Internal medicine
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A 20-year-old Japanese woman had an attack of acute intermittent porphyria (AIP). Magnetic resonance imaging (MRI) revealed symmetrical lesions in the cerebrum and cerebellar hemisphere, corresponding to posterior reversible encephalopathy syndrome (PRES). ⋯ Repeated MRI and magnetic resonance angiography revealed exacerbated PRES, part of which showed a small infarction, accompanied by progressive vasoconstriction. These findings suggest that the recovery of NO by heme replacement alone is insufficient for preventing brain damage during an AIP attack.
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A 73-year-old man complained of sternoclavicular joint pain; blood tests revealed elevated C-reactive protein. The patient developed delirium; magnetic resonance imaging showed metastatic bone tumors. ⋯ Autopsy revealed abdominal aortic intimal sarcoma with metastasis to the peritracheal lymph nodes and sternum. Peripheral arterial embolism and bone metastasis are common symptoms of aortic intimal sarcoma, which implies a place for aortic intimal sarcoma in differential diagnoses of embolism or bone tumors of unknown origin.
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Intravascular images of coronary stenosis by antiphospholipid syndrome (APS) would be beneficial to understand the mechanism of this disease. A 59-year-old woman with APS/systemic lupus erythematosis (SLE) underwent emergent coronary angiography which revealed stenosis with micro channels in the proximal left anterior descending artery. ⋯ White thrombi were floating distal to the stenosis. Intravascular images obtained by optical coherence tomography (OCT) revealed the microstructure of complex coronary stenosis, which had ambiguous findings on angiography and intravascular ultrasound (IVUS) in an acute coronary syndrome (ACS) patient with APS/SLE.
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Review Case Reports
GM-CSF Autoantibody-positive Pulmonary Alveolar Proteinosis with Simultaneous Myeloproliferative Neoplasm.
Pulmonary alveolar proteinosis (PAP) is classified as autoimmune, secondary, or genetic. We herein describe a 69-year-old man with autoimmune PAP, simultaneously diagnosed with myeloproliferative neoplasm (MPN). ⋯ Throughout the clinical course, no progression of PAP was observed, despite the progression to leukemia. There are few reports of autoimmune PAP with hematological malignancy, and this case demonstrated that an evaluation for GM-CSF autoantibodies is important for distinguishing the autoimmune and secondary forms of PAP, even if the patient has hematological malignancy.
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The incidence of co-infection with Treponema pallidum and human immunodeficiency virus (HIV) is increasing in developing and developed countries. The neurological complications of both infections occasionally occur simultaneously during a clinical course. ⋯ A cerebrospinal fluid examination confirmed the diagnosis of active neurosyphilis based on an elevated T. pallidum hemagglutination assay index. A change in the patient's immune status, possibly due to HIV, might have converted the syphilis from latent to active, leading to LETM of the spinal cord.