Serial diffusion-weighted MRI (DWI) in a patient with sporadic Creuztfeldt-Jakob disease]

Serial DWIs were performed in a patient with CJD who developed symptoms acutely and progressed rapidly. DWI discloed an increased signal in the frontal and parietal inner cortical areas, and in the caudate nuclei and putamina 20 days after the onset of symptoms. T2-weighted images showed only signal abnormality in the caudate nuclei and putamina, but not in the cerebral cortex. In the CSF obtained 15 days after the onset of symptoms, total tau protein was markedly elevated and 14-3-3 protein was positive. Measurement of these proteins are highly specific and sensitive for the diagnosis of CJD, but not available as a rapid routine examination at present. DWI is not specific, but useful for making the diagnosis of CJD in the early stage of the disease.     

Pulmonary surfactant proteins and lipids as modulators of inflammation and innate immunity

Objectives: The pulmonary surfactant system of the human lung consists of unique lipids and proteins that contribute to the biophysical and innate immune properties of the organ. Surfactant protein A (SP‐A) is an oligomeric protein consisting of 18 protomers with collagen and lectin–like domains that recognizes glycoconjugates, lipids and protein determinants on both host cells and invading microorganisms. The authors examined the interaction of SP‐A with Mycoplasma pneumoniae and the influence of the protein upon the innate immune response to the bacteria. Methodology: The authors quantified SP‐A interaction with bacteria using ELISA, and identified the major surface ligand by thin layer chromatography, HPLC and mass spectrometry. The inflammatory response of human and rat macrophages was measured by quantifying tumour necrosis factor‐α secretion using ELISA, and nitric oxide production. Results: SP‐A bound the bacteria with high affinity and enhanced the inflammatory response of human and rat macrophages to the organism and its membranes. Analysis of the interaction of SP‐A with the bacteria revealed that the major ligand was a phospholipid. The lipid ligand was purified by a combination of thin layer and HPLC, and identified by mass spectrometry. The mass spectrometry demonstrated that the SP‐A reactive lipid consisted of several disaturated molecular species of phosphatidylglycerol (PtdGro). Additional experiments were performed to determine if disaturated PtdGro was capable of interfering with the action of SP‐A as an inhibitor of bacterial lipopolysaccharide‐induced inflammatory mediator production by macrophages. The disaturated PtdGro failed to alter the anti‐inflammatory action of SP‐A but unexpectedly these same studies revealed that unsaturated PtdGro can modify the host response to lipopolysaccharide. Conclusions: These findings reveal that both the lipids and proteins of pulmonary surfactant play a role in regulating the host response to invading microorganisms.     

Successful mitral valve repair for active infective endocarditis accompanied by anorexia nervosa

Infective endocarditis of the mitral area accompanied by anorexia nervosa is extremely rare. A 34-year-old Japanese woman presented with high fever and a heart murmur that had developed over the previous 2-month period. Echocardiography revealed mitral regurgitation and vegetation attached to the anterior mitral leaflet, which had markedly prolapsed to the left atrium. We removed the vegetation with a small part of the anterior mitral leaflet and successfully repaired the mitral valve. The patient showed good recovery, and the mitral regurgitation and left ventricular chamber size had satisfactorily decreased at 2 months after the operation.     

Intrapulmonary Schwannoma

A 58-year-old woman was admitted due to an abnormal shadow on chest X-ray, without any symptoms. Chest computed tomography showed a round mass in the anterior segment of the right upper lobe. Segmentectomy was performed and histopathological examination revealed a primary neurogenic tumor of Schwann cell origin. Immunohistochemical staining demonstrated the presence of S-100 protein in the tumor cells. We present a case of intrapulmonary schwannoma and review 62 cases of primary schwannoma of the lung.     

Solitary right ventricle metastasis by renal cell carcinoma.

A 57-year-old man with a history of renal cell carcinoma presented with presyncope. He underwent nephrectomy years earlier followed by HLA-matched allogeneic peripheral-blood stem-cell transplantation. Echocardiographic investigation revealed a solitary right ventricle mass without contiguous vena caval or right atrial involvement. The mass was pathologically confirmed to be metastatic carcinoma in the right ventricular cavity. This case highlights the need to consider an underlying neoplastic syndrome in patients presenting isolated right ventricle mass by echocardiography.     

ENDOSCOPIC MUCOSAL RESECTION AND SUBMUCOSAL DISSECTION METHOD FOR LARGE COLORECTAL TUMORS

The goal of endoscopic mucosal resection (EMR) is to allow the endoscopist to obtain tissue or resect lesions not previously amenable to standard biopsy or excisional techniques and to remove malignant lesions without open surgery. In this article, we describe the results of conventional EMR and EMR using an insulation‐tipped (IT) electrosurgical knife (submucosal dissection method) for large colorectal mucosal neoplasms and discuss the problems and future prospects of these procedures. At present, conventional EMR is much more feasible than EMR using IT‐knife from the perspectives of time, money, complication, and organ preservation. However, larger lesions tend to be resected in a piecemeal fashion; and it is difficult to confirm whether EMR has been complete. For accurate histopathological assessment of the resected specimen en bloc EMR is desirable although further experience is needed to establish its safety and efficacy. Further improvements of in EMR with special knife techniques are required to simply and safely remove large colorectal neoplasms.     

Long-term survival of a pulmonary artery sarcoma produced by aggressive surgical resection and adjuvant chemoradiotherapy.

Malignant pulmonary artery tumors represented by sarcomas are rare, but fatal. Early diagnosis and radical surgical resection offer the only chance for survival. However, surgical intervention has some challenging aspects, and prognosis is poor even after tumor resection. We report a case of a pulmonary artery sarcoma between the right ventricular outflow tract and the pulmonary artery branches. The tumor was aggressively extracted with reconstruction using a cryopreserved pulmonary valved allograft, followed by adjuvant chemoradiotherapy. At 56 months after surgery, the patient is well without any evidence of recurrence, demonstrating that aggressive surgical resection with adjuvant chemoradiotherapy can prolong survival.     

International project--MGTX study]

MGTX Study is a multi-center, international, single-blinded, randomized trial to determine whether extended transsternal thymectomy (ETTX) for patients with myasthenia gravis receiving the prednisone protocol confers added benefits to the prednisone protocol alone. The aims of the study are answering following three questions. 1) Does ETTX combined with prednisone result in a greater improvement in myasthenic weakness, compared to prednisone alone? 2) Does ETTX combined with prednisone result in a lower total dose of prednisone, thus decreasing the likelihood of concurrent and long-term toxic effects, compared to prednisone alone? 3) Does ETTX combined with prednisone enhance quality of life by reducing adverse events and symptoms associated with the therapies, compared to prednisone alone? Primary outcome measure is consists with 1) Comparison of the prednisone protocol alone to prednisone protocol plus ETTX, based on the clinical response to therapy measured over the 3 year trial period by the Area Under the Quantitative Myasthenia Gravis (QMG) Weakness Score (AUQMG), and 2) Testing the difference in the total prednisone used over the 3 year trial period measured by pill count from blister packs (Area Under the Prednisone Dose Time Curve, AUDTC), conditional on the results of comparing AUQMG.     

Pulmonary injury by an over-the-counter drug, Nospole G]

A 78-year-old woman was admitted with dyspnea. She had caught a cold and took an over-the-counter drug (Nospole G) for 2 weeks before admission. Chest radiography and CT scanning showed bilateral diffuse and interstitial shadows, and arterial blood gas analysis demonstrated severe hypoxemia. Withdrawal of Nospole and treatment with both corticosteroid and sivelestat sodium resulted in improvement of clinical findings and successful recovery from mechanical ventilation. A drug lymphocyte stimulation test for Nospole G was positive. Based on these findings, we determined that this patient had drug-induced pneumonitis caused by Nospole G. Finally, she died of sepsis caused by multidrug-resistant Staphylococcus aureus (MRSA) infection. In summary, we report here an elderly case of drug-induced pneumonitis successfully weaned from mechanical ventilation by early treatment with corticosteroid and sivelestat sodium, monitored by changes of markers for interstitial pneumonitis (KL-6, SP-A, SP-D).     

Delayed onset massive oedema and deterioration in traumatic brain injury.

A 52-year-old man fell from standing and a computed tomography (CT) scan revealed traumatic intracerebral haematoma and subarachnoid haemorrhage in the temporal cortex. He was treated without surgery and discharged. On day 30 after the accident, he had no neurological deficit. On day 37 he complained of headache and urinary incontinence, and on day 39 he was hospitalized due to progressive neurological deterioration (reduced conciousness, dilated pupils, and left hemiplegia). A CT scan revealed a diffuse low-density in the right cerebral hemisphere with marked midline shift. Emergency decompressive craniectomy and right temporal lobectomy were performed. Angiography after surgery revealed moderate vasospasm in the right middle and anterior cerebral arteries. The patient remained severely disabled. Delayed onset neurological deterioration can be caused by brain oedema and vasospasm after traumatic brain injury, despite an intervening period of improvement.