Erythromelalgia is a rare neurovascular pain syndrome characterized by a triad of redness, increased temperature, and burning pain primarily in the extremities. Erythromelalgia can present as a primary or secondary form, and secondary erythromelalgia associated with a myeloproliferative disease such as essential thrombocythemia often responds dramatically to aspirin therapy, as in the present case. Herein, we describe a typical case of a 48-year-old woman with secondary erythromelalgia linked to essential thrombocythemia in the unilateral hand. As this case demonstrates, detecting and visualizing the hyperthermal area through infrared thermography of an erythromelalgic patient can assist in diagnosing the patient, assessing the therapeutic results, and understanding the disease course of erythromelalgia.
Aspirin ; Burns ; Erythromelalgia ; Extremities ; Female ; Hand ; Humans ; Neuralgia ; Thermography ; Thrombocythemia, Essential

No abstract available.
Paramyxoviridae Infections* ; Polymerase Chain Reaction* ; Reverse Transcription*

BACKGROUND: Paroxysmal atrial fibrillation (PAF) causes not only severe symptoms and hemodynamic changes, but may progress to chronic atrial fibrillation. Autonomic nervous system or atrial premature beat (APB) has been suggested to contribute to the spontaneous initiation of PAF, but the exact mechanism has been largely unknown. METHODS: One hundred and twenty nine episodes of PAF lasting longer than 5 sec were analyzed in 18 patients (M:F=11:?). Two minutes of normal sinus rhythm before the onset of PAF, and the initial one minute of PAF were printed and analyzed. RESULTS: Most of PAFs were initiated by APBs (38%) or rapid atrial tachycardias (AT, 59%). The frequency of APBs tended to increase immediately before PAF onset (p=0.08). The coupling intervals and coupling indices were not significantly different between PAF-producing APBs and benign APBs. More than half of PAF episodes were initiated by rapid ATs (rate, 357+/-50 bpm). After the onset, they accelerated over several seconds and then degenerated into AF. In some cases, transition from AF to atrial flutter and vice versa were observed. Heart rate, measured at 60-second intervals during 2 minutes before PAF onset, did not change significantly (p=0.44). CONCLUSION: Most of PAFs were initiated by APBs or rapid ATs. Heart rate did not change significantly but the frequency of APBs tended to increase immediately before PAF onset. Rapid ATs frequently accelerated and degenerated into AF. In this regard, Holter monitoring could be useful in identifying patients with PAF triggered by rapid ATs.
Atrial Fibrillation* ; Atrial Flutter ; Autonomic Nervous System ; Cardiac Complexes, Premature ; Electrocardiography, Ambulatory* ; Heart Rate ; Hemodynamics ; Humans ; Tachycardia

No abstract available.
Blood Flow Velocity*

Hemangioblastoma is uncommon, accounting for 1 to 2.5% of all primary neoplasms of the central nervous system. Ten to 20% of hemangioblastomas occur as part of Von Hippel-Lindau disease(VHL). Multiple hemangioblastomas are seen only with VHL and these are seen in up to half of VHL-associated hemangioblastomas. We have treated two cases of multiple hemangioblastomas associated with VHL disease in a family, sister(case I, 48/female) and her brother(case II, 41/male). Both patients had renal and pancreatic cysts in addition to CNS hemangioblastomas. Their hemangioblastomas were removed totally, and then their neurological symptoms had Improved. With a review of the literature, the authors present a family of VHL-associated multiple hemangioblastomas.
Central Nervous System ; Hemangioblastoma* ; Humans ; Pancreatic Cyst

The microvasculature of the brain is also quite susceptible to ischemic insult, and substantial portions of the brain are not reperfused after restoration of the blood supply following overtime of critical ischemic periods. The purpose of this series of experiments was to determine the effects of ischemia on subsequential regional cerebral blood flow measurements and cortical electric activities following reperfusion after ischemia and also to define the proper time of vascular occlusion without irreversible neural damage. Cerebral ischemia was induced in cat by bilateral common carotid occlusions for periods of 10, 30, to 60 minutes, and the blood flow(rCBF) was measured by hydrogen clearance technique following ischemia, restoration of blood supply and electroencephalogram recovery could be predicted according to the rCBF. Forty adult cats weighing 2.7 to 4.0kg were used in this study. The animals were divided into 4 groups of 10 cats each : normal control, 10 min-clamped, 30 min-clamped, and 60 min-clamped groups. The results obtained were as follows : 1) The mean rCBF was 24.6+/-7.0ml/100g/min in control group. 2) Bilateral carotid occlusions resulted in a reduction of the rCBF (12.4+/-4.1ml/100g/min) to 50% of control flow on both hemispheres. 3) Sequential changes of the rCBF after reperfusion. (1) There was restored the rCBF(21.3+/-5.1ml/100g/min) to control flow in the 10 minutes-clamped group. (2) There was a 85% recovery of control flow in the 30 minutes-clamped group. (3) There was a only 25% recovery of control flow in the 60 minutes-clamped group. 4) A close correlation was found between cortical electrical activity and rCBF suggesting a threshold relationship. (1) The changes of cortical electric activity began to notice at rCBF less than 17.4+/-4.7ml/100g/min. (2) The changes of cortical electric activity noted at rCBF more than 10.2+/-2.3ml/100g/min. 5) There was no evidence of ischemic involvement at the cortex, white matter and basal ganglia in the 10 minutes clamped group, but demonstrated a dense wedge shaped infarct at the cortex and uncus herniation in the 60 minutes clamped group. The rCBF and cortical electric activity restorted to normal values in reperfusion within 10 minutes after occlusion of both common carotid arteries.
Adult ; Animals ; Basal Ganglia ; Brain ; Brain Ischemia ; Carotid Artery, Common* ; Cats* ; Electroencephalography ; Humans ; Hydrogen ; Ischemia ; Microvessels ; Reference Values ; Reperfusion*

A visual stimulus elicits a complicated series of potentials from the human cortex. The visual evoked potential test has advantages in detecting lesions that alter electrophysiology but do not produce detectable alterations of radiodensity, displace surrounding structures or change vascular supply. A sequence of four wave component(I, II, III, IV) that occured in the 100 msec following the flash stimulation can be recorded from scalp electrodes in 20 patients with focal lesions of the visual conducting system using computer averaging techniques. 1) 20 patients presenting with visual symptoms were classified as four groups according to the locations of the lesions in visual pathway : optic nerve lesion(8 cases), chiasmatic lesion(5 cases), optic tract or radiation lesions(4 cases) and visual cortical lesion(3 cases). 2) In optic neuritis, wave patterns of VEP was suppressed markedly in I(P30~40 msec) and II(N50~60 msec) wave components and in the optic nerve injuries, flat wave patterns were noted. 3) In chiasmatic lesions, VEP showed mild prolongation of latency(wave II, N50~60 msec) throughout and mild supperssion of the amplitudes of the wave components presented. On stimulation of one eye, latency and amplitude of the contralateral sided eye were markedly delayed and suppressed. 4) VEP of optic tract and optic radiation lesions showed marked prolongation and suppression of wave pattern in late components(wave II, III). In multiple sclerosis, VEP abnormality was presented in early state(N80~90 msec). 5) In the visual cortical lesions, the wave patterns of the VEP were almost symmetrically flat on both side. Serial evaluation of the VEP could provide good information for the evaluation of visual pathway lesions and functional recovery marker.
Electrodes ; Electrophysiology ; Evoked Potentials, Visual* ; Humans ; Multiple Sclerosis ; Optic Nerve ; Optic Nerve Injuries ; Optic Neuritis ; Scalp ; Visual Pathways*