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Therapeutic occlusion of the vertebral artery (VA) is one of the treatments for unclippable aneurysms and other lesions, although controversy still surrounds the appropriate site for occlusion to attain selective thrombosis of the lesion while avoiding ischaemic complications. The lower two-thirds of the lateral medulla are supplied by perforating branches of both the VA and the posterior inferior cerebellar artery (PICA). However, in patients without a PICA or in whom the origin of the PICA is low (at or below the foramen magnum), the VA is usually the only source of perforating vessels. We retrospectively studied the results of VA occlusion on such anatomically high-risk patients, and propose a safer procedure. Five high-risk patients underwent therapeutic occlusion of the VA for dissecting aneurysms or arteriovenous fistula. A lateral medullary syndrome developed due to propagation of thrombus after the procedure in two patients in whom angiography did not demonstrate the anterior spinal artery (ASA) within the stump of the VA. Ischaemic signs did not develop in the other three patients, in whom the ASA was visible, and retrograde flow was observed proximal to the origin of the ASA. This suggests that the ASA may play a role in preventing propagation of thrombus in the VA distal to the site of occlusion and supply blood to its perforating arteries in high-risk patients. Angiographic assessment of the ASA may be useful for predicting the likelihood of the lateral medullary syndrome developing with therapeutic occlusion of the VA in patients without a PICA or with one whose origin is low.

The survival benefit of second-line chemotherapy with docetaxel in platinum-refractory patients with advanced esophageal cancer (AEC) remains unclear. A retrospective analysis of AEC patients with Eastern Cooperative Oncology Group performance status (PS)≤2 was performed, and major organ functions were preserved, who determined to receive docetaxel or best supportive care (BSC) alone after failure of platinum-based chemotherapy. The post-progression survival (PPS), defined as survival time after disease progression following platinum-based chemotherapy, was analyzed by multivariate Cox regression analysis using factors identified as significant in univariate analysis of various 20 characteristics (age, sex, PS, primary tumor location, etc) including Glasgow prognostic score (GPS), which is a well-known prognostic factor in many malignant tumors. Sixty-six and 45 patients were determined to receive docetaxel and BSC between January 2007 and December 2011, respectively. The median PPS was 5.4 months (95% confidence interval [CI] 4.8-6.0) in the docetaxel group and 3.3 months (95% CI 2.5-4.0) in the BSC group (hazard ratio [HR] 0.56, 95% CI 0.38-0.84, P=0.005). Univariate analysis revealed six significant factors: treatment, PS, GPS, number of metastatic organs, liver metastasis, and bone metastasis. Multivariate analysis including these significant factors revealed three independent prognostic factors: docetaxel treatment (HR 0.62, 95% CI 0.39-0.99, P=0.043), better GPS (HR 0.61, 95% CI 0.46-0.81, P=0.001), and no bone metastasis (HR 0.31, 95% CI 0.15-0.68, P=0.003). There was a trend for PPS in favor of the docetaxel group compared with patients who refused docetaxel treatment in the BSC group (adjusted HR 0.61, 95% CI 0.29-1.29, P=0.20). Docetaxel treatment may have prolonged survival in platinum-refractory patients with AEC.

To examine the effect of pulsed intravenous methylprednisolone (IVMP) on polymositis (PM) and dermatomyositis (DM), two biopsies were taken from the same muscle before and after IVMP in one case each of PM and DM. After IVMP, numerous muscle fibers were seen undergoing regeneration. Among all subsets of infiltrating cells that decreased in number, macrophages in the perivascular area decreased markedly. Also conspicuous was decrease of CD8+ cells in the endomysium in PM and that of B-cells in the perivascular area in DM. Among infiltrating cells, frequency of those which expressed the signal transducers and activators of transcription 1 (STAT-1) fell considerably, but remained still abnormally high. Regions on the endothelial cells of blood vessels that expressed MHC antigens and intercellular adhesion molecules 1 (ICAM-1) also decreased but remained more widespread than normal. These results agreed with the favorable clinical effect of IVMP, but showed that many of the immunological parameters remained abnormal.

OBJECTIVES—To evaluate the influences of chronic alcohol consumption on brain volume among social drinkers, as it is well known that alcohol misusers have a high risk of brain shrinkage. METHODS—Frontal lobe volumes on MRI were compared with the current alcohol habits of consecutive 1432 non-alcoholic subjects. RESULTS—After adjusting for other variables, age was found to be the most powerful promoting factor for the shrinkage with a odds ratio of 2.8 (95% confidence interval (95% CI) 1.23-3.06) for each 10 years of age. Regarding alcohol habit, 667 of the subjects were abstainers, and 157, 362, and 246 of the subjects were light (average 88.2 g ethanol/week), moderate (181.2 g/week), and heavy (418.1 g/week) drinkers, respectively. Moderate alcohol consumption did not increase the incidence of frontal lobe shrinkage (odds ratio 0.98; 95% CI 0.73-1.33), whereas heavy drinkers were at a higher risk compared with abstainers (1.80; 1.32-2.46). The contributory rate of alcohol consumption for frontal lobe shrinkage was 11.3%. CONCLUSION—The brain tends to shrink physiologically with age. Heavy alcohol consumption seems to exaggerate this shrinkage in social drinkers. Moderate alcohol consumption does not seem to affect brain volume.

A 31-year-old female was clinically diagnosed as having a anterior mediastinal yolk sac tumor because of the elevation of the AFP (17,500 ng/ml), a large mass lesion (9 x 5 cm) in the anterior mediastinum and bilateral lung metastases. After 4 courses of chemotherapy with cisplatin (CDDP), etoposide (VP-16) and bleomycin hydrochloride (BLM), the mediastinal mass reduced in size significantly and the serum AFP level reached within normal range. Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed a weak uptake in the mediastinum, accordingly the operation was performed. The tumor was completely removed and there were no viable foci of the tumor in part of the tumor. After the operation, 4 courses of chemotherapy with carboplatin (CBDCA), VP-16 and ifosfamide (IFM) were performed. She is alive without evidence of recurrence in 5 months after operation. It was noticed that the serum AFP is a useful indicator for determing the chance of operation after chemotherapy.

This prospective study was designed to elucidate the meaning of systemic inflammatory response syndrome (SIRS), the mechanisms that lead to the protraction of SIRS, and whether SIRS is potentially a simple and effective postoperative indicator or not in patients who are operated on with cardiopulmonary bypass (CPB). We studied the duration of SIRS in 90 patients. Our data showed that 83 (92%) of the 90 patients met 2 or more criteria for SIRS and that significant relationship between the protraction of SIRS and the duration of aortic clamping time, CPB time, operation time, and the risk of postoperative organ damage and complication. Those results suggest that SIRS after cardiac surgery with CPB can be regarded as right criteria and highly valuable indexes of preparatory state of organ dysfunction.

A 21 year-old woman was admitted to our hospital because of chest and back pain after blunt chest trauma. On admission, consciousness was clear and a physical examination showed labored breathing. Her vital signs were stable, but her breathing gradually worsened, and artificial respiration was started. The chest roentgenogram and a subsequent chest computed tomographic scans revealed contusions, hemothorax of the left lung and multiple rib fractures. A transthoracic echocardiography (TTE) revealed normal left ventricular wall motion and mild mitral regurgitation (MR). TTE was carried out repeatedly, and revealed gradually progressive MR and prolapse of the posterior medial leaflet, although there was no congestive heart failure. After her general condition had recovered, surgery was performed. Intraoperative transesophageal echocardiography (TEE) revealed torn chordae at the posterior medial leaflet. The leaflet where the chorda was torn was cut and plicated, and posterior mitral annuloplasty was performed using a prosthetic ring. One month later following discharge, the MR had disappeared on TTE.

To clarify the clinical features of genetically unclassified autosomal dominant spinocerebellar ataxias (AD-SCAs) treated in our hospital, we retrospectively analyzed the clinical findings of patients who could not be molecularly classified into SCA types 1 through 3, 6 through 8, 12 and dentato-rubro-pallido-luysian atrophy (DRPLA). The clinical findings of 19 patients from 15 different families which form 14 percent of all patients with different types of AD-SCAs (136 patients, 105 families) were collected. Based on these, 17 patients from 13 families showed late-onset (mean age at onset: 46 years old) and slowly progressive truncal and limb ataxias of cerebellar origin. Some of them showed gaze-evoked nystagmus, increased deep reflexes without Babinski sign, tremor, mildly decreased vibration sense and mild rectourinary disturbances. All but one were still ambulatory after 15 years of their mean disease duration. An earlier disease onset in successive generations was observed in 8 families. Brain MRIs revealed cerebellar atrophy predominantly in the anterior vermis in all the patients and mild brainstem atrophy in only 3 patients. Although the clinical phenotype manifested by most of the genetically unclassified AD-SCAs is similar to that of SCA6, whether the phenotype is caused by expansion of triplet repeats or some mutations in a single gene or different mutations in various genes is still unclarified.

Despite its potential clinical relevance, alpha(v)beta(3) expression has been analyzed only in a limited number of melanoma lesions, mostly nodular melanoma (NM) and superficial spreading melanoma (SSM). Therefore, in the present study, we have correlated alpha(v)beta(3) expression in 33 acral lentiginous melanomas (ALMs), 6 lentigo maligna melanomas, 7 mucosal melanomas, 12 NMs and 9 SSMs with their antigenic profile, with their histo-pathological characteristics and with the clinical course of the disease. Furthermore, we have compared alpha(v)beta(3) expression in ALM lesions with that in NM and SSM lesions since this information helps to clarify the relationship of the latter 2 histotypes with ALM. Such a relationship is uncertain since ALM has a clinical course similar to that of NM and SSM despite different antigenic profiles and biological characteristics. The level of alpha(v)beta(3) expression in primary lesions was not correlated with that of high-m. w. melanoma-associated antigen and intercellular adhesion molecule-1, with lesion thickness and with disease recurrence in ALM but was significantly correlated with these 4 parameters in the other melanoma histotypes analyzed. Therefore, alpha(v)beta(3) expression appears to have a differential clinical significance in ALM and in the other histotypes of melanoma we have analyzed since it appears to play a significant role in the progression of the disease only in non-ALM histotypes.

To identify lesions responsible for vocal cord abductor paralysis (VCAP) in multiple system atrophy (MSA), we performed a morphometric study of the nucleus ambiguus which innervates the intrinsic laryngeal muscles.Two autopsied cases of MSA presenting with VCAP and one control were examined. Both cases of MSA showed selective neurogenic atrophy of the posterior cricoarytenoid muscles among the intrinsic laryngeal muscles, while no abnormalities were seen in the control. From a block of the medulla oblongata, sections 10 microm thickness were cut serially without spacing and stained with cresyl violet. The ambiguus neurons were counted in all the sections to make a histogram.In the control case, ambiguus neurons showed densely populated areas and sparsely populated areas alternately with significant difference in the mean neuronal density between two areas. In MSA, ambiguus neurons were significantly decreased in number at all levels. It indicates that the neurogenic atrophy of the posterior cricoarytenoid muscle is derived from the neuronal loss of the nucleus ambiguus.Though it has still been controversial whether or not the ambiguus neurons are decreased in number in MSA with VCAP, we speculated possible reasons for the disagreement on the involvement of the nucleus ambiguus as follows: different mechanism of VCAP are playing role, and histometric data have been disturbed by factors such as split-cell counting error and marked variation in the distribution of the ambiguus neurons.

Human parvovirus B19 (HPV-B19) infection is recently known to produce variable clinical manifestations, but neurological disorder associated with this infection is uncommon. We reported a case of GBS associated with transient lupus-like status following HPV-B19 infection. This is the first report describing an adult case of GBS following HPV-B19 infection. A healthy female, aged 33, developed erythema infectiosum simultaneously with her 5-year-old daughter. On the same day, she noticed leg fatigue, which worsened in the following days. On the day 11th, she became unable to walk. Mild pancytopenia, liver injury, proteinuria, hypocomplementemia, and increased anti-nuclear antibody (ANA) were revealed at a hospital. Polymerase chain reaction detected HPV-B19 DNA in the serum. On the day 17th, she was admitted to our hospital because of moderate generalized weakness and mild sensory disturbance, which were symmetrical and distal-dominant. The deep tendon reflexes were absent. Analysis of cerebrospinal fluid showed albuminocytologic dissociation. Other laboratory data were normal except positive ANA. Enzyme-linked immunosorbent assay (ELISA) showed high titer of the serum IgM antibody to GM1 and GD1b. Serum anti HPV-B19 IgM and IgG tested by ELISA were also positive. She improved gradually after 2 courses of double filtrated plasma pheresis.

Two abnormal SOD1 mRNAs, exon 2-skipping and exon 2 and 3-skipping species, were identified from occipital brain tissue of sporadic amyotrophic lateral sclerosis (ALS) patients carrying no mutations in the SOD1 gene. Both transcripts were ubiquitously expressed in non-neuronal as well as neuronal tissues from a subject without neurological diseases. The expression pattern did not show disease specificity or lesional selectivity associated with ALS. Transient expression studies revealed weak expression of the proteins derived from the exon 2-skipping SOD1 cDNA in a cell-free translation system but not in cells. The putative abnormal SOD1 protein may accumulate and exert toxic effects on motor neurons in ALS when the proteolytic system is disturbed by aging or some causal factors.

In this study, we attempted to determine which patients with ureteral calculi are likely to have an unsuccessful outcome for extracorporeal shock wave lithotripsy (ESWL). The records of 388 patients with ureteral calculi treated with ESWL at Ijinkai Takeda General Hospital between January 1997 and December 1998 were retrospectively reviewed. Three hundred seventeen patients had been treated with ESWL alone (single session 156, multiple sessions 161) and 71 with transurethral ureterolithotripsy (TUL) due to failure of ESWL. We compared these two groups by multivariate logistic regression analysis, which revealed that (1) middle and lower ureteral calculi, (2) stones8 mm, (3) moderate to severe hydronephrosis and (4) failure of the first session ESWL were factors associated with poor outcome of ESWL. Although each predictive value of these factors was relatively low (29.6-38.6%), the rate of failure of ESWL was 85.0% when patients had all four factors. We therefore recommend an early change in treatment modality to TUL for patients with ureteral calculi when all four risk factors listed above are present.

It remains controversial whether the cure of H. pylori infection improves NUD symptoms.To conduct a double-blind placebo-controlled single centre study with concealed allocation to investigate this question.Ninety NUD patients with H. pylori infection were randomly assigned to either the treatment group (50 patients) or placebo group (40 patients). The treatment group received omeprazole, amoxycillin, clarithromycin and the placebo group received omeprazole and placebos for 7 days. Symptoms were assessed every week for up to 12 weeks after completion of medication by a symptom questionnaire. Alteration of histological parameters for gastritis was also evaluated.The infection was cured in 41 out of 48 patients in the treatment group and none in the placebo group. There was no significant difference in the mean symptom scores at any assessment point up to 12 weeks between the treatment and placebo groups. Regarding histological parameters, activity and inflammation, not atrophy or intestinal metaplasia, were significantly improved in the treatment group.Although histological parameters were significantly improved in the treatment group, there was no significant improvement in symptoms of NUD in the treatment group compared to placebo.

Angiographically occult vascular malformations (AOVMs) are reported. Although cavernous malformations, the most common type among the spinal intramedullary AOVMs, have been well documented, the natural history and clinical features of non-cavernous type AOVMs of the spinal cord remain obscure. We have evaluated four cases of non-cavernous AOVMs in the past 9 years. Although the clinical features and magnetic-resonance imaging (MRI) of three cases were similar to those previously reported, the other one had unusual features of insidious onset with limb asymmetry. The natural history of intramedullary occult AVMs remains enigmatic. One speculation for the neurological deterioration is that it results from repeated hemorrhages around the vessels or from intralumenal thrombosis.

Reliability and validity of a rating scale for post-stroke psychiatric symptoms were examined by the videotape method. The scale comprised 10 items categorized into two symptom domains of decreased spontaneity and impaired emotion. Also, two items for global assessment of the two symptom domains were added. Each item had seven anchor points from 0, representing no impairment, to 6, corresponding to complete impairment. Face validity of the scale was confirmed through the questionnaire survey. Nine neurologists independently assessed psychiatric symptoms in 30 videotaped post-stroke patients. Weighted kappa coefficients of more than 0.5 were noted for all the items except for one item. Data from three cerebral metabolism enhancers trials were used to examine the validity. Changes in severity in the Global Change Scale (GCS) from the baseline to the final assessment was assessed by raters' impression in these trials. Factorial validity of the scale was confirmed by the factor analysis. GCS in the three trials were considerably related to the summed scores of the items in the two categories. Namely, in the box plot figures, boxes of the middle 50% of data well differentiated the adjacent categories of GCS. However, overlap from vertical bars was observed. These results suggest reliability and validity of the scale.

Pigmentary macular dystrophy (PMD) was detected in a 57-year-old Japanese man with SCA1, five years after the onset of ataxia. Family history revealed 7 other ataxic members in three generations. Among them, only his younger brother, who had already died, had developed central scotoma with normal peripheral fields, 15 years after the ataxia had appeared. None of the other family members, with or without ataxia, had visual disturbances. The numbers of CAG repeats in the patient's SCA1 gene were 27 and 47, the latter of which was within pathological range and belonged to mild to moderate CAG expansion in previously reported cases. The PMD seen in our patient could be one of the clinical features associated with SCA1, since patients with SCA1 often show various ocular changes. Although genetic analysis ruled out SCA7 in this patient, the phenotypic resemblance of this patient to SCA7 may reflect the necessity in the future, for genetic differentiation between SCA1 and SCA7 in patients with autosomal dominant cerebellar ataxia and PMD.

A recent trend in curative therapy for Helicobacter pylori infection is the so-called triple therapy, which consists of a proton pump inhibitor (PPI) and two different antimicrobials. Various regimens employing this triple therapy have been reported. However, little is known about the effectiveness of rabeprazole, a recently developed proton pump inhibitor, when used in the triple therapy.To validate its usefulness by comparing rabeprazole with omeprazole and lansoprazole, in combination with amoxycillin and clarithromycin.221 H. pylori-positive patients with peptic ulcer disease were randomized to receive one of three different proton pump inhibitor/amoxycillin-clarithromycin (PPI/AC) regimens for 7 days. (i) OAC regimen (n = 75): omeprazole 20 mg b.d., amoxycillin (AMOX) 500 mg t.d.s. and clarithromycin (CAM) 200 mg b.d.; (ii) LAC regimen (n = 74): lansoprazole 30 mg b.d. , AMOX 500 mg t.d.s. and CAM 200 mg b.d.; and (iii) RAC regimen (n = 72): rabeprazole 20 mg b.d., AMOX 500 mg t.d.s. and CAM 200 mg b.d. Cure of the infection was determined by the 13C urea breath test 1 month after completion of the treatment.Intention-to-treat based cure rates for OAC, LAC and RAC regimens were 85% (95% CI, 75-92), 84% (95%, CI 73-91) and 88% (95% CI, 78-94), respectively, and per protocol based cure rates of these regimens were 88% (95% CI, 78-94), 91% (95%, CI 82-99) and 93% (95% CI, 84-98), respectively. Adverse effects in the entire study population, which included diarrhoea, glossitis or skin rash, were reported by 15% of the patients, and complete compliance was achieved in 95% of these patients.1-week proton pump inhibitor/AC regimens for H. pylori infection were effective in the Japanese population. Rabeprazole can be considered as equivalent to omeprazole and lansoprazole in the PPI/AC triple therapy.

The purpose of this study was to investigate the cortical reorganization associated with congenital brain lesion such as intracerebral arteriovenous malformation (AVM). Dipole source localization of somatosensory evoked potential (SEP) was performed in five patients with AVM encompassing sensorimotor cortex. Dipole tracing method combined with the scalp-skull-brain head model (Homma et al., 1994) was used to locate dipole source of an early cortical component of SEP elicited by median nerve electrical stimulation. The location of dipole source of SEP, which could be considered as the hand area of primary somatosensory area, was shown in the realistic section of the head and could be easily superimposed on the magnetic resonance imaging. SEP was recorded three times in each patients and the results were reproducible. In 2 patients whose postcentral gyrus was not involved in AVM, the dipole source of SEP was localized in the intact postcentral gyrus. The locations of dipole sources of SEP in the both hemisphere were symmetrical. In 3 patients whose postcentral gyrus was encompassed by AVM, the dipole source of SEP was localized in the surrounding intact gyrus which was distant from the usual region of postcentral gyrus. Somatotopy was different from the normal pattern. The hand area was located more medially than usual observed in normal postcentral gyrus. Despite the paucity of the number of patients and data obtained by dipole source localization, our findings support the existence of reorganization in the cerebral cortex with congenital lesion such as AVM. These findings of aberrant mapping of cortical function may be explained by the plasticity of brain function. The developing brain can inherit function that would normally have been performed by the region of brain involved in the lesion. We demonstrated that dipole tracing of SEP was a noninvasive method used to localize areas of eloquent cortex in patients harboring AVM. This method is of value in treatment planning.

We have developed a practical, low-cost ventricular assist device (VAD) comprising a newly designed blood chamber with a silicone lenticular sac and two silicone tri-leaflet valves (STV), made en bloc. This new VAD is seamless, can be made cost-effectively and assembled with the blood chamber and valve as one body. This novel design should reduce the incident of thrombus formation because of the absence of a junction at the connecting ring and because of the use of flexible silicone materials which have both antithrombogenecity and biocompatibility. In in vitro hemodynamics testing, a batch of 3 consecutively manufactured VADs with STVs underwent hydrodynamic functional testing. These showed less regurgitation, a lower value of water hammer phenomenon, and a slightly greater pressure gradient across the valves than a mechanical valve (MV) [Björk-Shiley monostrut valve]. The flow and pulsatile efficiency were adequate and similar to that of a VAD with MVs. In in vitro durability and hemolysis tests, a VAD with STV functioned well for 54 days and showed similar hemolytic profiles to a VAD with MVs. In an in vivo acute experiment using an adult sheep, our device was problem-free providing sufficient output as a left ventricular assist device (LVAD). Although it will be necessary to decrease the pressure gradient across this STV in the future, our device showed efficient performance as a practical land cost-effective VAD for short term use.

Iodine-123-labeled meta-iodobenzylguanidine ([123I]MIBG), an analogue of guanetidine, is used as a tracer for evaluation of the function of sympathetic neurons. To investigate cardiac sympathetic function in Machado-Joseph disease (MJD), [123I] MIBG myocardial scintigraphy was performed in 12 patients with MJD and 20 controls. In planar imaging studies, the heart to the mediastinum of the average count ratio (H/M) was calculated for both early and delayed images. The mean values of H/M in delayed images of MJD was lower than those of controls (p0.01), but this did not correlate with duration of the illness. The mean values of H/M in MJD patients with abnormal sympathetic skin response (SSR) was lower than that with normal SSR (p0.05). The decrease of H/M in delayed image is more sensitive than abnormal SSR. The increase of heart rate after standing in the patients with low H/M was lower than those with normal H/M. Abnormal cardiac sympathetic function revealed by [123I]MIBG myocardial scintigraphy in MJD can be seen earlier than abnormal sudomotor system detected by SSR.

Accumulating reports indicate a relationship between mitochondrial DNA mutation and impaired glucose-induced insulin secretion leading to a subtype of noninsulin-dependent diabetes mellitus. DNA from a 45-yr-old Japanese woman with noninsulin-dependent diabetes mellitus and muscle atrophy was isolated and studied for mitochondrial DNA mutations. We identified a mitochondrial DNA C-T heteroplasmic mutation at nucleotide position 3256. The mutation was located in the transfer ribonucleic acidLeu in a region conserved in evolution. Eight other members of her family were examined for the mutation. Six of them had the same mutation together with noninsulin-dependent diabetes mellitus, and one teenage boy had the mutation and impaired glucose tolerance. The other family member who did not have this mutation had normal glucose tolerance. The enzyme activity of the mitochondrial oxidative phosphorylation pathway in the muscle of the proband was measured. The enzyme activity was decreased in the proband, especially in complex I. This mutation might be responsible for the abnormal glucose metabolism.

We described a 41-year-old man with chorea-acanthocytosis, who presented severe choreoathetosis in all the limbs, neck, face and tongue. Especially, the oral area was affected most strikingly. He had difficulty in eating, because he pushed foods out of the mouth with the tongue. He received left posteroventral pallidotomy (PVP). His involuntary movements became much less enough for the patient to eat with a spoon in a sitting position by himself. Six months after the first operation, although involuntary movement on the right hand remained reduced, choreoballistic movement appeared on the left side. He received PVP on the right side. The choreoballistic movement was remarkably improved after the right PVP. No neurological aggravation has been found for 7 months after the second PVP. Bilateral PVP was fully effective as for the improvement of the activity of daily life. The output from the internal globus pallidus (GPi) is well-known to be inhibitory. Therefore, it is understandable that the coagulation of GPi results in improvement of hypokinesia and rigidity in patients with Parkinson's disease. However, some clinical reports, including ours, have described the significant reduction of hyperkinetic choreoballistic movement after PVP. The exact mechanism of these surgical effects has not been elucidated yet, and further clinical and basic studies are needed to answer this question.

Here we report 2 cases of opsoclonus-polymyoclonus syndrome (OPS) associated with viral encephalitis. They had sleep disturbance, and visual hallucination. Case 1 had auditory hallucination in addition, and case 2 had dreamlike behavior. Those hallucination, which were colorful and vivid, usually appeared at the bed time. Their hallucinations were similar to peduncular hallucinations and you may also call hypnagogic hallucinations, which are often seen in patients with narcolepsy. Dreamlike behavior is observed during REM sleep in patients with brainstem damage or sometimes in the healthy elderly people. The presence of sleep disturbance, hypnagogic hallucination, and dreamlike behavior suggests that there may be some relationship between OPS and REM sleep. Considering that REM sleep is suppressed by serotonergic projection of the dorsal raphe nucleus in addition to several reports about brainstem lesion with serotonergic abnormalities in this disorder, we considered that dysfunction of serotonergic neurons of the dorsal raphe nucleus might be related in the development of OPS.

Objective - To clarify how chronic solvent abuse affects cognitive function using magnetic resonance imaging as an index of brain damage. Material and methods - A total of 25 chronic solvent abusers underwent magnetic resonance imaging with quantitative neurometry and Wechsler Adult Intelligence Scales revised. Results - The abusers with white matter change (n=10) showed significantly (P < 0.05) lower performance IQ, especially in Digit Symbol subtest (P

We report a 29-year-old man with diabetes insipidus and cerebellar ataxia who developed spinal cord swelling 15 years after the onset. He was well until 14 years of the age when he noted dizziness. Two years after there was an onset of gait disturbance and slurred speech. He also noted polydipsia and polyuria. He was evaluated at the neurosurgery service of our hospital when he was 17 years of the age. Neurologic examination at that time revealed memory loss, horizontal nystagmus, cerebellar ataxic gait, dysmetria and decomposition more on the left. Cranial CT scan revealed a mass lesion involving the left subthalamic region and the head of the caudate area. Spinal fluid was unremarkable, however, human chorionic gonadotropin was increased to 27 mIU/ml. He was treated by radiation therapy (3,000 rads for total brain area and 5,460 rads for focal region). His CT scan and memory loss improved, however, cerebellar ataxia was unchanged. Three years after the radiation, he started to show choreic movement in his neck and left upper extremity. He was admitted to our service in August 14, 1995 when he was 29 years of the age. On admission, he was alert but disoriented to time; calculation was also poor. Higher cerebral functions were intact. The optic fundi were normal without papilledema. Visual field appeared intact. Gaze nystagmus was observed in all the directions, but more prominent in the horizontal direction. Speech was slurred. Otherwise, cranial nerves were unremarkable. Motor wise, he showed marked truncal and gait ataxia; he was unable to walk because of ataxia. Muscle atrophy and marked weakness was noted in both upper extremities more on the left side. Deep tendon reflexes were diminished in the upper extremities but active in the lower extremities. He was polyuric; urinary specific gravity was low. Spinal fluid contained 6 cells/cmm and 113 mg/ dl of protein; Queckenstedt was positive. MRI revealed swelling of the cervical cord; in addition, the entire cervical region and the medullar oblongata appeared as high signal intensity areas. No mass lesion was noted in the supratentorial structures but the third ventricle was markedly enlarged. Surgical biopsy was performed on the cervical lesion. The patient was discussed in neurologic CPC, and the chief discussant arrived at the conclusion that the patient had germinoma with syncytiotrophoblastic giant cells in the diencephalic region which appeared to have been cured by radiation therapy; he thought that the cervical lesion was the seeding of germinoma. Cerebellar ataxia was ascribed to the remote effect of germinoma. Most of the participants thought that the original tumor was germinoma and the cervical lesion was its spread. Some participants thought that his ataxia was caused by germinoma cells involving the medulla and the inferior cerebellar peduncles. Histologic observation of the biopsied tissue from the spinal cord revealed the typical two cell patterned germinoma. Most of the tumor cells were not stained for an antibody against HCG, but some tumor cells were positively stained. Germinoma is very radio-sensitive; this patient showed T2 high signal lesion involving the medulla oblongata and cervical cord continuously. Probably, tumor cells in the lower brain stem escaped radiation, and gradually spread to the spinal cord over many years. At the time of operation, the surface of the spinal cord was free from tumor cells. Therefore, tumor cells invaded the spinal cord continuously from the medulla oblongata. He was treated with cervical radiation, and his neurologic as well as radiologic findings showed marked improvement.

We described a 34-year-old man who developed opsoclonus-polymyoclonus syndrome (OPS) associated with benign encephalitis. His older sister also suffered from the same syndrome 12 years ago. We examined HLA types in 6 patients with OPS who were admitted to our hospital, including these sibling cases. All 6 patients have type A2 antigen and statistical analysis suggested that there is a positive relationship between OPS and HLA A2 antigen. Considering the two facts that the existence of the sibling cases in this relatively rare disorder and sharing of HLA A2 antigen in our 6 cases, some genetic factors might be involved in the development of OPS.

We report a 42-year-old woman who presented with intermittent episodes of muscular weakness lasting approximate 7 to 10 days, about once a year, since 1985. During these episodes of weakness, hypokalemia and elevation of serum creatine kinase were noted. On admission, xerostomia, keratoconjunctivitis sicca (positive Schirmer test), abnormal sialographic findings (apple tree-like appearance), and positive serum autoantibodies against ss-A and nucleus were noted. These findings were compatible with Sjögren syndrome. She also had hyperchloremic metabolic acidosis with normal anion gap, hypokalemia and renal calcifications. Therefore, she had renal tubular acidosis (type 1) in addition to Sjögren syndrome. Brain magnetic resonance imaging (MRI) demonstrated a symmetrical circumscribed lesion in the ventral part of the central pons. We considered this lesion to be central pontine myelinolysis (CPM), because of its typical location and characteristic MRI appearance. This CPM was clinically silent. Pathogenesis and etiology of CPM are obscure, and CPM associated with hypokalemia without hyponatremia has rarely been reported in the literature. However, this is the first report of CPM associated with Sjögren syndrome and hypokalemic myopathy complicated with renal tubular acidosis.

Rigid spine syndrome (RSS) is thought to be a group of heterogeneous muscle disorders. We reviewed the clinical and myopathologic findings of 55 patients with RSS including 49 previously reported and our own 6 patients. The results were as follows: the first, 80% showed restrictive ventilatory failure and 40% atrophy of the sternocleidomastoid muscle (SCM), which became manifest in the early stage of the illness, namely in less than 5 years from the onset. The second, paraspinal muscles usually began to be involved from the cervical semispinal muscle extending to other paraspinal muscles such as the more laterally located splenium muscle. The third, muscle biopsy demonstrated rimmed vacuoles (RV) in 41% of the 55 patients. RV were more frequently seen in the paraspinal muscles than the limb muscles, where marked myopathic changes of endomysial fibrosis and increased variation of the muscle fiber size were recognized. Based on the anatomical analysis of the paraspinal muscles, alligator sign, a radiological finding of the posterior opening between the cervical spine C1 and C2, seemed to result from the shortening of the cervical semispinal muscle due to its degeneration. This sign may, however, disappear as the whole paraspinal muscles including the semispinal muscles are affected. The RV seen in RSS seems to be a secondary, nonspecific structure related to the muscle degeneration. On the other hand, the above findings, namely the atrophy of SCM, restrictive ventilatory failure, and RV, have not been reported in Emery-Dreifuss muscular dystrophy (EDMD) so far. Accordingly, those findings and the absence of cardiac conduction abnormality are valuable for differential diagnosis of RSS from EDMD.

We reported a 31-year-old woman with epilepsia partialis continua (EPC) whose epileptic focus was delineated by PET. Neurologic examination revealed EPC in the face around the corner of her mouth, her thumb and forefinger on the left, slight exaggeration in the left biceps brachii reflex, and the digiti quiniti sign on the left. Laboratory tests showed pancytopenia, positive anti-nuclear and anti-DNA antibodies and decreased levels of serum complements, being consistent with systemic lupus erythematosus (SLE). Electroencephalogram showed paroxysmal discharges over the right frontoparietal region. PET with 2-18F-fluoro-2-deoxy-D glucose (FDG) clearly demonstrated a hypermetabolic area in the right frontoparietal cortex. T2-weighted images in MRI revealed reversible high signal intensity area in the right insula, postcentral gyrus and angular gyrus, while there were no abnormal findings in T1-weighted images with or without Gd-DTPA. There are only a few reports of EPC with the epileptic focus delineated by PET. Since a focal increase in glucose metabolism observed in them as well as in the present patient is a consistent indicator of the epileptic focus, the PET study is likely to provide significant information available not only for the diagnosis but also for the treatment of the condition. Finally, to our knowledge, this is the first patient with SLE presenting EPC. It is important to realize that vasculopathy associated with SLE may cause EPC and brain lesions with reversible high signal intensities shown by MRI.

Despite the increasing use of alternative techniques, endomyocardial biopsy remains the primary method for diagnosing cardiac allograft rejection. Between March 1986 and May 1994, 2,894 endomyocardial biopsies performed on 183 heart transplant patients were reviewed. A total of 53 (1.8%) complications occurred. 33 (1.1%) complications were associated with the introduction, including carotid puncture (0.9%), neurological reaction (0.1%), and pneumothorax (0.1%). Complications during biopsy included arrhythmias (0.4%) and ventricular perforation (0.2%). In addition, we observed three episodes of allergic reaction to a reusable biotome, three episodes of liver biopsy, and one case of pacemaker dislodgement. All complications were without significant long-term sequelae. In contrast to the cardiomyopathy population, no severe ventricular perforations or deaths occurred. Thus although endomyocardial biopsy has some risk, it continues to be a safe and effective way of monitoring rejection.

In order to study the cerebral circulation and metabolism in 6 patients with mitochondrial myopathies and 6 normal control subjects, positron emission tomography with oxygen-15 labelled tracers was performed. Four patients had predominantly central nervous system involvement, whereas 2 had myopathy alone. In comparison with normal control subjects, patients with mitochondrial myopathies showed decreased cerebral blood flow (CBF), decreased cerebral oxygen metabolic rate (CMRO2), and low oxygen extraction fraction (OEF) value due to an uncoupling of CBF and CMRO2. The reduction of OEF was remarkable in the 2 patients having predominantly central nervous disease and 1 having myopathy alone. We suppose that these findings are greatly related to mitochondrial myopathies and that they reflect the disturbance of mitochondrial oxidative metabolism in the brain. The patients whose central nervous symptoms progressed rapidly tended to show the reduction of OEF, but the degree of the reduction of OEF was not necessarily related to the severity of the central nervous symptoms. These findings suggest that the disturbance of cerebral metabolism is not uniform in patients with the same central nervous disease.

We studied cerebral blood flow, oxygen metabolism and their relation to clinical symptoms in 45 patients with spinocerebellar degeneration (SCD) and 12 normal control subjects using positron emission tomography (PET). Regions of interest were acquired for the cerebellar hemispheres, cerebellar vermis, brainstem, thalami, and cerebral cortices. PET studies in these patients revealed that regional cerebral blood flow (CBF), regional cerebral oxygen metabolic rate (CMRO2), CBF/mean CBF of each cerebral cortex (CBF/mCBF) and CMRO2/mean CMRO2 of each cerebral cortex (CMRO2/mCMRO2) in the cerebellar hemispheres, cerebellar vermis, and brainstem showed a significant decrease in comparison with the normal control subjects, while in the cerebral cortices and thalami, SCD patients showed normal values. CBF/mCBF and CMRO2/mCMRO2 were significantly decreased in patients with olivopontocerebellar atrophy (OPCA) and Menzel type of hereditary ataxia (Menzel type) in the cerebellar hemispheres, cerebellar vermis, and brainstem, whereas patients with late cortical cerebellar atrophy (LCCA) and Holmes type of hereditary ataxia (Holmes type) revealed a significant decrease of CBF/mCBF and CMRO2/mCMRO2 in the cerebellar hemispheres and cerebellar vermis, but not in the brainstem. Patients with OPCA showed a significant decrease of CBF in the cerebellar hemispheres, cerebellar vermis, brainstem and that of CMRO2 in the cerebellar hemispheres and cerebellar vermis. Patients with LCCA showed a significant decrease of CBF in the right cerebellar hemisphere and cerebellar vermis. However, a significant reduction of CBF and CMRO2 was not observed in patients with Menzel type or Holmes type in these regions. In patients with LCCA and Holmes type, the severity of upper limb ataxia and dysdiadochokinesis were significantly correlated with CBF/mCBF and CMRO2/mCMRO2 in the cerebellar hemispheres and brainstem.(ABSTRACT TRUNCATED AT 250 WORDS)

Blood Group related antigens Lewis A, Lewis B, CA 19-9, and sialyl SSEA-1 were examined in normal human skin and oral mucosa, using monoclonal antibodies to the respective antigen immunohistochemically. These antigens were not expressed on normal keratinocytes but Lewis A and Lewis B were expressed on oral mucous epithelium. Lewis A, Lewis B, and CA 19-9 were expressed clearly on intraepidermal and dermal eccrine duct, but sialyl SSEA-1 was poorly expressed in some cases. In secreting portion of eccrine gland, Lewis A was expressed in 20%, Lewis B was expressed in 70%, and CA 19-9 was expressed in 10%, and sialyl SSEA-1 was expressed weakly in 20% of cases. It's noteworthy that sialyl SSEA-1 was expressed on dendritic cell in epidermis and some infiltrated small round cell in dermis. There is no correlation between patient's peripheral blood type (Lewis) and expression of Lewis antigen in normal skin.

Legionnaires' disease in a 37-year-old male who had had silicosis was reported. He was admitted because of dyspnea. The chest X-ray film and CT scan showed infiltrative shadow and swelling of mediastinal lymph nodes. Open lung biopsy was done and Legionella pneumohila was detected. REP and EM were started and infiltrative shadow of X-ray was disappeared. Pleuro-pneumonectomy and thoracoplasty were performed because of hemoptysis and postoperative empyema. The patient is now well.

An adult female showed mild cerebellar ataxia and CSF pleocytosis following an acute infection of the upper respiratory tract, and was diagnosed as having acute cerebellar ataxia (ACA). CT and MR appearances in the acute stage revealed moderate swelling of the cerebellum and bilaterally increased signal intensity in the cerebellar cortex.

Left pneumonectomy with combined resection of aortic wall was performed for three patients with lung cancer invaded aorta. In clinical and pathological examination, the following results were obtained. 1: In all three cases, metastasized subaortic lymphnodes invaded to medial wall of the descending aorta. 2: Microscopically, invasion of aortic wall was limited to the adventitia adjacent to the muscle layer. We think that the resection of whole layer of the aortic wall was preferable. 3: The resections of aortic wall were performed under partial clamp of aorta in one case, and under total clamp with the use of cardiopulmonary bypass in two cases. If the area of invasion is wide, total clamp was recommended. 4: Two patients died of recurrent disease 56 months and 20 months after surgery. In the other one, postoperative empyema was occurred. He died of bleeding from the suture line of Dacron patch to the aortic wall three months postoperatively. Covering of the bronchial stump and the patch of the aorta should be considered to prevent postoperative empyema.

A 25-year-old female with McArdle's disease was reported. She had no characteristic symptoms for McArdle's disease such as muscle cramp and brown urine, but had general fatiguability from childhood. On examination, she showed no neurological abnormalities including muscle atrophy and weakness. On laboratory examination, serum creatinine kinase (CK) level was elevated, though serum lactic acid level remained unchanged after the ischemic forearm exercise test. Muscle biopsy from the biceps brachii showed almost completely absent phosphorylase activity both histochemically and biochemically. Thus, she was diagnosed as having McArdle's disease. The skinned fiber test of the muscle showed no enhanced Ca induced Ca release (CICR), and serum VLDL level was normal. Her 27-year-old elder brother had similar clinical symptoms and serological abnormalities and may also have McArdle's disease, although muscle biopsy was not performed. A possibility of McArdle's disease should be considered when we encounter a patient who has only general fatigue and high serum CK level.

Seventeen primary and sixteen metastatic surgically removed melanoma lesions were stained indirect immunoperoxidase with anti-GD3, GD2, GD2 + GD3, and acetyl-GD3 monoclonal antibodies. In primary melanoma 94%, 59%, 53%, and 76% of the lesions tested were evidently stained by anti-GD3, GD2, GD2 + GD3, and acetyl-GD3 monoclonal antibodies, respectively. In metastatic lesions 94%, 63%, 31%, and 31% of the lesions were stained by monoclonal antibodies, respectively. Expression of GD2 + GD3 and acetyl-GD3 were evidently decreased from primary lesion to metastatic lesions. And the heterogeneity of positive cells in lesions were noteworthy. It is also interesting that expression of ganglioside in ALM were much decreased than that of ganglioside in NM.

We quantitatively evaluated the cerebellar functions of 23 patients with spinocerebellar degeneration (SCD) and 10 normal controls using positron emission tomography (PET). Statistical analysis was performed using the Student's test. Regional cerebral blood flow (CBF) and regional cerebral metabolic rate (CMR) of the cerebellar hemisphere and cerebellar vermis were significantly lower in patients with SCD than in normal subjects (p less than 0.001). However, no significant difference between the both groups was seen in the CBFs and the CMRs of the occipital cortex and frontal cortex. Even in the patients with SCD who had not apparent cerebellar atrophy on CT, their CBFs and CMRs of the cerebellum were significantly low, and with advance of cerebellar atrophy, they tended to fall. In the patients with SCD, the fall of CMR was more prominent than that of CBF. Neither CBF, nor CMR of the cerebellum showed correlation to the duration of the illness. The present investigation suggested that PETs were valuable for the early diagnosis and the research on the pathogenesis of SCD.