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Background: Magnetic resonance imaging (MRI) is useful for demonstrating demyelinating lesions in patients with multiple sclerosis (MS). Magnetic resonance imaging studies show that MS lesions are generally not uniform in shape, size, or distribution. Linearly shaped lesions at the trigeminal root entry zone have been occasionally reported in single cases of MS, but, to our knowlege, the frequency and the clinical features of such patients have not been comprehensively characterized., Objective: To describe the frequency and the clinical and laboratory features of patients with MS who had linearly shaped lesions at the trigeminal root as seen on MRI., Design and Setting: A retrospective review of medical records and MRI films of Japanese patients with MS admitted to a university hospital and its affiliated hospital in Sendai, Japan., Patients and Methods: Brain MRI films of 74 consecutive Japanese patients with MS (51 females and 23 males) were studied retrospectively and the clinical and laboratory features of the patients with linearly shaped lesions at the trigeminal root were also investigated retrospectively., Results: Five patients (6.8%) were shown to have T1-weighted-hypointense, T2-weighted-hyperintense, nonenhanced linear lesions in the pons on MRI, and these were uniformly localized in the intramedullary portion of the trigeminal root. All of these patients had clinically definite MS and had various types of facial sensory disturbances, such as neuralgia (1 patient), hypesthesia (2 patients), or paresthesia (3 patients). No other clinical or laboratory feature was characteristic in these 5 patients., Conclusions: Linear pontine trigeminal root lesions were common in our patients with MS. They were associated with various facial sensory symptoms. Since similar lesions are formed in animal models of herpes simplex virus infection, further study is needed to clarify whether these MS lesions are virally induced.

A 68-year-old man was hospitalized on March 4, 1998 for disturbances in consciousness. In 1995, he had received proximal subtotal gastrectomy and reconstructive surgery of the jejunal interposition for gastric cancer. Thereafter he had been taking enough food without the habit of taking liquor. In October 1997, his short term memory was becoming gradually worse. On February 12, 1998, he suffered from numbness in the feet, and then dysphagia, unsteady gait, and diplopia developed gradually. On February 26, brain MRI showed no abnormalities. On March 3, he had a fever of 38.5 degrees C and his consciousness became unclear. Neurological examination revealed semi-coma, total ophthalmoplegia, and absence of doll's eye movement. Deep tendon reflexes were absent. The serum thiamine level was 9 ng/ml (normal range: 20-50). Brain MRI demonstrated symmetrical high intensity lesions in the periaqueductal area of the midbrain, dorsomedial nuclei of bilateral thalami, and vestibular nuclei. About 30 seconds after intravenous infusion of thiamine, his consciousness improved dramatically, but returned to semi-coma after about two minutes. Wernicke-Korsakoff syndrome usually occurs acutely. In the present case, however, the disease showed slow onset, chronic progression, and then rapid worsening after fever. Reconstructive surgery of the jejunal interposition might have caused the slow onset of Wernicke-Korsakoff syndrome, and fever might have facilitated the rapid progression of the disease. An immediate high concentration of thiamine modifies the kinetics of acetylcholine receptor ion channels, thereby maintaining wakefulness, and the level of consciousness may change dramatically.

An autopsy case of MND with dementia, 67 years old, man, was reported. The behavioral disturbance appeared at 61 years old, and was followed by the upper limb weakness and respiratory failure. He had remained on a respirator for 56 months. The total duration of the disease was 74 months, which was far longer than mean duration of the cases previously reported, 25-30 months. The pathological findings were about essentially identical to those mentioned before, such as cortical alterations in temporal and frontal lobes, neuronal loss with gliosis in amygdaloid body and substantia nigra, and loss of lower motoneurons in anterior horn of spinal cord and in the several motor cranial nerve nuclei. There were several Bunina bodies in the remnant lower motoneurons, but no changes in the pyramidal tracts or loss of Betz cells in the precentral gyrus. Intracytoplasmic ubiquitin-immunopositive inclusions in the granule cells of hippocampal dentate gyrus were also encountered, but the number of which did not increased, compared to the preceding data. There may be frequently little correlation between the disease duration and the number of such inclusions. The regional alteration in the intermediate zone between subiculum and entorhinal cortex was revealed also at the level of the splenium of corpus callosum. It has been reported that such parahippocampal lesion may initiate at the anterior part of temporal lobe and become undiscernible at more posterior level than the lateral geniculate body. The formation of such parahippocampal lesion may be concerning with the length of disease duration. In addition, there were numerous senile plaques, diffuse or neuritic type only in the parietal lobe cortex without any other changes.

The patient, a 65-year-old woman, had liver cirrhosis, and had blood transfusion at the age of 49 and 56. Early in September, 1989, she gradually developed numbness of the legs, staggering gait, and apathy with hallucination. In October, she became incontinent and unable to stand, and was admitted to Konan Hospital. On admission, she was disoriented with poor comprehension. Cranial nerves were intact except for horizontal nystagmus on lateral gaze. She had generalized areflexia without pathological reflex. Muscular forces were fairly preserved. Superficial sensations were diminished in the upper limbs as well as below Th-7 level. Deep sensation was abolished in the distal parts of the extremities with athetotic finger postures on arm rising. She had urinary and fecal incontinence. Results of routine laboratory examinations were non-contributory. Chest CT scan and sputum cytology were normal. CSF contained one cell/microliter, 95 mg/dl of protein with positive oligoclonal IgG bands. Anti-HTLV-I antibody was positive in serum and CSF. Urodynamic studies showed neurogenic bladder of supranuclear type. MNCV was slightly decreased. SNAP and SEP were not evoked. On sural nerve biopsy, the density of myelinated fibers was 720/mm2, and that of unmyelinated fibers, 26,978/mm2. ABR and VEP were abnormal. EEG showed diffuse theta waves with paroxysmal delta and sharp waves. T2-weighted MR images of the brain showed patchy areas of high signal intensity in the cerebral white matter. Soon after administration of methylprednisolone, her consciousness became clear. EEG normalized in 4 months. Twenty months after the onset, she became ambulant with crutch, but still has dysuria and sensory deficits in the hands and lower limbs. The possible relationship between encephalomyeloneuritis and HTLV-I infection was discussed.

We here present a case of 44-year-old woman, a carrier of human T-lymphotrophic virus type-1 (HTLV-1), who suffered from limbic encephalitis and breast cancer. In December 1997, the patient's behavior became abnormal. Three weeks later, she became markedly forgetful. At that time neurological examinations revealed that she had anterograde and retrograde amnesia, disorientation, and confabulation, although her consciousness was clear. Anti-Hu and anti-Yo antibodies and antinuclear antibodies in the serum were negative. Flow cytometric study of the peripheral blood lymphocytes showed an increased percentage of CD3+CD25+ cells, although the percentages of CD4+CD45RA+ and CD4+CD45RO+ cells were normal. Lymphocytic responses to phytohemagglutinin or concanavalin A were normal. Anti-HTLV-1 antibody was positive both in the serum and in the cerebrospinal fluid (CSF). The level of immunoglobulin G was high and two oligoclonal immunoglobulin G bands were positive in the CSF. Cytological study of the CSF showed no atypical cells. Findings for herpes simplex virus type I and II DNAs were negative with polymerase chain reaction in the CSF. There was no elevation of antibody titers against viruses including herpes simplex virus, cytomegalovirus, and measles virus, either in the serum or the CSF. Magnetic resonance imaging showed signal abnormalities in the medial portions of both temporal lobes, in particular, in the bilateral hippocampus. Six weeks after the onset, a cancerous tumor in her right breast was detected and removed by open surgery. The pathological diagnosis was invasive ductal carcinoma with neuroendocrine features. After mastectomy, anterograde and retrograde amnesia and disorientation mildly improved. The follow-up magnetic resonance imaging showed that signal abnormalities in the medial portions of both temporal lobes decreased and that the bilateral hippocampus became atrophic. We diagnosed the present case as paraneoplastic limbic encephalitis. There has been only one case report of limbic encephalitis associated with breast cancer.

Polychlorinated biphenyl (PCB) tolerant derivatives of a strong PCB degrader, Rhodococcus strain RHA1, were selected after growth in the presence of 100 micrograms/ml PCBs. Some of the derivatives did not grow on biphenyl but accumulated a yellow coloured metabolite suggesting a defect in the meta-ring-cleavage compound hydrolase step encoded by the bphD gene. Other derivatives failed to grow on biphenyl and exhibited little PCB transformation activity suggesting a defect in the initial ring-hydroxylation dioxygenase step encoded by the bphA gene. These organisms had a structural alteration in the linear plasmids coding for the bph genes in RHA1, which included the bph gene deletion. When a bphD containing plasmid was introduced into a tolerant derivative, RCD1, which was shown to have bphD deletion, the defect in the growth on biphenyl of RCD1 was overcome. The bph gene deletion seems to play a key role in these tolerant derivatives thereby suggesting that the toxic metabolic intermediate would be a main cause of the growth inhibition of RHA1 in the presence of high concentration PCBs.

Arterial thrombi are primarily composed of platelets. Platelets are bound to injured endothelial cells, sub-endothelial matrices, and other platelets by a range of adhesive proteins. Some of these reactions are governed by shear forces. The role of adhesive proteins in the pathogenesis of arterial thrombosis is not fully understood. The aim of this study was to examine the involvement of von Willebrand factor (vWF), fibrinogen (Fg), and fibronectin (FN) in the formation of microvascular thrombi in vivo using a helium-neon laser-induced thrombosis method. Transmission electron microscopy demonstrated that laser irradiation resulted in platelet-rich thrombosis in arterioles and venules, and revealed that this occurred in the absence of endothelial denudation. The mean wall shear rates in mesenteric arterioles and venules were 641 +/- 40 and 280 +/- 20 s-1, respectively. Shear rates increased approximately fivefold in arterioles and tenfold in venules during the formation of occlusive thrombi. Antibody to vWF inhibited thrombosis in arterioles and venules. Antibodies to Fg and FN inhibited thrombosis in venules but not in arterioles. These results confirm that vWF, Fg and FN were involved in thrombogenesis in vivo and demonstrated that significantly higher shear rates were required for the reactions involving vWF than those involving either Fg or FN.

Oxidative stress has been suggested to be involved in the pathogenesis of neurodegenerative diseases, such as Alzheimer disease (AD) and Parkinson disease (PD). Heme oxygenase-1 (HO-1), a key enzyme in heme catabolism, also functions as an antioxidant enzyme. Here, we show that a (GT)n repeat in the human HO-1 gene promoter region is highly polymorphic, although no particular alleles are associated with AD or PD. This newly identified genetic marker should allow us to study the possible involvement of HO-1 in certain human diseases.

The beneficial effect of plasma exchange, plasmapheresis or immunoadsorption therapy on Fisher's syndrome, suggested previously, has not been proved since no controlled studies have been conducted. In order to assess the effect of any treatment on Fisher's syndrome, simple and reasonable grading scales for evaluating the major neurological signs are needed. We tried immunoadsorption therapy in four patients with Fisher's syndrome, whose sera had anti-GQ1b antibodies. The clinical course was observed, with assessment of the severity of the major neurological signs based on grading scores; ranging from 0 to 30 for external ophthalmoplegia, from 0 to 10 for ataxia, and from 0 to 16 for areflexia. Tryptophan- or phenylalanine-linked polyvinyl alcohol gel column (TR-350, PH-350) was used as an adsorbent. In a patient who had IgG anti-GQ1b antibody and another patient who had both IgG and IgM anti-GQ1b antibodies, we compared the effectiveness of TR-350 and PH-350 to remove the anti-GQ1b antibody during the therapy. Two patients underwent immunoadsorption therapy at the height of clinical manifestations: in one patient, the therapy was discontinued because of critical hypotension and arrhythmia; the other was given only three sessions of therapy. The other two patients received six or seven sessions during the early recovering stage. All patients recovered without major neurological sequelae. Since ataxia was improved earlier than external ophthalmoplegia, the duration of hospitalization and the time of return to social life depended upon the recovery of external ophthalmoplegia. Analysis of the time course of external ophthalmoplegia score indicated that the improving period and the 50%-recovery day came earlier in the patients who were given a sufficient number of sessions than those who received an insufficient number of sessions. The treatment with TR-350 reduced the IgG anti-GQ1b antibody titer more than that with PH-350, but reduced the IgM anti-GQ1b antibody titer similarly. Immunoadsorption therapy using TR-350 has a probable beneficial effect on Fisher's syndrome even though it is carried out after the height of illness. The evaluation method for the severity of external ophthalmoplegia that we used in the present study is useful for assessing the effect of therapy on Fisher's syndrome.

We reported the first case of chorea-acanthocytosis associated with low glycohemoglobin A1c. Fasting blood sugar, daily profile of blood sugar, serum fructosamine and 75 g glucose tolerance test were all within normal limits. While abnormal hemoglobins were not detected, red blood cell half-life was sharply reduced to 13.4 days. These findings suggest that the low glycohemoglobin A1c in this case is highly ascribable to the reduction of red blood cell half-life rather than to continuous hypoglycemia. However, we can not rule out that the abnormalities of red blood cell membrane, suggested factors in this disease, may be related to the low glycohemoglobin A1c value. Further investigations on chorea-acanthocytosis cases with normal red blood cell half-life are necessary in order to elucidate whether an abnormal glycohemoglobin A1c value is a specific indication regarding this disease.

The cytotoxicity of four commercial tissue conditioners was evaluated by the agar overlay method. Every material either fresh or stored for 1 hour, 24 hours, 8 days, or 15 days, showed some degree of cytotoxicity. Coe Comfort and Kerr FITT materials exhibited marked cytolysis, whereas GC Soft-Liner and Visco-gel materials caused a decolorized zone with minimal cell damage. Based on the sum of reaction indices, the tissue conditioners tested seemed to be more cytotoxic than autopolymerizing denture base resins. Further analyses are needed to identify the cytotoxic components that continued to leach out from the tissue conditioners for at least 15 days.

The patient was a 61-year-old female who suffered from the sudden attack of a speech disorder. The episodes of speech disorder lasted for less than 1 min, but occurred several times a day, paroxysmally. A CT revealed a low density area on the surface of the left frontal lobe and an EEG showed an abnormal wave at the frontoparietal area, more dominantly on the left. This condition was diagnosed as a kind of epilepsy. The paroxysmal speech disorder occurred very often during the 2 months after the first episode. However, with therapy using anticonvulsives and antibiotics, the condition began to improve after 4 months and disappeared completely after 6 months. It is assumed that the inflammatory change which occurred at the frontal lobe stimulated the supplementary motor area paroxysmally, and subsequently the speech disorder occurred as a kind of epilepsy.

A 51-year-old Japanese woman with hypokalemia due to distal renal tubular acidosis associated with Sjögren's syndrome exhibited a decreased plasma aldosterone level despite elevated plasma renin activity. Our studies revealed selective hypoaldosteronism with normal adrenoglucocorticoid function. In the presence of a low level of serum potassium (3.6 mEq/l), plasma levels of deoxycorticosterone and corticosterone were normal, while plasma aldosterone was very low. The levels of these three mineralocorticoids showed only minor changes during infusion of angiotensin II. Furosemide administration under almost the same level of serum potassium (3.7 mEq/l) resulted in only a slight increase of plasma aldosterone. Since hypokalemia might possibly suppress the synthesis of aldosterone in the zona glomerulosa, angiotensin II was also infused under a normal level of potassium (4.3 mEq/l). However, angiotensin II also failed to stimulate any secretion of aldosterone, despite a progressive rise in blood pressure and sufficient suppression of plasma renin activity. On the other hand, rapid ACTH administration in the presence of 4.4 mEq/l of serum potassium increased both plasma aldosterone and cortisol. These results suggest that adrenal insensitivity to angiotensin II was the cause of the selective hypoaldosteronism in our patient, possibly due to a dysfunction of adrenal angiotensin II receptors, a disorder of postreceptors or both.

A patient with familial idiopathic hypoparathyroidism whose major symptoms were paroxysmal kinesigenic choreoathetosis (PKC) and paroxysmal dystonic choreoathetosis (PDC) was reported. CT revealed marked calcification in the basal ganglia, the thalamus and the dentate nucleus of cerebellum. Positron emission tomographic (PET) study showed decreased glucose metabolism in the basal ganglia and thalamus. The paroxysms were eliminated by the normalization of serum calcium level. Thus the paroxysms seemed to be induced by hypocalcemia which stimulated the lesions in the basal ganglia and thalamus, on one occasion, as in PKC, and on the other occasion, as in PDC. Concomitant occurrence of both PKC and PDC in a patient is unique and is of interest in considering the pathomechanisms of these different but related disorders.

CT scan is able to show cerebral atrophy more safely and more easily than pneumo-encephalography or cerebral angiography. Then, various methods have been reported for quantitative analysis of cerebral atrophy on CT scan. Generally, cerebral atrophy might be judged from the ventricular dilatation with some indices, calculated from various ventricular width. But, there is no general agreement on what index is the most reliable. In this paper, we attempted to establish the index, easy to measure and most reliable. Our method is as follow. Method. We carried out the CT scan (EMI 1010) on 89 neurologically intact patients. Scans were parallel to orbito-meatal line (OML), and were 10 mm in thickness. On CT scan films, various width, area of anterior horns and area of bodies of lateral ventricles were measured (Fig. 1). Measurement about the anterior horns of lateral ventricles were carried out on image the most clearly showed the foramen of Monro. And measurements about the bodies of lateral ventricles were on image, 20 mm above the image of anterior horn. Correlations of various width and areas were calculated (Table 1). Then we proposed new indices with high correlations (over 0.9) with ventricular area; Anterior horn CVI (Cerebro-Ventricular Index) and Body CVI (Fig. 2, 3). Patients with myotonic dystrophy show cerebral atrophy. We carried out the CT scan (GE-CT/T 8800) on 17 myotonic dystrophy patients and 30 controls. Between the two groups, age and sex were almost matched (Fig. 4).(ABSTRACT TRUNCATED AT 250 WORDS)

The allo-suppressor effect of normal T lymphocytes on the production of migration inhibition factor by sensitized T lymphocytes of Graves' disease in response to human thyroid antigen has been studied further by a modified migration inhibition factor test employing purified T lymphocyte preparations. The production of migration inhibition factor was consistently abolished when normal T lymphocytes were mixed with the Graves' disease lymphocytes in various ratios (1:9, 2:8, and 5:5). However, pretreatment of the normal T lymphocytes with cimetidine (an H-2 histamine receptor antagonist) led to a demonstrable loss in their allo-suppressor properties, whereas pretreatment with chlorpheniramine (an H-1 histamine receptor antagonist) had no such effect. These studies indicate that a subset of normal T lymphocytes bearing H-2 histamine receptors suppresses the production or release of migration inhibition factor by sensitized T lymphocytes, and further suggest the possibility that there may be an abnormality in the H-2 receptors on Graves' disease suppressor T lymphocytes. It is conceivable that this defect is fundamental in the pathogenesis of Graves' disease.

T-Lymphocyte sensitization in Graves' disease (GD) and Hashimoto's thyroiditis (HT) was studied by an indirect migration inhibition factor test using normal T-lymphocytes as second stage indicator cells. In the first stage, mononuclear cells or T-lymphocytes, fractionated by the standard Ficoll-Hypaque procedure from the blood of patients with untreated GD and HT, were cultured in Eagle's medium containing thyroid antigen, and their cell-free supernatants were saved. Normal T-lymphocytes as second stage indicator cells were packed in capillary tubes and placed in planchettes with the above supernatants to complete the indirect migration inhibition factor test. Inhibition of the migration of indicator T-lymphocytes was demonstrated when either GD or HT culture supernatants were employed. Moreover, there was a good correlation between the indirect using the culture supernatants and the direct migration inhibition factor test using mononuclear cells or T-lymphocytes. On the other hand, in both direct and indirect migration inhibition factor tests using mononuclear cells and mononuclear cell culture supernatants, respectively, in the presence of human liver antigen as a nonspecific antigen, there was no significant difference between controls and patients. From these results, we can conclude that GD and HT T-lymphocytes are sensitized to thyroid antigen and produce the lymphokine, migration inhibition factor, into the supernatant when exposed to this antigen.

We studied endocrine functions of the hypothalamus, pituitary, thyroid, gonad, adrenal and pancreas in 26 patients with myotonic dystrophy. The following findings were obtained: (1) Hyporesponsivenesses of HGH to insulin, arginine and L-dopa test were observed in 25%, 45% and 54.5% of patients, respectively. To the administration of sulpiride and TRH, hypo- or hyperresponses of plasma PRL were observed in 4 or 2 patients, respectively. (2) Depressed plasma levels of testosterone were observed in 4 patients and those of estradiol were observed in 3 patients. Among these 7 patients, five were hypergonadotropic and the remaining two were hypogonadotropic. From the data of plasma cortisol and urinary 17-OHCS, the adrenal functions in patients with myotonic dystrophy were considered to be normal. The renin-angiotensin-aldosterone systems in these patients were considered to be grossly normal. (3) Basal levels of T3, T4 and free T4 were within the normal ranges, but 6 of 19 patients showed low levels of 131I-uptake and 11 of 24 patients showed reduced BMR values. (4) The basal level of plasma insulin was elevated in 5 patients. Insulin responses to oral glucose were exaggerated and delayed in 21 of 26 patients. From these results and the statistical evaluation of each laboratory and clinical data, we concluded that 1) the degrees of disturbances of endocrine functions are at random in each patient. 2) The measures of intellectual function obtained by WAIS were directly correlated with basal levels of TSH, but inversely with basal levels of somatostatin. No specific correlation was found between endocrinological disturbances and neurological severities. 3) Impairment of some endocrine functions, such as the gonadal, thyroid and somatotroph functions, was closely correlated with age and the duration of disease.

A 61 year old male with left main coronary artery disease died suddenly during ambulatory Holter monitoring. The monitoring documented ST depression followed by ST elevation, followed by junctional and ventricular escape rhythms with no measurable blood pressure by cuff methods. The mechanism of sudden death in patients with left main coronary artery disease is thought to be a combination of ventricular fibrillation or bradyarrhythmia and electromechanical dissociation secondary to a sudden onset of severe pump failure.

Clinical features such as types of diseases, sex ratio, age of onset, sites of initial involvement, the appearance of bulbar signs, and duration of illness were studied in 52 patients with motor neuron disease (MND) with a special reference to immunoglobulin in cerebrospinal fluid (CSF) and serum. Although MND has been thought to be a degenerative disease of unknown cause, our data suggested there are some immunological abnormalities in this disease. The duration of illness and the abnormalities of CSF immunoglobulins appeared to be correlated with the type of disease and the site of initial involvement. However, whether or not these abnormalities in CSF immunoglobulins are directly related to the pathogenesis of MND remains unclear.

The involvement of cell-mediated immunity in the pathogenesis of Graves' disease (GD) and Hashimoto's thyroiditis (HT) was investigated by employing a modified migration inhibition factor test using preparations of isolated T-lymphocytes. The migration of T-lymphocytes from patients with GD and HT in response to crude human thyroid antigen was significantly inhibited compared to the migration of T-lymphocytes from normal subjects. This response was organ specific. When normal T-lymphocytes were mixed with GD or HT T-lymphocytes in ratios varying from 1:9 to 1:1, the migration inhibition activity of the GD or HT T-lymphocytes in response to thyroid antigen was abolished, but was not abolished when two different GD or HT T-lymphocyte preparations were mixed. Mitomycin C inhibited this suppressive effect of normal T-lymphocytes in vitro, but did not influence the migration inhibition activity of the antigen-sensitized GD or HT T-lymphocytes. On the other hand, the migration inhibition of GD and HT T-lymphocytes was prevented by puromycin. There thus appears to be activity in normal T-lymphocytes which can suppress the ability of GD and HT T-lymphocytes to respond to the thyroid antigen, which is lacking in the GD and HT T-lymphocytes themselves. Our results are consistent with the hypothesis that there is a defect in suppressor T-lymphocyte function in GD and HT.