Lewy body-type degeneration in cardiac plexus in Parkinson's and incidental Lewy body diseases

Heart tissues of patients with PD or incidental Lewy body (LB) disease (ILBD) were examined by light and electron microscopy. LBs and alpha-synuclein-positive neurites were identified in the hearts from 9 of 11 patients with PD and from 7 of 7 patients with ILBD. LBs were present in both tyrosine hydroxylase-positive and -negative nerve processes, which are nerves of extrinsic sympathetic and intrinsic origin, respectively. These findings provide histologic evidence that the postganglionic sympathetic and intrinsic neurons in the heart are involved in the PD disease process.     

A novel MPZ gene mutation in dominantly inherited neuropathy with focally folded myelin sheaths

We found the association of a heterozygous novel MPZ gene point mutation, Ile62Phe in exon 2, with autosomal dominant motor and sensory neuropathy with focally folded myelin sheaths. Family study revealed that de novo Ile62Phe mutation on the MPZ gene occurred in the proband and was inherited by her children with early onset slowly progressive neuropathy. Our study suggests that the characteristic pathologic findings of the sural nerve in these patients are closely related to the site and nature of amino acid substitutions of the MPZ gene.     

The early induction of cyclooxygenase 2 associated with neurofibrillary degeneration in brains of patients with Fukuyama-type congenital muscular dystrophy

Brains of thirteen patients with Fukuyama-type congenital muscular dystrophy (FCMD) were evaluated regarding the expression of cyclooxygenase 2 (COX2), an enzyme involved in the synthetic pathway of prostaglandins and thromboxanes, as well as neurofibrillary tangles (NFT). The neuronal induction of COX2 was demonstrated with immunohistochemistry and Western blotting confirmed the up-regulation. Preceded by COX2 immunoreactivity, NFT-containing neurons appeared in the majority of FCMD patients without beta-amyloid deposition or senile plaques. The hippocampus did not demonstrate neurodegeneration, while, in other areas, neurons with NFT spread in a similar manner to Alzheimer's disease. NFT-bearing neurons were concomitantly shown to be immunoreactive to COX2. The precedent induction of COX2, therefore, may be related to the formation of NFT in this genetic disorder.     

Hyperthermo-chemotherapy combined with cytoreductive surgery for the treatment of gastric cancer with peritoneal dissemination

Continuous hyperthermic peritoneal perfusion (CHPP) with anticancer agents (mitomycin C and cisplatin) in warm saline was performed in patients with peritoneal dissemination of gastric cancer following resection of the primary lesion. The effect of CHPP was examined by a second-look operation. This study includes 41 cases of gastric cancer with peritoneal dissemination but without liver metastasis treated during the past 6 years. The overall median survival was 14.6 months to 64.2 months from CHPP to death and the 3-year survival rate was 28.5%. Second look surgery revealed a remarkable diminution in the degree of peritoneal dissemination in 7 (50%) of 14 patients with disappearance of ascites after only one course of CHPP in 7 (77.8%) of 9 patients. Long-term 3 year-survival was noted in 4 (9.8%) patients on CHPP. Side effects were renal insufficiency in 2 (5%) patients, leukopenia in 2 (5%) patients, and perforation of the small intestine in 1 (2%) patient. These results suggest the effectiveness of CHPP in the treatment of gastric cancer with peritoneal dissemination.

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Resumen La perfusión hipertérmica continua (PHTC) con agentes anticancerosos (mitocina G y cisplatino) y solutión salina fue realizada en pacientes con cáncer gástrico con diseminación peritoneal después de resección de la lesión primaria, y el efecto de PHTC fue determinado mediante reexploración (operación de second look, OSL). La población de pacientes está constituída por 41 casos de cáncer gástrico con diseminación peritoneal pero sin metástasis hepáticas, tratdos en el curso de los últimos 6 años. La sobrevida media global fue de 437 dias (rango 28 a 1925 días) desde la PHTC hasta la muerte y la tasa de sobrevida a 3 años fue 28.5%. La OSL reveló una notoria disminución de la diseminación peritoneal en 7 (50%) de 14 casos y desaparición de la ascites después de sólo un ciclo de PHTC en 7 de 9 casos con ascitis. Sobrevida de 3 años ocurrió en 4 casos. Los efectos colaterales fueron insuficiencia renal en 2 casos (5%), leucopenia en 2 casos (5%) y perforación del intestino delgado en 1 caso (2%). Los anteriores resultados sugieren que la PHTC es eficaz en el tratamiento del cáncer gástrico con diseminación peritoneal.

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Résumé La perfusion péritonéale continue hyperthermique (PPCH) avec des agents anticancéreux comme le mitomycine C et la cis-platine avec sérum physiologique chauffé a été instaurée lorsqu'une carcinose d'origine gastrique a été trouvée. Les effets de la PCH ont été évalués chez 16 patients lors d'un second-look (SL). Cette étude concerne 41 patients avec carcinose péritonéale sans métastase hépatique observés au cours des 6 dernières années. La survie globale médiane était de 437 jours (extrêmes 28 à 1925 jours): le taux de survie a 3 ans était de 28.5%. Les lésions avaient diminué de façon notable chez 7 (50%) de 14 patients. L'ascite a disparu dans 7 des 9 cas. Une survie à long terme (3 ans) a été notée dans 4 cas. Les effets secondaires ont été une insuffisance rénale dans 2 cas (5%), une leucopénie dans 2 cas (5%) et une perforation de l'intestin grêle dans un cas (2%). Les résultats suggèrent que la PPCH est efficace dans le traitement du cancer gastrique avec dissémination péritonéale.

     

Brain lesion in congenital myotonic dystrophy

Congenital myotonic dystrophy (CMyD) affects the brain, causing mental changes and psychomotor retardation. However, the pathophysiology of the brain dysfunctions in CMyD remain to be clarified. We described two cases of CMyD with brain abnormalities. Case 1 was diagnosed as having ventricular dilation at 17 days after birth, and died at 3 years and 6 months. Case 2 was diagnosed as having ventricular dilation at birth, and died at 1 year and 3 months. Pathologically, both cases showed remote hypoxic ischemic brain damage and leptomeningeal glioneuronal heterotopia (LGH). In our patients, the white matter changes may have been caused by perinatal asphyxia, and LGH by embryological abnormalities. Taken our data and those of previous reports together, it is suggested that cerebral abnormalities in CMyD are ascribed to both hypoxic ischemic changes and histogenetic abnormalities.     

Giant osteoma of the temporal bone with otitis media: a case study

A 71-year-old female presented with a giant osteoma of the right temporal bone and otitis media. Computed tomography (CT) and magnetic resonance imaging (MRI) confirmed the presence of the giant osteoma. An operation was performed to alleviate the difficult-to-treat otitis media and to address the related cosmetic problem. During the operation, three-dimensional CT (3D-CT) was very helpful in understanding the relationship between the tumor and the peripheral structures. Removal of the tumor improved the patient's otitis media. Osteoma of the temporal bone is rare. Only twenty-one cases of mastoid osteoma have been reported in Japan. In the present study, we review osteomas of the temporal bone and discuss their management.     

Correlation between high-resolution computed tomographic, magnetic resonance and pathological findings in cases with non-cancerous but suspicious lung nodules

Computed tomography scans, including thin-section high-resolution computed tomography (HRCT), occasionally fail to differentiate between small non-cancerous nodules from lung cancers. We describe nine such lesions ( < 20 mm in diameter) initially identified through our screening program for lung cancer using CT scanning. Pathological diagnoses included nodular fibrosis (n = 4), granuloma (n = 1), cryptococcoma (n = 1), localised organising pneumonia (n = 1), inflammatory pseudo-tumour (n = 1) and sclerosing haemangioma (n = 1). High-resolution CT findings, together with MRI findings with contrast-enhanced dynamic studies, were retrospectively evaluated. Additional cases should be identified and radiologically characterised in order to reduce the number of non-cancerous tumours that are treated by unnecessary surgery. Received: 28 February 2000; Accepted: 29 February 2000     

The problem of a long-term follow up in patients with renal cell carcinoma

PURPOSE: We investigated on a problem of long-term follow up in patients with renal cell carcinoma. PATIENTS AND METHODS: A total of 287 patients with renal cell carcinoma treated in Nara Medical University and affiliated facilities from January 1980 to December 1990 were examined. And we investigated the trend of explanation to patients including 287 patients from 1991 to 1995. RESULTS: Up to December 1995, there are 76 patients (26.5%) unable to be followed and 211 patients able to be followed. The former group patients were less declared cancer rather than the latter group patients. Of 76, 22 patients (28.9%) might misunderstand completely recovering from the cancer disease. For the recent 5 years, those patients who were declared cancer increased, and those patients who were explained benign disease decreased. CONCLUSION: These results suggested that declaration of cancer is important for patients with renal cell carcinoma to be followed for a long-term.     

A case of chromophobe cell renal carcinoma

We report a case of chromophobe cell renal carcinoma. A 27-year-old male suffered from right flank pain and gross hematuria. Drip infusion pyelography and ultrasonographic examination revealed a right renal mass and it was diagnosed as renal oncocytoma by needle biopsy of the right kidney. He was referred to our hospital for the operation and right radical nephrectomy was performed. The cut surface of the tumor was beige in color. The cytoplasm of the tumor cells stained positively for colloidal iron and showed a negative reaction for Vimentin. From these results, this tumor was given a diagnosis of chromophobe cell renal carcinoma.     

贵州省2003～2004年麻疹疫苗强化免疫效果评价

目的总结贵州省实施麻疹疫苗(MV)强化免疫的效果。方法综合分析强化免疫期间的现场调查、项目年度评估报告和强化免疫后1年的麻疹监测系统和法定传染病报告系统的疫情资料。结果本次强化免疫共计接种目标儿童8 611 528人,报告接种率和调查接种率均>95%;共报告预防接种异常反应354例,发生率4.11/10万;接种后经过1个最长潜伏期,麻疹发病数明显减少,每周报告疑似麻疹2~34例,且近1/3为风疹;目前78.3%的麻疹病例发生在8月龄~14岁,病例71.1%无免疫史或免疫史不详。结论高质量MV强化免疫能够迅速降低麻疹发病,实施该项工作的关键环节包括广泛的社会动员、细致的培训和清晰的物流是前提保障;目标人群的确定必须结合当地流行病学监测的资料;尽可能在较大范围内开展,以形成有效的人群免疫屏障阻断麻疹病毒传播;接种时间选择在麻疹低发季节,且尽可能在15d内完成;保证>95%接种率是活动的前提目标;必须针对可能发生的预防接种后异常反应做好充分准备;如果该地区常规免疫服务质量在1~2年内不能明显加强,需要同时考虑4~5年后的后续免疫。