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Hypoxia-inducible factor prolyl hydroxylase (HIF-PH) inhibitors are increasingly used to treat renal anemia. Ischemic stroke is a rare severe adverse event of HIF-PH inhibitor therapy, and its clinical characteristics have not been described to date. We report three cases of ischemic stroke during treatment with daprodustat, a HIF-PH inhibitor, for anemia associated with non-dialysis-dependent chronic kidney disease (CKD). In two patients, the hemoglobin level exceeded the target hemoglobin level of 13 g/dL for renal anemia. Two patients developed ischemic stroke within two months after the daprodustat administration. None of the three patients experienced a recurrence of ischemic stroke after daprodustat discontinuation. Daprodustat therapy is a risk factor for ischemic stroke, particularly during excessive elevation of hemoglobin levels or the early phases of treatment. Daprodustat should be discontinued to mitigate the risk of ischemic stroke recurrence., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Uchio et al.)

A 40-year-old woman with neuromyelitis optica spectrum disorder (NMOSD) and anti-aquaporin 4 antibodies suffered three NMOSD episodes between 35 and 37 years of age. Despite treatment with prednisolone and azathioprine, her condition repeatedly relapsed. We introduced satralizumab, targeting interleukin-6 receptors, which stabilized her condition. At the age of 38, she became pregnant and delivered a healthy baby at 38 weeks. Post delivery, both mother and child stayed healthy with no NMOSD relapses. This case illustrates the efficacy and safety of satralizumab in managing NMOSD, especially for women in their reproductive years who are planning pregnancy., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Nakashima et al.)

Gastrointestinal manifestations are a very rare complication of dermatomyositis (DM) and are much less frequent in adult cases than in juvenile cases. Only a few previous papers have reported adult patients who had DM with anti-nuclear matrix protein 2 (anti-NXP2) antibodies and who developed gastrointestinal ulcers. Herein, we report a similar case of a 50-year-old man who had DM with anti-NXP2 antibodies followed by relapsing multiple gastrointestinal ulcers. Even after the administration of prednisolone, his muscle weakness and myalgia deteriorated and gastrointestinal ulcers relapsed. In contrast, intravenous immunoglobulin and azathioprine improved his muscle weakness and gastrointestinal ulcers. Based on the parallel disease activity of the muscular and gastrointestinal symptoms, we considered that his gastrointestinal ulcers were a complication of DM with anti-NXP2 antibodies. We also propose that early intensive immunosuppressive therapy would be required for the muscular and gastrointestinal symptoms in DM with anti-NXP2 antibodies., (© 2023 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.)

Guillain-Barré syndrome (GBS) usually develops after preceding infection, but cardiac surgery can also occasionally cause GBS. Currently, cardiac catheterizations have already become common therapeutic options for heart diseases, but there have been no reports of GBS occurrence after that. Herein, we present a rare case in which GBS occurred following catheterization. An 85-year-old-man with sudden onset chest pain was rushed to our hospital and diagnosed with ST-elevated myocardial infarction. He underwent emergent percutaneous coronary intervention (PCI) to left anterior descending artery, but he still had exertional chest pain. Echocardiography revealed severe aortic stenosis (AS) and our heart team considered AS was the cause of symptom and decided to perform and transcatheter aortic valve implantation (TAVI), 11 days after the PCI. However, 5 days after the TAVI procedure, he presented with symmetrical muscular weakness of extremities. Cranial magnetic resonance imaging showed no significant lesion. Based on several signs including albuminocytologic dissociation in cerebrospinal fluid examination, demyelinating polyneuropathy in nerve conduction study, positive anti-ganglioside antibody, and the lack of preceding infection, he was diagnosed with GBS triggered by cardiac catheterizations. We administered high-dose intravenous immunoglobulin therapy and his motor strength gradually improved, finally discharged with full motor strength after 7 months rehabilitation., Learning Objective: •Cardiac surgery has been already reported as a non-infectious risk factor of Guillain-Barré syndrome (GBS) in previous literatures, and cardiac catheterization such as percutaneous coronary intervention and transcatheter aortic valve implantation, which were relatively less invasive procedure, may be a potential risk factor for GBS occurrence as well.•If a patient complains of progressive, symmetrical neurological symptoms after cardiac catheterization, GBS should be considered as the possible cause, and nerve conduction study and cerebrospinal fluid examination may be helpful for the diagnosis., Competing Interests: Dr. Asami is a clinical proctor for Medtronic and he reports having received remuneration from Medtronic, Edwards Lifesciences, Boston Scientific, Abbott Medical, and Canon Medical Systems. Dr. Tanabe is a clinical proctor for Edwards Lifesciences and he receives remuneration from Medtronic, Edwards Lifesciences, Boston Scientific, Abbott Medical, Japan Lifeline, and Orbusneich., (© 2023 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.)

Competing Interests: None

Orbital intravascular lymphoma is rare and typically of B-cell lineage. In this study, we report a patient who developed orbital lesions of intravascular natural killer/T-cell lymphoma (IVNKL), an extremely rare lymphoma. An 88-year-old man presented with rapidly progressive right vision loss and double vision. A neurological examination revealed that he had decreased visual acuity and severe oculomotor impairment in the right eye. Magnetic resonance imaging showed right-dominant, nonmass lesions in both orbits. No lesions were found in the lymph nodes, skin, or brain. The patient received immunosuppressive and antifungal therapy, but his clinical condition rapidly deteriorated, and he died of multiple organ failure. Autopsy revealed natural killer/T-cell lymphoma proliferation within the lumina of small blood vessels in multiple organs, including the ocular adnexa of the right orbit. These findings show that he was ultimately diagnosed with IVNKL. IVNKL could initially cause ocular symptoms due to the involvement of the ocular adnexa. Ocular involvements have not been described previously. Even if patients initially present with only ocular symptoms, IVNKL should be considered., Competing Interests: None, (Copyright © Japan Medical Association.)

Laryngeal metastasis is an extremely rare condition. To the best of our knowledge, there has been no previous report on a laryngeal metastasis from renal cell carcinoma, which describes on details of the CT and MR imaging findings. A male patient in his 80s. Laryngoscopy revealed reddish-colored masses in the right false vocal cord and in the subglottic larynx. CT and MR imaging of this case showed multiple hypervascularized lesions with a wash-out effect in the supra and subglottis of the larynx and in the right intervertebral foramen of the cervical spine. Angiography revealed a hypervascular tumor consistent with the subglottic lesion. The histopathology and immunohistochemistry findings were compatible with laryngeal metastasis from renal clear cell carcinoma. A history of postoperative renal clear cell carcinoma about 7 years ago was later confirmed, which was not stated at the time of the initial imaging evaluation. It is a possible differential diagnosis in cases of multiple hypervascular masses in the head and neck region with a history of renal carcinoma. In particular, if the contrast-enhancement pattern of the lesion on the dynamic CT is similar to that of renal cell carcinoma. It is also important to reconfirm the patient's medical history, including postoperative status., (© 2021 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Cases of myasthenia gravis with inflammatory myopathy usually show elevated creatine kinase (CK) levels. There are few case reports of myasthenia gravis with inflammatory myopathy without elevated CK levels, and clinical features and useful diagnostic methods for these patients are little known. We describe the case of a 79-year-old man with myasthenia gravis that was complicated with inflammatory myopathy without elevated CK levels and successfully treated with immunological treatment. Initially, he was diagnosed with ocular myasthenia gravis and treated with pyridostigmine, but dysphagia and weakness in the neck and bilateral upper limb without fatigability gradually developed. Needle electromyography revealed myopathic changes, and the results of muscle biopsy were consistent with inflammatory myopathy. Blood tests showed normal CK levels throughout the clinical course and elevated myoglobin levels alone. The possibility of developing inflammatory myopathy in patients with myasthenia gravis cannot be excluded, even if CK levels are normal., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Interhemispheric subdural hematoma (ISDH) is a rare subtype of subdural hematoma. We report the case of an 81-year-old woman on hemodialysis with sudden nausea and vomiting. A computed tomography (CT) scan of the brain showed a bilaterally symmetrical increase in the thickness and density of the falx cerebri. At first, the findings were overlooked, but were later identified as an acute ISDH. The patient was treated conservatively and the symptoms completely resolved. The possibility of ISDH should be considered even if CT images of the brain are symmetrical., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Uchio et al.)

The cortical silent period (CSP) induced by transcranial magnetic stimulation (TMS) has been reported to be prolonged in 2 Creutzfeldt-Jakob disease (CJD) patients who presented with periodic myoclonus. Herein, we will show a prominent prolongation of TMS-induced CSP in a patient with CJD who did not have periodic myoclonus. The patient was a 66-year-old woman who developed rapidly progressive dementia. No myoclonic jerks were observed. Brain magnetic resonance imaging showed high-intensity lesions in the cerebral cortex, basal ganglia, and thalamus on diffusion-weighted images. Electroencephalography (EEG) showed diffuse and continuous slow waves, but no periodic synchronous discharges (PSDs). A TMS study revealed that the duration of CSP was prominently prolonged: the duration of CSP (370 ms) equaled that of the mean + 6.5 SD of the normal value. One month after admission, the patient exhibited akinetic mutism and developed periodic myoclonus in her limbs. The clinical course was compatible with CJD. To date, CSP has been measured in only 2 CJD patients. The common findings in both cases were marked prolongation of CSP, periodic myoclonus, and PSD on EEG. In short, we demonstrated that TMS-induced CSP was prominently prolonged even at the early stage of CJD without periodic myoclonus or PSD. In other disorders, the CSP has not been reported to be comparably prolonged to that of CJD patients. Therefore, we conclude that TMS-induced CSP could be prominently prolonged even in the early stage of CJD. The marked prolongation of the CSP might be an early biomarker of CJD., Competing Interests: The authors have no disclosures. The authors have no potential conflicts of interest., (Copyright © 2020 by S. Karger AG, Basel.)

Background: Cases of exacerbation of pre-existing neuromuscular diseases induced by immune checkpoint inhibitors (ICIs) have rarely been reported because patients with autoimmune diseases have generally been excluded from ICI therapy due to the increased risk of exacerbation. We describe the first case of an elderly patient who experienced exacerbation of a previously undiagnosed sporadic inclusion body myositis (sIBM), the most common myopathy in the geriatric population, which was triggered by anti-programmed cell death-1 therapy., Case Presentation: A 75-year-old man who was receiving pembrolizumab presented with limb weakness. Three years prior, he had noticed slowly progressive limb weakness, but he received no diagnosis. After the first infusion of pembrolizumab, his creatine kinase (CK) levels had increased. The neurological examination and muscle biopsy findings confirmed the diagnosis of sIBM and suggested exacerbation of sIBM induced by pembrolizumab. After the patient's CK levels decreased, pembrolizumab was restarted. The tumor progressed after its treatment with pembrolizumab. The patient died after 15 months of follow-up., Conclusions: In patients with slowly progressive limb weakness, sIBM should be explored before ICI therapy. In addition, if patients show high CK levels after ICI introduction, it is necessary to confirm whether they have sIBM in order to avoid unnecessary immunosuppressive therapies and assess whether they can tolerate ICI reintroduction., Competing Interests: Competing interestsThe authors declare that they have no competing interests., (© The Author(s) 2020.)

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome has previously been reported in only 2 patients with progressive supranuclear palsy (PSP). Herein, we report a third case of WEBINO syndrome with PSP. The patient was an 81-year-old man who had experienced gradually increasing gait disturbance and occasional falls since the age of 78 years. At 80 years of age, he presented with cognitive impairment, parkinsonism, and oculomotor abnormalities. The oculomotor abnormalities consisted of vertical gaze palsy and loss of eye convergence. Brain magnetic resonance imaging demonstrated marked atrophy of the midbrain. He was diagnosed with PSP. At the age of 81 years, he presented with alternating extropia in his forward gaze and adduction paresis and outward nystagmus of the abducted eye in his horizontal gaze, both of which were compatible with WEBINO syndrome. Previously, we reported the first case of PSP with WEBINO syndrome, and another group recently reported a second case. In light of the previous cases and the present case, WEBINO syndrome in PSP should not be considered extremely rare. Furthermore, WEBINO syndrome has not been reported in other neurodegenerative disorders, which suggests that it might be a useful and specific diagnostic finding in PSP., Competing Interests: The authors have no disclosures. The authors have no potential conflicts of interest.