Internal medicine
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We encountered a 77-year-old Japanese man who presented with nephrotic-range proteinuria 20 days after receiving ramucirumab treatment for metastatic sigmoid colon cancer. A kidney biopsy showed two characteristic histological findings. The first finding was podocyte injury with cellular crescent-like formation, although focal segmental glomerulosclerosis (FSGS) (collapsing variant) according to the Columbia classification may have been a more appropriate name for this injury, as hypertrophy and hyperplasia of epithelial cells, presumably resulting from podocyte injury, were seen between Bowman's capsule and the glomerular basement membrane (GBM); these changes appeared to be due to the collapse of the GBM rather than to GBM destruction with fibrinoid necrosis. ⋯ Proteinuria and renal dysfunction subsided after discontinuation of ramucirumab. Bevacizumab has been reported to induce glomerular microangiopathy with endothelial damage and swelling six months after treatment, but in this case, ramucirumab may have induced focal segmental glomerulosclerosis (FSGS) collapsing variant and glomerular microangiopathy with endotheliopathy via mesangial damage within 1 month. We believe that the damage to the glomerular podocyte and endothelial cells via mesangial damage secondary to ramucirumab in our patient was a different type of glomerular microangiopathy than the endothelial cell damage with enlargement of the subendothelial space caused by bevacizumab.
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Bing-Neel syndrome (BNS) is a rare central nervous system manifestation of lymphoplasmacytic lymphoma/Waldenström macroglobulinemia (LPL/WM). We herein report a 62-year-old man with LPL/WM after multiple chemotherapies. He had weakness of lower extremities and elevated serum IgM levels. ⋯ He was diagnosed with BNS and started on treatment with tirabrutinib 480 mg daily. Within three months, he showed clinical and radiologic improvement. Tirabrutinib may have utility as an effective treatment for BNS.
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Bow hunter's stroke is a rare cause of vertebrobasilar infarction. There is no consensus regarding the optimal treatment. We herein report a case of bow hunter's stroke successfully treated by endovascular treatment. ⋯ Dynamic angiography revealed thrombus formation and hypoperfusion of the right vertebral artery upon head rotation to the left. Endovascular parent artery occlusion of the right vertebral artery was performed, and there was no recurrence at follow-up MRI. Endovascular parent artery occlusion might be a useful treatment for bow hunter's stroke.
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Case Reports
Female Urinary Retention Progressing to Possible Multiple System Atrophy-cerebellar Form after 12 Years.
We herein report a 73-year-old Japanese woman with possible multiple system atrophy-cerebellar form (MSA-C) who suffered from urinary retention (sacral autonomic disorder) for 12 years before exhibiting cerebellar ataxia. A peculiar combination of findings on urodynamics and sphincter electromyography (EMG), e.g. detrusor hyperactivity with impaired contraction (DHIC), detrusor-sphincter dyssynergia (DSD) and neurogenic sphincter EMG (upper and lower neuron-type autonomic dysfunction), seems to have been predictive of future development of MSA.
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Pituitary inflammation due to IgG4-related disease is a rare condition and is sometimes accompanied by central diabetes insipidus. Central diabetes insipidus produces a strong thirst sensation, which may be difficult to distinguish when complicated by salivary insufficiency. A 45-year-old man was admitted to our department for a thorough examination of his thirst and polyuria. ⋯ His symptoms were subsequently alleviated, and his serum IgG4 level finally normalized. We should remember that IgG4-related disease can be accompanied by hypophysitis and that central diabetes insipidus is brought about by IgG4-related hypophysitis. This case report should remind physicians of the fact that pituitary inflammation due to IgG4-related disease is very rare and can be masked by symptoms due to salivary gland inflammation, which can lead to pitfalls in the diagnosis in clinical practice.