[Patent foramen ovale presented hypoxemia with cardiac sarcoidosis].

A 53-year-old woman with a double, double, double (DDD) pacemaker due to complete atrioventricular block was admitted to our hospital with a diagnosis of congestive heart failure. At the time of admission, she was in a hypoxic state with cyanosis and clubbed finger. The ultrasonic cardiogram showed a severe degree of tricuspid valve regurgitation and a thin left ventricular septal wall. Transesophageal echocardiography revealed a patent foramen ovale (PFO) with continuous right to left shunt flow. She was diagnosed with cardiac sarcoidosis with hypoxemia caused by PFO. PFO closure and tricuspid valve annuloplasty (DeVega method) were performed. Following surgery, the patient's hypoxemia improved and the cyanosis disappeared. The patient was discharged 37 days after the operation.

Detection of anti-neutrophil cytoplasmic antibodies in MRL/Mp-lpr/lpr mice and analysis of their target antigens.

Anti-neutrophil cytoplasmic antibodies (ANCA) have been widely studied and recognized to be clinically very important for some human diseases including systemic rheumatic diseases. We analyzed ANCA response and their target antigens in MRL/Mp-lpr/lpr (MRL-lpr) mice, an animal model of systemic rheumatic disease. P-ANCA was detected in 57% of the mice. Antibodies to the known P-ANCA target antigens at the same age were examined. Among these, antibodies to high mobility group (HMG) proteins HMG1 and HMG2 were detected in 57% of the mice, 75% of which were also positive for P-ANCA. These anti-HMG1/HMG2 activities were absorbed by preincubation with a mixture of HMG1 and HMG2. In contrast, antibodies to myeloperoxidase and cathepsin G were detected in 14% and 7%, respectively, but these activities were not inhibited by preincubation with corresponding antigens. In addition, the titers of P-ANCA and anti-HMG1/HMG2 antibodies in MRL-lpr mice were significantly correlated with each other. Thus, HMG1 and HMG2 were considered to be significant target antigens of P-ANCA in MRL-lpr mice.

Possible association of active gastritis, featuring accelerated cell turnover and p53 overexpression, with cancer development at anastomoses after gastrojejunostomy. Comparison with gastroduodenostomy.

To cast light on tumorigenesis in the remnant stomach after distal gastrectomy for peptic ulcer or gastric cancer, 45 cases in gastroduodenostomy (Billroth I, 17 cases) and gastrojejunostomy (Billroth II, 28 cases) groups were compared for a series of parameters. Cancers in Billroth II were significantly more predominant in the anastomosis area and more frequently associated with Epstein-Barr virus infection. Active gastritis, accelerated epithelial cell turnover (as assessed by measurements of apoptosis and cell proliferation), DNA damage, and foveolar cell hyperplasia were all greater in anastomotic areas after Billroth II than in proximal areas after Billroth II or either area after Billroth I. K-ras mutations were rare, but Epstein-Barr virus infection in cancers was seen frequently in anastomosis cases. In conclusion, active gastritis, possibly induced by enterogastric reflux, is linked to tumorigenesis in anastomosis sites in Billroth II cases.

Predominant activation of I1-waves from the leg motor area by transcranial magnetic stimulation.

We performed transcranial magnetic stimulation (TMS) to elucidate the D- and I-wave components comprising the motor evoked potentials (MEPs) elicited from the leg motor area, especially at near-threshold intensity. Recordings were made from the tibialis anterior muscle using needle electrodes. A figure-of-eight coil was placed so as to induce current in the brain in eight different directions, starting from the posterior-to-anterior direction and rotating it in 45 degrees steps. The latencies were compared with those evoked by transcranial electrical stimulation (TES) and TMS using a double cone coil. Although the latencies of MEPs ranged from D to I3 waves, the most prominent component evoked by TMS at near-threshold intensity represented the I1 wave. With the double cone coil, the elicited peaks always represented I1 waves, and D waves were evoked only at very high stimulus intensities, suggesting a high effectiveness of this coil in inducing I1 waves. Using the figure-of-eight coil, current flowing anteriorly or toward the hemisphere contralateral to the recorded muscle was more effective in eliciting large responses than current flowing posteriorly or toward the ipsilateral hemisphere. The effective directions induced I1 waves with the lowest threshold, whereas the less effective directions elicited I1 and I2 waves with a similar frequency. Higher stimulus intensities resulted in concomitant activation of D through I3 waves with increasing amount of D waves, but still the predominance of I1 waves was apparent. The amount of I waves, especially of I1 waves, was greater than predicted by the hypothesis that TMS over the leg motor area activates the output cells directly, but rather suggests predominant transsynaptic activation. The results accord with those of recent human epidural recordings.

Single fiber EMG and repetitive nerve stimulation of the same extensor digitorum communis muscle in myasthenia gravis.

OBJECTIVE: To compare voluntary single fiber electromyography (v-SFEMG) and repetitive nerve stimulation (RNS) at the same extensor digitorum communis (EDC) muscle in myasthenia gravis (MG). METHODS: We examined v-SFEMG and RNS successively on the same day in the same EDC muscle. We studied 45 examinations of both v-SFEMG and RNS in 29 patients suffering from MG, together with examinations of RNS in 30 control subjects. RESULTS: Forty-one of 45 (91%) v-SFEMGs showed abnormal results, whereas only 18/45 (40%) RNSs showed an abnormal decrement. The percentage of decrement showed similar correlations with 3 v-SFEMG parameters: percentage of abnormal pairs, percentage of blocking pairs, and the mean MCD value. Examinations showing a significant decrement in RNS had at least 60%, and usually no less than 90%, abnormal pairs, and 10-80% blocking pairs. Some muscles without a decrement had up to 50% blocking pairs. CONCLUSIONS: These results suggest that the blocking phenomenon observed in v-SFEMG is not a direct counterpart of the decrement in RNS. This must be partly because fibers contributing to the decrement are continuously blocked during voluntary contraction, and partly, because smaller motor units explored by v-SFEMG are probably more abnormal in MG than larger motor units mainly contributing to a decrement. Both factors make v-SFEMG much more sensitive than RNS.

Somatosensory evoked high-frequency oscillation in Parkinson's disease and myoclonus epilepsy.

AIM: A high-frequency oscillation in the range of 600-900 Hz has been shown to be a component of the somatosensory evoked potential (SEP) in humans. In the present communication, we studied these oscillation potentials in two neurological disorders. SUBJECTS AND METHODS: Subjects were 20 healthy volunteers, 17 patients with Parkinson's disease (PD) and 3 with myoclonus epilepsy (ME). Median nerve SEPs were recorded using filters set at 0.5 and 3000 Hz. Several peaks of oscillation were obtained by digitally filtering raw SEPs from 500 to 1000 Hz, and their amplitudes and onset latencies were measured. RESULTS: In normal subjects, several oscillation potentials were observed at the latency of 0 to 8 ms after the onset of N20. In PD patients, the oscillation potentials at normal latencies were significantly larger than those of normal subjects. Moreover, in 7 of 17 PD patients, they were extremely enlarged (>mean +/- 3 SD of normal values). In contrast, in patients with ME, abnormally enlarged oscillation potentials were seen at longer latencies (7-14 ms) in spite of normal-sized early oscillation potentials. Magnetoencephalographic analyses showed that any oscillation potentials originated from the primary sensory cortex. CONCLUSIONS: There are at least two mechanisms for producing the oscillation potentials of SEP. Those around N20 have some relation with the basal ganglia function and are enlarged in PD patients, the others around P25-N33 are enhanced in ME patients.

Input-output organization of the foot motor area in humans.

OBJECTIVE: A well-organized input-output relation similar to that of the monkey motor cortex has been demonstrated in the human hand motor area (Terao Y, Ugawa Y, Uesaka Y, Hanajima R, Gemba-Shimizu K, Ohki Y, Kanazawa I. Input-output organization in the hand area of the human motor cortex, Electroenceph clin Neurophysiol 1995;97:375-381). The aim of this study is to investigate the input-output organization of the human foot motor area. METHODS: We studied the effect of tactile stimuli given to the toe tip on the sizes of following responses; motor evoked potentials (MEPs) elicited by transcranial magnetic or electrical stimulation (TMS or TES) over the motor cortex and magnetic stimulation at the foramen magnum level. RESULTS: Air stimuli applied to the toe tip facilitated magnetically evoked MEPs of mainly the muscle attached to that toe, although a less prominent facilitation was also noted in muscles attached to the adjacent toes. Neither responses evoked by TES, nor those by stimulation at the foramen magnum level, were affected by air stimuli. These results suggest that the observed facilitatory effect occurs at the cortical level. CONCLUSION: A fairly well-organized input-output relation is present also in the foot motor area in humans, although the facilitatory effect is not so topographically restricted as is noted for the hand motor area.

The human hand motor area is transiently suppressed by an unexpected auditory stimulus.

OBJECTIVE: To study the effect of a loud auditory stimulus on the excitability of the human motor cortex. METHODS: Ten normal volunteers participated in this study. The size of responses to transcranial magnetic or electrical cortical stimulation (TMS or TES) given at different times (ISIs) after a loud sound were compared with those to TMS or TES alone (control response). Different intensities and durations of sound were used at several intertrial intervals (ITIs). In addition, we examined how the presence of a preceding click modulated the effect of a loud sound (prepulse inhibition). The incidence of startle response evoked by various stimuli was also studied. RESULTS: A loud auditory stimulus suppressed EMG responses to TMS when it preceded the magnetic stimulus by 30-60 ms, whereas it did not affect responses to TES. This suggests that the suppression occurred at a cortical level. Significant suppression was evoked only when the sound was louder than 80 dB and longer than 50 ms in duration. Such stimuli frequently elicited a startle response when given alone. The effect was not evoked if the ITI was 5 s, but was evoked when it was longer than 20 s. A preceding click reduced the suppression elicited by loud sounds. CONCLUSIONS: Auditory stimuli that produced the greatest effect on responses to TMS had the same characteristics as those which yielded the most consistent auditory startle. We suggest that modulation of cortical excitability occurs in parallel with the auditory startle and both may arise from the same region of the brain-stem.

Corticonigral degeneration with neuronal achromasia presenting with primary progressive aphasia: ultrastructural and immunocytochemical studies.

We describe a clinico-pathological variant of a degenerative disorder involving Broca's, Wernicke's, and supplementary motor areas, which presented as primary progressive aphasia, dysarthria, bucco-facial apraxia, and hearing loss as initial symptoms, followed by organic personality changes. Postmortem examination revealed severe focal atrophy of the cerebral convolutions in the frontal operculum, superior frontal gyrus, and superior and transverse temporal gyri in addition to diffuse atrophy of the frontal and temporal lobes in both hemispheres. Microscopical examination revealed argyrophilic neuronal inclusions (ANIs) in the neuronal perikarya and presynaptic terminal throughout the central nervous system, as well as neuronal loss and swollen chromatolytic neurons in the affected cortices. Neocortical ANIs showed a positive immunoreaction with an anti-tau antibody but only a weak reaction with an anti-ubiquitin antibody immunohistochemically. Ultrastructurally, neocortical ANIs consisted of 15-nm thick smooth-surfaced tubules and tubules with constrictions at 120-150-nm intervals; thus they were different from the typical paired helical filaments of the 80-nm interval constrictions observed in the subiculum. ANIs were also found in the basal ganglia, brain stem nuclei, and cervical cord. Accordingly, ANIs appear distinct from neurofibrillary tangles (NFTs) of progressive supranuclear palsy, NFTs of Alzheimer-type dementia, and Pick bodies. The authors consider that this case fits the histopathological criteria of corticonigral degeneration with neuronal achromasia except for the unusual extension to the temporal lobes.

A single motor unit recording technique for studying the differential activation of corticospinal volleys by transcranial magnetic stimulation.

The purpose of this method is to establish a single motor unit recording technique to study the differential activation of corticospinal volleys by various types of transcranial magnetic stimulation (TMS). TMS is performed with various coil orientations over the hand or leg motor areas and surface EMG, and single motor unit recordings are made either from the studied hand or leg muscle. Transcranial electrical stimulation (TES) is also performed over the motor cortex as well as at the foramen magnum level to determine the latency of D waves. The intensity of stimulation is set just above the motor threshold for each type of stimulation. This method makes it possible to activate some I volleys (especially I1 and I3 waves) preferentially, if not selectively, from the hand and leg motor areas. The obtained results accord well with recent epidural recording studies, which lends support to the validity of this method.

[Establishment and characterization of strain KE-39 derived from human gastric cancer].

We succeeded in establishing a human gastric carcinoma cell line (KE-39) from oncocytes obtained from the primary focus of a 77-year-old male stomach cancer patient. From a histopathological point of view the gastric carcinoma was a poorly differentiated adenocarcinoma exhibiting a funicular from and a structure with a solid vesicular focus. The oncocytes adhered to glass and proliferated in cell clusters, with a doubling time of about 38.4 hours. Upon transplantation of the cancer cells into nude mice, no visible tumors were found, but from a histological point of view poorly differentiated adenocarcinoma similar to the primary focus was found in all cases. With immunological staining they were found to be positive for anti-CEA antibody and anti-CA19-9 antibody, but negative for anti-ICAM-1 antibody. KE-39 is a cell line which was established from the primary focus, and it was reported in the belief that it is a useful cell line, upon the investigation of its cancer metastasis mechanism and cytological characteristics.

Impact of pre-stent plaque debulking for chronic coronary total occlusions on restenosis reduction.

BACKGROUND: While stenting improves the long-term angiographic outcomes of successfully recanalized chronic coronary total occlusions (CTO), the restenosis rate still remains high. The massive plaque burden in CTO is considered to be one of the causes of in-stent restenosis. METHODS: We examined the pre-stent plaque debulking strategy with high-speed rotational atherectomy (RA) for 50 CTO (Thrombolysis in Myocardial Infarction flow grade 0; estimated occlusive duration, 3 months). Angiographic follow-up results were compared to those of 120 consecutive CTO recanalized with primary stenting in which RA could be indicated retrospectively. Angiographic restenosis was defined as diameter stenosis > 50% at 6-month follow-up. RESULTS: RA could be performed safely in all lesions without any major complications. Adjunctive ballooning and stenting could be performed without high-pressure dilatation (8.4 +/- 1.7 atmospheres). Follow-up angiography was performed in 48 lesions 184 +/- 61 days after the procedure. There were no significant differences in baseline characteristics between the two groups; however, the implanted stent type was different. Quantitative coronary angiography revealed that diameter stenosis was smaller at follow-up (36.2 +/- 20.0% versus 52.2 +/- 26.7%; p = 0.0003) as well as post-procedure (7.8 +/- 11.5% versus 17.8 +/- 13.6%; p < 0.0001) compared with the control group. Angiographic restenosis was also significantly reduced (29.2% versus 52.5%; p = 0.0061). CONCLUSIONS: RA is a safe procedure for plaque debulking of CTO in selected cases. Plaque debulking of CTO facilitates subsequent stent expansion and may reduce the restenosis rate.

Polymyositis as a paraneoplastic manifestation of hepatocellular carcinoma.

A case of hepatocellular carcinoma associated with polymyositis is reported. A 70-year-old man noticed muscular weakness mainly in the proximal limb muscles. The clinical course, a raised level of serum creatine kinase and electromyographic findings suggested polymyositis, and the pathological findings on muscle biopsy were compatible with this diagnosis. Computed tomography of the upper abdomen revealed a mass lesion in segment VII and VIII of the liver, which was diagnosed pathologically as hepatocellular carcinoma. The patient underwent systematic resection of segment VII and the dorsal part of segment VIII of the liver. After surgery, the weakness improved and the serum creatine kinase level normalized without medical treatment for the polymyositis. The relief of neurological symptoms and signs after complete resection of the tumor strongly suggests paraneoplastic polymyositis, which has been described only rarely in association with hepatocellular carcinoma.

Regional cerebral blood flow measurement using Tc-99m HMPAO SPECT in a patient with adrenoleukodystrophy.

Regional cerebral blood flow (rCBF) measurement using Tc-99m HMPAO angiography and SPECT was performed in a patient with adrenoleukodystrophy. The whole brain CBF showed great reduction to 31 ml/100 g/min as compared with an age-matched normal value of 45.9 ml/100 g/min. rCBF showed extremely decreased values of approximately 28 and 10 ml/100 g/min in bilateral frontal cortex and white matter respectively. The SPECT scan provided useful information on gray matter involvement to MRI findings of widespread white matter lesions. This quantitative nuclear medicine technique may contribute to pathophysiologic evaluation of adrenoleukodystrophy complementary with MRI.

Interictal cerebral and cerebellar blood flow in temporal lobe epilepsy as measured by a noninvasive technique using Tc-99m HMPAO.

The usefulness of noninvasive regional cerebral blood flow measurements using Tc-99m hexamethylpropylene amine oxime (HMPAO) was evaluated in 45 patients with interictal temporal lobe epilepsy. Seizure frequency did not show any influence on flow values or side-to-side asymmetry. Patients taking phenytoin had a significantly lower mean cerebral blood flow (mean 9.5%) and lower mean cerebellar blood flow (mean 12.9%) than those not taking it. Moreover, phenytoin reduced regional cerebral blood flow in the temporal regions more prominently on the contralateral side of the epileptic focus than on the ipsilateral side. Positive MRI findings of hippocampal sclerosis along with visually detected temporal hypoperfusion on SPECT showed a lower regional cerebral blood flow and greater flow asymmetry than other imaging findings in the temporal region. Results suggest that noninvasive cerebral blood flow measurements using Tc-99m HMPAO may give useful information about interictal cerebral blood flow around an epileptic focus and about the effects of antiepileptic drugs on brain function in temporal lobe epilepsy.

[The effect of intraoperative high-dose tranexamic acid on blood loss after operation for acute aortic dissection].

The effect of high dose tranexamic acid on blood loss after operations for acute aortic dissection was evaluated. Twenty-eight patients undergoing emergent operations for acute aortic dissection were studied. There were two groups, group T with 13 patients (group T) who were given 7 g of tranexamic acid after induction of anesthesia and 3 g of it after CPB and group C with 15 patients who did not receive tranexamic acid. There was a tendency that group T had less bleeding during operation and after operation (559.6 +/- 865.8 ml in group T and 805.8 +/- 442.9 ml in group C, 1719.2 +/- 1008.7 ml in group T and 3547.7 +/- 4580.1 ml in group C, respectively), but there was no significant difference between two groups. The removal of drainage tubes after operation was significantly earlier in group T (5.0 +/- 2.3 post operative day in group T and 8.1 +/- 5.2 post operative day in group C; p < 0.05). FDP and D-dimer level as measures of fibrinolytic activity were elevated at pre- and postoperative period in both groups, but they tended to be lower in group T at postoperative period. One patient required reexploration because of excessive bleeding and no mediastinal infection was reported in group T, whereas 4 patients underwent reexploration and 2 patients developed mediastinitis in group C. There were 5 hospital death (33.3%) in group C and 2 (15.4%) in group T. High dose of tranexamic acid seems to control fibrinolytic activity, thereby reducing blood loss and requirements, which may contribute to lower morbidity and mortality in operations for acute aortic dissection.

[Relationship between imaging and pathological features and clinical factors in surgical cases of temporal lobe epilepsy].

The relationships between imaging, pathology and presumed causes in surgical cases of temporal lobe epilepsy (TLE) was studied. The subject was 62 patients (33 males and 29 females) who had had no attacks for more than one year after surgery. Average age at surgery was 28.2 +/- 9.9 years. Obvious neurological abnormalities were not found in these cases. MRI, PET and SPECT were performed. Hematoxylin and eosin was used for pathological judgement. Their medical charts were used to investigate their clinical factors. Although patients suspected of having encephalitis/meningitis had been hospitalized for 2 days to three months during childhood due to disturbance of consciousness with high fever and convulsion for several days; they were not diagnosed with encephalitis/meningitis at that time, and they suffered almost no handicaps other than epilepsy for several years following their comatose episodes. [Result] (1) On MRI, mesial temporal sclerosis (MTS) was detected in 48 of 52 patients (92%); 32 (62%) had high-signal intensity on T 2-weighted images; 31 (60%) had atrophy ¿23 (44%) had high-signal intensity on T 2 + atrophy¿; 5 (10%) had calcified lesions; and 2 (4%) had cystic lesions. On PET and SPECT, abnormal cerebral blood flow was noted in 33 of 36 (92%). (2) On pathological findings (61 cases), Ammon's horn sclerosis (AHS), tumors, gliosis in lateral temporal and meningeal inflammatory finding were detected in 42 (69%), 10 (23%) and 8 (13%) cases, respectively, whereas 2 showed no abnormalities. The 2 patients with normal pathology showed both high-signal intensity and atrophy on MRI. (3) The presumed causes of TLE were encephalitis/meningitis and/or suspected of these diseases in 15 patients (24%), injuries at birth in 5 (8%), and none in 42 (68%). The presumed causes in the 43 patients with AHS were encephalitis/meningitis in 11, injuries at birth in 3, and none in 29. Of the 15 patients in whom encephalitis/meningitis was estimated as the causes of TLE, only 6 (40%) had pathological evidence of meningeal inflammatory finding. Of the 42 patients in whom cause could not be determined, 2 had pathological evidence of meningeal inflammatory finding.

[Mitral valve replacement under cardiopulmonary bypass which complicated by multiple sclerosis: a case report].

The 60-year-old women who complicated by multiple sclerosis was referred to our hospital under diagnosis of mitral valve stenosis and tricuspid valve regurgitation. The mitral valve replacement and the tricuspid valve annuloplasty were performed under cardiopulmonary bypass. In the state of multiple sclerosis, even an operation by general anesthesia could become a cause of abrupt change, but by appropriate management during and after surgery, the post operative course was uneventful without any complications, and the patient discharged on the 35th post operative day.

[A case report of surgical treatment for coronary aneurysm following repeated PTCA].

A 53-year-old man who developed coronary artery aneurysm following repeated percutaneous transluminal coronary angioplasty (PTCA) was reported. At the first coronary angiography, the severity of coronary stenosis was 95% at areas #6 and #7. The first PTCA provided sufficient coronary dilatation, but 3 months later, PTCA was needed again because of the recurrence of stenosis. This second PTCA provided sufficient dilatation, but the coronary dissection remained. Eight months after the second PTCA, the patient suffered from recurrent angina. The repeated coronary angiography showed 95% stenosis of the original lesion and coronary aneurysm formation (4 mm in diameter) at the area of dissection which developed at the former PTCA site. Therefore, coronary artery bypass surgery with left internal thoracic artery to area #8 was performed.

[Aortic valve replacement with the Toronto stentless porcine valve in a patient with clipping for cerebral arterial aneurysm].

A 48-year-old man, who had a cerebral arterial aneurysm, was admitted in our institution for operation of aortic valve stenosis. At first, he underwent clipping for cerebral arterial aneurysm under precise management of his hemodynamic condition. After the clipping operation, we performed aortic valve replacement with the Toronto stentless porcine valve because no anticoagulant therapy was ideal for patient with cerebrovascular disease and larger effective orifice area was preferable for stenotic aortic annulus. By means of echocardiography, mean pressure gradient of the aoric valve decreased from 42 mmHg to 22 mmHg after the valve operation. He was discharged from the hospital on the 23rd postoperative day, and he has been doing well without thromboembolic events and bleeding complications for five postoperative months. This experience suggest that the Toronto stentless porcine valve might be one of the valve of choice for patients with aortic valve disease and cerebrovascular disease.