Kimura's disease associated with minimal change nephrotic syndrome: A case report

Kimura's disease is a rare disorder that involves regional lymph nodes and the major salivary glands, which become infiltrated by eosinophils and lymphocytes. Renal lesions associated with Kimura's disease are rare. We describe the case of a 60-year-old Japanese woman who first noted a nodular mass in a salivary gland. As the nodule grew, nephrotic syndrome and heart failure developed. A biopsy of the nodule revealed Kimura's disease, and surgical excision was performed. After the operation, the heart failure and nephrotic syndrome, which were diagnosed as minimal change disease on renal biopsy, improved rapidly without steroid therapy. Four months later, the nephrotic syndrome recurred without recurrence of Kimura's disease. The patient showed marked improvement during prednisolone therapy (40 mg/d) and was in complete remission 4 weeks after the initiation of steroid therapy. This case shows that surgical excision and prednisolone therapy are useful for nephrotic syndrome associated with Kimura's disease.     

Altered corticomotor representation in patients with Parkinson's disease.

In 6 patients with Parkinson's disease (PD) and 6 age-matched controls, transcranial magnetic stimulation was applied at 56 regions over the motor cortex and premotor cortex of each hemisphere, with the first dorsal interosseous (FDI) muscle of both hands activated at 15% maximum voluntary contraction during stimulation. For each site, motor evoked potential (MEP) landmarks were recovered, including MEP amplitude, MEP onset latency, and silent period duration. Scaled MEP amplitudes were used to construct individual cortical maps of the FDI muscles. The maps revealed an anterior displacement of the muscle representation in PD patients. This anterior shift over motor cortical areas may reflect increased contributions of corticocortical connections between motor cortex and premotor cortical areas, possibly enhanced by the visual feedback aspect of the task. These alterations may reflect adaptations to the impairments in striatocortical circuits in PD.     

Graft versus graft and graft versus host reactions after HLA-identical bone marrow transplantation in a patient with severe combined immunodeficiency with transplacentally acquired lymphoid chimerism

We describe a patient with severe combined immunodeficiency and transplacental transfer of maternal T cells who received an unfractionated HLA-identical sibling bone marrow transplant without prior conditioning. He presented prior to transplantation with a dermatitis later diagnosed as mild graft versus host disease. He had a normal absolute lymphocyte count, but proliferative responses to mitogens were very low. Antigens of the noninherited maternal HLA haplotype were detected on his blood lymphocytes. After transplantation, he developed a severe reaction including fever, cutaneous erythema and hepatosplenomegaly. Lymphocytes carrying the noninherited maternal HLA haplotype disappeared from his circulation, and his unprimed mononuclear cells became spontaneously cytotoxic to maternal lymphoblasts. He subsequently developed a lymphocytosis of 69,000/mm³, diarrhea, elevated transaminases and a worsening rash, necessitating treatment with immunosuppressive agents. Full T-cell engraftment and evidence of B-cell function later ensued and spontaneously cytotoxic lymphocytes against maternal cells disappeared by 47 days post-transplantation. We postulate that the patient's constellation of signs and symptoms after transplantation represented a combination of severe graft versus graft and mild graft versus host reactions.     

Fast acquisition of myocardial SPECT images with Tc-99m sestamibi for the diagnosis of coronary artery disease

Background  Shortening the acquisition time for myocardial single-photon emission computed tomographic (SPECT) imaging increases patient comfort and laboratory throughput. The purpose of this study was to compare the diagnostic accuracy for coronary artery disease detection of myocardial SPECT images acquired in 5 to 10 minutes versus 25 minutes using Tc-99m methoxyisobutylisonitrile (Tc-99m sestamibi) and a single-head gamma camera. Methods and Results  Forty-one subjects had a standard 1-day rest/stress Tc-99m sestamibi myocardial SPECT study. Two sets of rest and stress images were acquired on the same day for each subject. One set of images was acquired with a 5- to 10-minute fast acquisition protocol; the second set of images was acquired with a 25-minute standard protocol. The accuracies of the fast and standard protocols for identifying individuals with and without coronary artery disease were equivalent. Accuracy was 76% for the fast protocol and 73% for the standard protocol in individuals with at least one coronary stenosis ≥70%. The accuracies of the two protocols for identifying individual coronary arteries with stenoses ≥70% also were equivalent. Accuracy was 77% for the fast protocol and 74% for the standard protocol. Conclusions  SPECT myocardial images may be acquired in as little as 5 to 10 minutes using Tc-99m sestamibi and a 1-day rest/stress protocol. Accuracy is equivalent to that attained in studies with longer imaging time.     

Marchiafava-Bignami disease: CT and MR findings

We describe characteristic findings in a patient with Marchiafava-Bignami disease who was serially imaged with CT and MR. Involvement of the entire corpus callosum was visualized with both types of imaging. Following administration of gadolinium-DTPA, the lesion was clearly enhanced in the subacute stage. Correspondence to: S. Otake     

利培酮与奋乃静治疗脑血管病所致精神障碍的对照研究

目的 比较利培酮与奋乃静治疗脑血管病所致精神障碍的有效性及安全性。方法 将 63例患者按治疗药物分为两组,利培酮组(33例)和奋乃静组 (30例 ),比较两组患者的疗效及副反应。结果在治疗前两组间性别、年龄、病程、BPRS、MMSE等均无统计学差异(P>0. 05),两组在治疗前后BPRS评分均具显著性差异(35. 6±7. 3, 27. 3±6. 1,P<0. 01) (34. 9±6. 5, 28. 1±5. 7,P<0. 01),利培酮组治疗前后MMSE有显著性差异(13. 7±9. 1, 19. 5±9. 4,P<0. 05),两组间在治疗后BPRS无显著性差异(27. 3±6. 1,28. 1±5. 7,P>0. 05),而MMSE和TESS有显著性差异。结论 利培酮治疗脑血管病所致精神障碍与奋乃静疗效相当,但利培酮对认知功能的影响和安全性方面优于奋乃静。     

Hallucinations and sleep–wake cycle in Alzheimer's disease: a questionnaire-based study in 218 patients

Abstract The aim was to evaluate the relationship between hallucinations and the sleep-wake cycle in a sample of Alzheimer's disease (AD) patients in the early-moderate stage. Two hundred and eighteen AD patients (66 males, 152 females, mean age 74.3±6.85) were administered a sleep questionnaire in the presence of a care-giver. Twenty-six out of 218 (12%) reported the occurrence of hallucinations, mainly visual. In 18/28 (69%) hallucinations occurred when the patient was awake and in 8 (31%) hallucinations were reported to occur close to a specific phase of the sleep-wake cycle. Vivid dreams were reported in 25/218 (11%) and violent sleep-related and dream-related behaviours (probable REM behaviour episodes) in 22/218 (10%). Both REM phenomena were more frequent in AD hallucinators than in AD non-hallucinators (26.9% vs. 9.3%, and 26.9% vs. 7.8%, p<0.007). Our data indicate a lower incidence of hallucinations and presumable REM behaviour disorder (RBD) in AD, at least in the early-moderate phase, than that observed in synucleinopathies. However, the higher occurrence of vivid dreams and RBD in AD patients with hallucinations compared to those without hallucinations indicates a potential role of disordered REM sleep in influencing the occurrence of hallucinations in AD, similar to what has been observed in synucleinopathies.     

经皮肺活检及支气管肺泡灌洗对周围性肺疾病诊断的临床价值

目的：探讨经皮肺活检及支气管肺泡灌洗(BAL)对周围性肺疾病诊断的价值。方法：对以结节或块状阴影为主的52例病人行经皮肺活检；对以弥漫性周围性肺疾病为主的33例病人行BAL检查；对采用两种不同方法的检查结果进行阳性率分析。结果：52例经皮肺活检对肺周围性病变诊断敏感性为88％，特异性100％。33例行BAL检查对结核及恶性弥漫性肺周围性病变诊断敏感性为75％～80％，特异性100％。结论：BAL检查及经皮肺活检对肺周围块状阴影或弥漫性病变的确诊有很高价值。     

SARS家庭聚集型病例8例报告分析

SARS是由变异冠状病毒引起的新型烈性传染病，具有在家庭和医院内聚集发病的特点。其影像学表现对于临床诊断和随诊观察具有重要价值。本文报告了分属3个家庭的8个病例发病早期(1周)影像学表现(包括胸部平片和CT)和部分临床表现资料，讨论并分析SARS早期影像学表现的若干特点及与临床表现之间的关系。