Motor neuron disorder simulating ALS induced by chronic inhalation of pyrethroid insecticides.
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Adult↗
Biomedical subjects
H Murai
Publications and source records attributed to H Murai.
Infantile onset myasthenia gravis with MuSK antibodies.
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Adult↗
[Intralobar pulmonary sequestration with high level of serum CEA; report of a case].
We reported a case of intralobar pulmonary sequestration with a high level of the serum CEA. A 53-year-old woman whose chief complaint was cough was admitted to our hospital. Enhanced chest computed tomography (CT) revealed the mass in the left lower lung, lymph-nodes swelling, and the aberrant artery. Magnetic resonance angiography (MRA) conformed the aberrant artery from the descending aorta. The level of serum CEA elevated at 9.6 ng/ml. Left lower lobectomy was performed. A diagnosis of intralobar pulmonary sequestration (Pryce type II) was established in this case. Histopathologically, the peribronchial epithelial cells in pulmonary sequestration showed weak positive for anti-CEA monoclonal antibody. Postoperative course was uneventful and the serum CEA level was 3.5 ng/ml in the normal range at the postoperative 17th day.
Bronchopulmonary Sequestration↗
Infrasternal mediastinoscopic surgery for anterior mediastinal masses.
BACKGROUND: Infrasternal mediastinoscopic surgery is a new alternative to the thoracoscopic approach for patients with anterior mediastinal masses. METHODS: We applied this technique to 18 thymectomies, one thymomectomy, and one cystectomy in a total of 20 patients with anterior mediastinal masses and then assessed the surgical results. RESULTS: Infrasternal mediastinoscopic surgery was accomplished in 18 of the 20 patients (90%). The pathological diagnoses included 13 Masaoka stage I thymomas, one stage II thymoma, two stage III thymomas, one thymic cyst, one pericardial cyst, one thymic granuloma, and one mature teratoma. Two patients with stage III thymoma required conversion to sternotomy, one for invasion into the innominate vein and the other for invasion into the pericardium. There was no surgically related mortality or complications in any patients. CONCLUSION: Infrasternal mediastinoscopic surgery is safe and feasible for stage I thymoma and other benign tumors in the anterior mediastinum.
Adult↗
[Chronic hemorrhagic pyothorax treated with preoperative internal thoracic and intercostal arterial embolization and perioperative non-invasive positive pressure ventilation].
We reported successful surgery for chronic hemorrhagic empyema with severe right heart insufficiency. The preoperative embolization of right internal thoracic artery and intercostals arteries was effective for the control of intraoperative bleeding. Non-invasive positive pressure ventilation (NIPPV) was useful for the perioperative respiratory management. A 62-year-old female with a history of right pneumonectomy and thoracoplasty for pulmonary tuberculosis was admitted because of dyspnea on effort on Dec 5th 2002. Her right heart insufficiency was worsened gradually. On May 20th 2003, we performed the transcatheter embolization of right internal thoracic and intercostals arteries for the control of intraoperative bleeding. The next day, the curettage and fenestration was performed for intraoperative cardiac dysfunction. The intraoperative bleeding was 1,596 ml and operative time was 2 hours 24 minutes. Due to CO2 narcosis, the ventilator under the intratracheal tube was needed for respiratory management in the postoperative course. The switching of the respiratory management with NIPPV from the intratracheal tube during 8 days, her respiratory and general conditions had been improved gradually. Because of methicillin-resistant Staphylococcus aureus (MRSA) infection of thoracic cavity, the radical thoracoplasty following the latissimus dorsi muscules flap and the omentopexy was performed. The operative course was uneventful and she needed overnight NIPPV without O2 inhalation and was discharged.
Chronic Disease↗
MRI of ectopic posterior pituitary bright spot with large adenomas: appearances and relationship to transient postoperative diabetes insipidus.
MRI of large pituitary adenomas has revealed that a posterior pituitary bright spot (PPBS), comprising ADH-containing neurosecretory granules, is commonly ectopic before surgery and attached to the tip of the pituitary stalk late after surgery. Although the PPBS indicates functional integrity of the posterior lobe, transient diabetes insipidus (DI), caused by deficiency of ADH, is frequent early after surgery. We attempted to clarify how the shape, signal intensity and site of the PPBS before surgery are related to transient DI in the early postoperative period. We carried out MRI on 15 patients with a large adenoma and an ectopic PPBS before surgery and then within 1 week (early), 1-2 months (intermediate) and 6 or more months (late) after the operation. There were nine who had transient DI, which subsided by the intermediate study; none had permanent DI. Regardless of transient DI, the PPBS was visible, and its signal intensity was similar, on all postoperative studies. Although 11 did not change in shape, four showed a remarkable change from a flat shape before surgery to a rounded one postoperatively. On the intermediate MRI, the PPBS had descended to the level of the diaphragma as mass effect disappeared.
Adenoma↗
Unusually widened perivascular spaces in the cerebral hemisphere: the importance of MRI for diagnosis of a benign clinical entity.
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Brain Edema↗
Heavily T2 weighted MR images of anterior optic pathways in patients with sellar and parasellar tumours - prediction of surgical anatomy.
OBJECTIVE: Location of anterior optic pathways in sellar and parasellar tumours was preoperatively evaluated, by use of heavily T2 weighted MR images. METHODS: Heavily T2 and conventional T1 weighted images were studied in 20 patients with sellar and parasellar tumours who underwent craniotomy. Pathology revealed pituitary adenoma in 5 patients, craniopharyngioma in 8 and parasellar meningioma in 7. Maximum sizes ranged from 15 mm to 58 mm. Sequence parameters of TR/TE for heavily T2 weighted and T1 weighted images were 5800/220 msec and 600/20 msec, respectively, and slice thickness was 3 mm for both. RESULTS: The anterior optic pathway was detected in 95% on heavily T2 weighted images and 50% on T1 weighted images. All preoperative heavily T2 weighted images were compatible with operative findings. The optic chiasms were most commonly supero-posterior in pituitary adenomas, anterior (prefixed) in craniopharyngiomas and posterior in meningiomas. The optic nerves were commonly located superior or lateral to the tumours. However, parasellar meningiomas, off the midline, revealed the optic nerves in various locations, depending on the tumour origin. In such tumours, heavily T2 weighted images provided surgical information on the width of the working space through prechiasmal and/or optico-carotid spaces in the pterional approach. Spatial relation of the tumours to the lamina terminalis, anterior commissure and anterior communicating artery complex was clearly shown in craniopharyngioma patients, who underwent the anterior interhemispheric approach. CONCLUSION: Heavily T2 weighted MR images are useful in determining the location of optic pathways and surgical approach and in individual prediction of the anatomy for even large sellar and parasellar tumours.
Adenoma↗
History of allergic disorders in common neurologic diseases in Japanese patients.
OBJECTIVE: To clarify the association between past and present history of allergic disorders and neurologic diseases. METHODS: The past and present history of common allergic disorders together with family history was prospectively studied in all out-patients at the Department of Neurology at Kyushu University Hospital from March 1998 to February 2000. RESULTS: Among 3113 out-patients, 2152 (69.1%) completed a questionnaire. Myelitis showed a statistically significant increase of concomitant atopic dermatitis (P=0.006) and concomitant and past atopic dermatitis (P=0.014), as compared with neurologically healthy controls. Moreover, patients with lower motoneuron disease (LMND) had a statistically significant increase of past and concomitant asthma (P=0.007). None of the other common neurologic diseases showed any increase of allergic disorders when compared with controls. CONCLUSIONS: The present study supports the significant association between allergic disorders and such spinal cord diseases as myelitis and LMND in Japanese patients.
Adolescent↗
Th2 shift in mononeuritis multiplex and increase of Th2 cells in chronic inflammatory demyelinating polyneuropathy: an intracellular cytokine analysis.
To elucidate the T helper 1 (Th1)/T helper 2 (Th2) balance in various inflammatory neuropathies, we measured the ratio of intracellular interferon-gamma (IFN-gamma)-positive to IL-4-positive cells (intracellular IFN-gamma/IL-4 ratio) by flow cytometry in peripheral blood CD4(+) T cells of 14 patients with mononeuritis multiplex (MNM), 12 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 10 patients with Guillain-Barré syndrome (GBS), 23 patients with neurodegenerative disorders and 36 healthy controls by intracellular labeling. The patients with MNM showed a significantly lower intracellular IFN-gamma/IL-4 ratio (P<0.05) and higher IL-4(+)/IFN-gamma(-) cell percentages (P<0.05) than the controls. The increase of IL-4(+)/IFN-gamma(-) cell percentages was especially prominent in MNM of unknown etiology (P<0.005). The patients with CIDP also showed significantly higher IL-4(+)/IFN-gamma(-) cell percentages (P<0.05) than the controls. The IL-4(+)/IFN-gamma(-) cell percentages were increased in some patients with GBS, but the difference was not significant compared with the controls. Thus, our results suggest that a Th2 shift is a characteristic of MNM and may play an important role in the development of the disease.
Adolescent↗
Tc1/Tc2 and Th1/Th2 balance in Asian and Western types of multiple sclerosis, HTLV-I-associated myelopathy/tropical spastic paraparesis and hyperIgEaemic myelitis.
CD8+ T cells, like CD4+ T cells, can differentiate into at least two subsets with distinct cytokine patterns: Tc1 cells produce Th1-like cytokines and Tc2 cells produce Th2-like cytokines. To clarify the immunopathological roles of Tc1 and Tc2 cells in central nervous system (CNS) inflammation, we examined intracellular cytokines in CD8+ and CD4+ T cells by flow cytometry and analyzed the Tc1/Tc2 balance as well as the Th1/Th2 balance in 80 patients with various CNS inflammatory diseases, including 20 with optico-spinal multiple sclerosis (OS-MS), 21 with conventional MS (C-MS), 22 with human T-lymphotropic virus type I (HTLV-I)-associated myelopathy/tropical spastic paraparesis (HAM/TSP) and 17 with hyperIgEaemic myelitis. Twenty-two healthy subjects were also examined as controls. Patients with OS-MS showed a significantly higher percentage of INF-gamma+IL-4- CD8+ T cells as well as CD4+ T cells and a significantly higher intracellular interferon-gamma (IFN-gamma)/interleukin-4 (IL-4) ratio both in CD8+ and CD4+ T cells throughout the relapse and remission phases than the healthy controls. Furthermore, the patients with OS-MS showed a significantly lower percentage of INF-gamma-IL-4+ CD4+ T cells as well as CD8+ T cells during the relapse phase than the healthy controls. On the other hand, the patients with C-MS showed a significantly higher percentage of IFN-gamma-IL-4+ CD8+ T cells in addition to more IFN-gamma+IL-4- CD4+ T cells during the relapse phase than the healthy controls. The HAM/TSP patients showed a significantly higher percentage of INF-gamma+IL-4- CD8+ T cells and a significantly higher intracellular IFN-gamma/IL-4 ratio in CD8+ T cells than the healthy controls. In contrast, in hyperIgEaemic myelitis, in addition to a significantly lower intracellular IFN-gamma/IL-4 ratio in CD4+ T cells, a tendency toward a lower intracellular IFN-gamma/IL-4 ratio in CD8+ T cells in comparison to the healthy controls was observed. These results clarified for the first time the distinct Tc1/Tc2 balance in each disease condition as follows: Tc1 cell response is predominant in OS-MS and HAM/TSP, while Tc2 cell response is predominant in hyperIgEaemic myelitis and at relapse phase of C-MS. Furthermore, our results suggest that CD8+ T cells play an adjunctive role in disease induction and the clinical course of MS.
Adult↗
UV-induced skin carcinogenesis in xeroderma pigmentosum group A (XPA) gene-knockout mice with nucleotide excision repair-deficiency.
Nucleotide excision repair (NER) removes a wide variety of lesions from the genome and is deficient in the genetic disorder, xeroderma pigmentosum (XP). In this paper, an in vitro analysis of the XP group A gene product (XPA protein) is reported. Results of an analysis on the pathogenesis of ultraviolet (UV)-B-induced skin cancer in the XPA gene-knockout mouse are also described: (1) contrary to wild type mice, significant bias of p53 mutations to the transcribed strand and no evident p53 mutational hot spots were detected in the skin tumors of XPA-knockout mice. (2) Skin cancer cell lines from UVB-irradiated XPA-knockout mice had a decreased mismatch repair activity and an abnormal cell cycle checkpoint, suggesting that the downregulation of mismatch repair helps cells escape killing by UVB and that mismatch repair-deficient clones are selected for during the tumorigenic transformation of XPA (-/-) cells. (3) The XPA-knockout mice showed a higher frequency of UVB-induced mutation in the rpsL transgene at a low dose of UVB-irradiation than the wild type mice. CC-->TT tandem transition, a hallmark of UV-induced mutation, was detected at higher frequency in the rpsL transgene in the XPA-knockout mice than the wild type mice. This rpsL/XPA mouse system will be useful for further analysing the role of NER in the mutagenesis induced by various carcinogens. (4) The UVB-induced immunosuppression was greatly enhanced in the XPA-knockout mice. It is possible that an enhanced impairment of the immune system by UVB irradiation is involved in the high incidence of skin cancer in XP.
Animals↗
Modulation of MUC1 mucin as an escape mechanism of breast cancer cells from autologous cytotoxic T-lymphocytes.
MUC1 mucin is known to serve as a target molecule in the killing of breast cancer cells by cytotoxic T-lymphocytes (CTLs). We searched for a possible mechanism allowing tumour cells to escape from autologous CTLs. When the killing of breast cancer cells by autologous lymphocytes was examined in 26 patients with breast cancer, significant tumour cell lysis was observed in 8 patients, whereas virtually no autologous tumour cell lysis was detected in as many as 18 patients. In the patients who showed negligible tumour cell lysis, the autologous tumour cells expressed MUC1-related antigenic epitopes much more weakly than the tumour cells in the patients who exhibited strong cytotoxicity (significant statistically at P< 0.0005-0.0045), suggesting that the unresponsiveness of cancer cells to CTLs observed in these patients was mainly due to loss of MUC1 expression or modulation of its antigenicity. A breast cancer cell line, NZK-1, established from one of the cytotoxicity-negative patients, did not express MUC1 and was resistant to killing by CTLs, while control breast cancer cell lines expressing MUC-1 were readily killed by CTLs. Transfection of NZK-1 cells with MUC1 cDNA induced significant lysis by autologous T-lymphocytes. These results supported the importance of MUC1 mucin in autologous anti-tumour immunity, but suggested that the major escape mechanism of tumour cells from autologous T-lymphocytes is the loss and/or modulation of MUC1 antigenicity on tumour cells, which would limit the effectiveness of possible immunotherapy designed to target the MUC1 mucin.
Antigenic Variation↗
Spinal cord lesions of myelitis with hyperIgEemia and mite antigen specific IgE (atopic myelitis) manifest eosinophilic inflammation.
We report the neuropathological findings of spinal cord specimens obtained from two patients who had localized myelitis with hyperIgEemia and mite antigen specific IgE (atopic myelitis). Both cases showed mild spinal cord dysfunction, and the gadolinium-enhanced area of the isolated spinal cord lesion observed on MRI was biopsied, respectively. Neuropathologically, both cases showed many perivascular lymphocyte cuffings associated with disrupted vessels, and the infiltration of eosinophils in the spinal cord lesions. Both myelin and axons were lost in the lesions, which were associated with astrogliosis. These findings suggest that an allergic mechanism may play a role in this condition.
Adult↗
Heavily T2 weighted MR assessment of fornical injury after anterior interhemispheric approach for large suprasellar tumors.
Fornical injury in transforaminal approach is well known. Its injury in the anterior interhemispheric approach (AIA) has been rarely highlighted. We report 2 cases with a large suprasellar tumor who underwent AIA. Postoperative heavily T2 weighted reversed (T2R) MR images demonstrated its unilateral injury. The clinical significance of symptom-free fornical injury after AIA is discussed. Cases 1 and 2 were a 15 year-old girl with a meningioma and a 49-year-old woman with a craniopharyngioma, respectively. They underwent AIA. Postoperative T2R images revealed unilateral fornical crus atrophy. They did not present associated memory deficits. Case 1 had the injury of both fornical column and anterior commissure. They were speculatively torn by intra-operative lateral retraction of the frontal lobes. Case 2 had unilateral atrophy of the mammillary body and postcommissural fornix, which were probably caused by ischemic damage related to surgical manipulation, since case 2 had an associated anterior thalamic infarct. During the operation for large suprasellar tumors, excessive laterally directed brain retraction should be avoided, since such manipulation may easily tear the overstretched anterior commissure and fornical column. Once we notice or suspect fornical injury on MR studies in cases of re-operation, we have to choose a surgical approach and operative manipulation to preserve an intact fornix. The MR evaluation of fornix should be included in the perioperative radiological assessment, since patients with unilateral fornical injury were free of memory disturbance, and T2R imaging is a useful MR sequence for depicting the anatomy related to the fornix.
Adolescent↗
Recurrent neurohypophyseal germinoma causing invasion localized to temporal bone marrow-unreported neuroimaging studies compared to autopsy findings.
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Adult↗
Infrasternal mediastinoscopic thymectomy in myasthenia gravis: surgical results in 23 patients.
BACKGROUND: Infrasternal mediastinoscopic surgery is a new approach to resection of the anterior mediastinal mass. METHODS: We evaluated this new approach in 23 patients with myasthenia gravis who underwent total thymectomy assisted by infrasternal mediastinoscopy between 1998 and 2000. The results were analyzed with special reference to morbidity and short-term improvement of the disease severity determined according to quantitative myasthenia gravis (QMG) scores. RESULTS: Complete removal of the thymic gland with the pericardial adipose tissue was accomplished through an infrasternal mediastinoscopic approach in 21 of the 23 (91.3%) patients. The remaining 2 patients required conversion to sternotomy, the one for insufficient sternal lifting with vascular tape and the other for invasion of a thymoma to the innominate vein. There was no related mortality and only one complication, a phrenic nerve injury in 1 patient (4.3%). Significant clinical improvement of disease was achieved in the short term and several advantages were apparent. CONCLUSIONS: Infrasternal mediastinoscopic thymectomy is safe and feasible for patients with myasthenia gravis.
Adult↗
Long-term Karnofsky performance status and neurological outcome in patients with neurohypophyseal germinomas.
Long-term posttreatment Karnofsky Performance Status (KPS) and neurological status of 16 patients with neurohypophyseal (NH) germinomas were retrospectively analysed, in order to deduce the best treatment modality to achieve a good outcome. The average age at treatment was 15.5 years (rouge 6-26) and they were followed up for 107.3 months (26-209). The KPS averaged 81.5 (0-100). Three patients were below 60 in the KPS. The first one had hemiparesis due to a delayed cerebrovascular accident (CVA), and the second became indifferent and inactive with recent memory loss. The third died from marginal recurrence of tumour and subsequent systemic metastasis. The low scores were all more or less attributable to complications related to irradiation: delayed CVA, diffuse cortical atrophy and inappropriate selection of irradiation field, respectively. The second case had the tumour origin at the hypothalamus, which indicates intraparenchymal tumour location as another factor to worsen the quality of life (QOL). Visual field and acuity, and EOM impairment were observed in eight and five patients, respectively. It improved in all patients but one. The visual and EOM dysfunction recovered satisfactorily and was not a disabling factor. The intraparenchymal lesion resulting in hemiparesis and higher cortical dysfunction due to either irradiation-related complications or tumour invasion is a major determining factor of lower KPS. Irradiation related complications are considered to be avoidable by reducing radiation dosage with appropriate chemotherapy and/or proper selection of irradiation field. Patients with smaller tumour size of less than 20 mm had higher KPS. Accordingly, repeated studies of tumour markers and neuroimages are required in patients with idiopathic diabetes insipidis, in order to detect the tumour, if present, at the small-sized and early stage.
Adolescent↗