 impulses/s, respectively, for stimulus temperature rates of 0.3, 2.0 and 6.0 degrees C/s. 4. Magnitude estimates of pain for thirteen subjects also increased with rate of rise of temperature stimuli. Mean normalized magnitude estimates of heat pain were: 11.8 +/- 1.55, 15.1 +/- 0.84 and 28.0 +/- 1.87 for stimulus rates of rise of 0.3, 2.0 and 6.0 degrees C/s, respectively. 5. Results of simultaneous recordings of reaction time for pain and of C nociceptor responses to heat ramps given at 2.0 degrees C/s, in three subjects, indicate that under those circumstances heat pain messages are exclusively mediated by C nociceptors.