A 75-year-old man, with a history of descending thoracic aortic rupture and dissection treated with aortic stenting at 73 years old, was admitted for rehabilitation following recurrent cerebral ischemic attacks. Upon admission, blood tests revealed elevated inflammatory markers, including a C-reactive protein (CRP) level of 10.75 mg/dL and a D-dimer level of 4.2 µg/mL, alongside microcytic anemia. Despite thorough evaluations using computed tomography (CT) and ultrasound, the origin of these abnormalities remained unidentified. Two months later, MRI using diffusion-weighted whole-body imaging with background body signal suppression (DWIBS) revealed hyperintensities in the thoracic aorta. He remained asymptomatic and progressed well during rehabilitation, prompting continued observation. However, three months after admission, he developed hemoptysis. Contrast-enhanced CT showed pneumonia, as well as enhanced lesions in the aortic wall, confirming aortic inflammation. Due to concerns about aortic stent ulceration, an emergency stent graft insertion extending to the superior mesenteric artery was performed. He recovered uneventfully and was discharged. DWIBS is an MRI-based tool that avoids exposure to radiation or contrast agents and is cost-effective. MRI using DWIBS demonstrated high signal accumulations in the aortic wall, indicative of inflammation. These findings suggest that DWIBS holds significant potential as a powerful imaging tool for detecting and assessing inflammation, particularly in the aorta.
Background/Objectives: We describe a case of intravascular large B-cell lymphoma (IVLBCL) presenting with recurrent cerebral infarctions and review similar reported cases. Our aim is to explore potential early diagnostic markers and discuss their prognostic implications. Methods/Results: A 79-year-old man with a history of hypertension, hyperuricemia, and postoperative bladder cancer presented with five to six cerebral infarctions over an 11-month period, despite successive changes in antiplatelet and anticoagulant medications. Neurological examination revealed decreased pain sensation, bilateral hearing loss, and right thenar atrophy. Laboratory studies showed elevated inflammatory markers and soluble IL-2 receptor. CSF analysis revealed elevated protein, β2-microglobulin, IL-6, and IL-10 levels. A skin biopsy was performed to investigate suspected IVLBCL. Histopathological examination of the skin biopsy revealed large pleomorphic CD20-positive cells within the vasculature, confirming a diagnosis of IVLBCL. The patient was treated with chemotherapy, including dose-adjusted R-CHOP and high-dose methotrexate, and achieved complete remission. No recurrence of cerebral infarction was observed during a two-year follow-up period. Conclusions: This case highlights the importance of considering IVLBCL in patients with recurrent strokes of unknown etiology, especially when laboratory or imaging findings suggest systemic involvement. Early recognition and appropriate tissue diagnosis, such as skin biopsy, are essential for timely treatment and favorable prognosis.
A 70-year-old Japanese woman with longstanding hearing loss and asthma developed floating sensations, left finger numbness, and postural instability one day after influenza vaccination, leading to hospital admission. Neurological examinations showed hearing loss, hyperreflexia, left-predominant ataxia, bilateral mild bathyanesthesia, and inability to tandem gait. Cerebrospinal fluid (CSF) analysis showed no pleocytosis or malignant cells, but revealed positive oligoclonal bands and elevated myelin basic protein. Despite no contrast agent use due to asthma, brain magnetic resonance imaging (MRI) revealed pontine hyperintensities on diffusion-weighted imaging (DWI) and T2-fluid attenuated inversion recovery (T2-FLAIR) sequences, along with hyperperfusion on arterial spin labeling (ASL) imaging. Serum anti-aquaporin-4 antibodies (AQP4-Ab) were negative by ELISA. Given the temporal proximity to vaccination and elevated demyelination markers, brainstem-type acute disseminated encephalomyelitis (ADEM) was initially suspected. Symptoms nearly resolved after two cycles of methylprednisolone pulse therapy. Notably, hyperperfusion gradually improved on ASL imaging. Post-discharge, a cell-based assay confirmed the diagnosis of neuromyelitis optica spectrum disorder (NMOSD) by detecting positive anti-AQP4-Ab. She has been relapse-free for about a year without any immunosuppressants or biologics. Although contrast-enhanced MRI remains the gold standard modality for lesion evaluation due to its high sensitivity, hyperperfusion on ASL may provide a useful alternative in patients for whom contrast agents are contraindicated, such as those with asthma or impaired renal function.
We present the case of an 83-year-old woman with a long-standing history of rheumatoid arthritis (RA) who was found collapsed at home. The patient presented with cardiopulmonary arrest and could not be resuscitated. A postmortem examination was performed to determine the cause of death. Postmortem computed tomography (CT) ruled out intracranial hemorrhage but revealed diffuse bilateral pulmonary consolidations and signs of bronchial obstruction. The autopsy revealed severe pulmonary edema and marked right ventricular hypertrophy. Microscopic examination of the lungs demonstrated characteristic features of pulmonary veno-occlusive disease (PVOD), including widespread fibrous intimal thickening and occlusion of small pulmonary veins and venules. Notably, there was no evidence of RA-associated interstitial lung disease (ILD). The direct cause of death was identified as pulmonary edema secondary to PVOD. This case highlights that PVOD can occur in patients with RA as a distinct pathological entity, independent of ILD. This finding is significant as it contrasts with previous reports where PVOD was associated with ILD. Therefore, clinicians should consider PVOD in the differential diagnosis of RA patients who present with unexplained pulmonary hypertension or progressive dyspnea, even in the absence of interstitial lung disease.