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

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.
Paramyxoviridae Infections* ; Polymerase Chain Reaction* ; Reverse Transcription*

No abstract available.
Blood Flow Velocity*

PURPOSE: To determine whether 1H magnetic resonance spectroscopy (MRS) is useful in differentiating idiopathic Parkinson's disease (IPD) from progressive supranuclear palsy (PSP), based on metabolite ratios. MATERIALS AND METHODS: Using a 1.5 T MR Unit, single voxel 1H MRS using STEAM with a TR of 2000ms and a TE of 135ms was performed in seven PD and eight PSP patients. Five age-matched volunteers(mean age, 63 years) andanother five younger healthy volunteers(mean age, 30 years) were studied as normal controls. The regions of interest were the putamen and pallidum, with a size of 2 X 2 X 2cm. After measuring the spectral intensities ofeach metabolite (N-acetylaspartate=NAA, choline=Cho, creatine=Cr and lactate), relative peak height ratios ofNAA/Cr, Cho/Cr and Naa/Cho, and lactate levels among four groups were compared. ESULTS: NAA/Cho and NAA/Crratios were statistically lower in the PSP group than the IPD group (1.21 +/-0.26 versus 1.45 +/-0.20, and 1.26 +/-.23 versus 1.38 +/-0.19, respectively : p<0.05). NAA/Cho and NAA/Cr ratios were significantly lower inage-matched controls than in younger normal controls (1.39 +/-0.21 versus 1.76 +/-0.15, and 1.36 +/-0.13 versus1.79 +/-0.17, respectively : p<0.05). However, NAA/Cho and NAA/Cr ratios between age-matched controls and IPD werenot significantly different (p>0.05). Cho/Cr ratios were not different among four groups. Lactate was not detectedin any patients. CONCLUSION: NAA/Cho and NAA/Cr ratios in the corpus striatum were significantly lower in the PSP group than in the age-matched control and IPD groups. These results suggest that loss of neuron cells in thecorpus striatum is more prominent in PSP than in IPD, and that NAA/Cho and NAA/Cr ratios may help in differential diagnosis of IPD and PSP.
Corpus Striatum ; Humans ; Lactic Acid ; Magnetic Resonance Spectroscopy* ; Neurons ; Parkinson Disease* ; Putamen ; Steam ; Supranuclear Palsy, Progressive*

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

PURPOSE: To evaluate regional differences of 1H magnetic resonance(MR) spectral patterns in normal adulthuman brains. MATERIALS AND METHODS: A total of 44 1H MR spectra in 25 volunteers aged 27-45 were obtained infive regions including the frontal lobe(10), parietal lobe(10), temporal lobe(5), basal ganglia(10) and thalamus(9). 1H MR spectroscopy(MRS) was performed using a PRESS sequence with a TR of 2000ms and a TE of 270msfrom a volume of cm on a 1.5T clinical MR unit. Relative metabolite ratios of NAA/Cho, NAA/Cr and CR/Cho in eachregion were measured and compared. RESULTS: A total of 44 reliable spectra were successfully obtained in allregions. NAA/Cho, NAA/Cr and Cr/Cho ratios varied considerably, ranging from 1.09 +/-0.2 to 2.46 +/-0.25, from1.72 +/-0.35 to 2.45 +/-0.25 and from 0.64 +/-0.1 to 1.01 +/-0.12, respectively. Significant regional difference sin metabolite ratios were observed; higher NAA/Cho and NAA/Cr ratios in the parietal lobe, lower NAA/Cho ratios inthe temporal lobe, and lower Cr/Cho ratios in the temporal lobe compared to those of other regions(p<0.05). Differences in metabolite ratios between the right and left frontal lobes, and between the right and left basalganglias were not significant. CONCLUSION: 1H MR spectra of the normal adult human brains using in vivo singlevoxel 1H MRS represented significant regional differences in metabolite ratios of NAA/Cho, NAA/Cr and Cr/Cho. Our1H MR spectroscopic results are a useful ueference for assessing the 1H MRS pattern of various intracranial diseases.
Adult* ; Brain* ; Frontal Lobe ; Humans ; Parietal Lobe ; Rabeprazole ; Temporal Lobe ; Volunteers