This review describes methods utilized in Japan to diagnose and treat patients with IgG4-related disease. A diagnosis of IgG4-related disease is based on elevated serum IgG4 concentration and an increased number of IgG4 + plasma cells. Differentiating IgG4-related disease from other disorders, especially malignancy, is quite important. Consensus treatment in Japan consists of an initial dose of prednisolone at 0.5-0.6 mg/kg/day, followed by careful and gradual dose reduction. Most patients require maintenance treatment at 5 to 10 mg/day. Patients refractory to glucocorticoids are either truly refractory or have been misdiagnosed, therefore requiring reassessment. 〔J Clin Exp Hematop 54(2) : 95-101, 2014〕
Transiently high intensities in the splenium of the corpus callosum (SCC) on T2-weighted magnetic resonance imaging (MRI) are increasingly reported [1]; however, their pathophysiology remains unknown. Here, we report various neuroimaging findings to clarify their pathophysiology. Case reportA 23-year-old man had fever of 5 daysÕ duration. He was hospitalized because of visual hallucinations (VH) and delirium. There was no previous history of neurological diseases, alcoholism or malnutrition. Chest X-ray and laboratory tests revealed left bronchopneumonia. A blood vitamin B1 level was normal. The serum arginine vasopressin (AVP) level was elevated (4.9 pg/ml) with normal osmolarity (307 mOsm/l) on admission but was normal (1.5 pg/ml) on day 5. Serum autoantibodies were negative. Routine cerebrospinal fluid (CSF) findings were normal, with normal myelin basic protein level and negative oligoclonal immunoglobulin G (IgG) bands. Infection of mycoplasma and various viruses including herpes simplex virus was excluded by serum and/or CSF antibody titers or polymerase chain reaction (PCR). We performed various neuroimaging analyses on day 2. T2-weighted MRI revealed a high intensity in the SCC, which showed negative enhancement with a single dose of gadolinium-DTPA, decreased water diffusivity on diffusion weighted MRI, and normal fractional anisotropy (FA) on tensor MRI (Fig. 1). Magnetic resonance spectroscopy (MRS) showed normal N-acetylaspartate (NAA)/creatine (Cr) and choline (Cho)/Cr ratios in the voxel mainly containing the SCC. Perfusion MRI and single photon emission computed tomography (SPECT) showed hypoperfusion in both occipital lobes with normal T2-wieghted and fluid attenuated inversion recovery (FLAIR) imaging, suggesting the diminished metabolic status. Electroencephalogram (EEG) revealed no epileptic discharges. The SCC lesions completely disappeared after 3 months. DiscussionThis is the first report to describe in detail neuroimaging findings of a transient SCC lesion. Diffusion tensor MRI permits the use of FA to evaluate the intrinsic directionality of water diffusion within fibrous structures of myelinated neural axons; ex. decreased FA mostly indicates neuroaxonal degeneration [2]. Normal FA in our patient may reflect intramyelinic edema, non-degenerative intercellular edema speculated previously [3], since the diffusion directionality of water trapped between myelin layers may not differ from that within fibers. The lesion reversibility also supports intramyelinic (intercellular) edema despite showing decreased water diffusion revealed by apparent diffusion coefficient (ADC) maps, which normally indicates frequently irreversible cytotoxic (intracellular) edema [4]. MRS revealed normal NAA/Cr and Cho/Cr ratios, reflecting no neuroaxonal damage and demyelination, respectively. By contrast, low NAA/Cr ratios are seen in ischemia and high Cho/Cr ratios in tumors and demyelinating diseases [5]. All of these findings support a non-degenerative change.Our SPECT and perfusion MRI findings indicated a tran...
Non-contrast-enhanced MR portography with the flow-out method improves the visualization of the intrahepatic portal vein in comparison with the flow-in method. J. Magn. Reson. Imaging 2014;40:583-587. © 2013 Wiley Periodicals, Inc.
A 23-year-old previously healthy man (Patient 1) and a 33-year-old woman with a past history of depression (Patient 2) developed neurological symptoms approximately 1 week after receipt of the first COVID-19 mRNA vaccination and deteriorated over the next week. Patient 1 reported nausea, headache, a high fever, and retrograde amnesia. Patient 2 reported visual disturbance, headache, dysarthria, a left forearm tremor, dysesthesia of the mouth and distal limbs, and visual agnosia. PCR test results for SARS-CoV-2 were negative. Complete blood cell count, biochemistry, and antibody test and cerebrospinal fluid test findings were unremarkable. Diffusion-weighted and fluid-attenuated inversion recovery MRI of the brain showed a high signal intensity lesion at the midline of the splenium of the corpus callosum compatible with cytotoxic lesions of the corpus callosum (CLOCCs). High-dose intravenous methylprednisolone improved their symptoms and imaging findings. CLOCCs should be considered in patients with neurological manifestation after COVID-19 vaccination.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.