Ohnishi Neurological Center

Background: Internal carotid artery dissection (ICAD) is an uncommon but important cause of ischemic stroke in young adults, particularly in Asian populations where its incidence is relatively low. While ICAD typically presents with headache, neck pain, or ischemic symptoms, it can also lead to isolated cranial nerve involvement, complicating the diagnostic process.Case Description: We report the case of a 45-year-old woman who presented with isolated right-sided glossopharyngeal nerve symptoms – specifically, ageusia and pharyngeal discomfort – along with mild posterior neck pain. Initial evaluation by otolaryngology failed to identify the etiology. Subsequent imaging at our hospital revealed a spontaneous dissection of the right internal carotid artery extending from the third cervical spinal cord level to the proximal petrous segment. Magnetic resonance angiography and digital subtraction angiography confirmed the diagnosis of ICAD. Conservative management with antiplatelet therapy (aspirin 100 mg/day) led to symptom improvement, and follow-up imaging after 180 days demonstrated near-complete resolution of the dissection.Conclusion: This case is noteworthy due to the absence of cerebral ischemic findings and the isolated involvement of the glossopharyngeal nerve, likely due to localized anterolateral expansion of the dissection cavity compressing the pharyngeal branch of the nerve. Although mild styloid process elongation was observed, there was no radiological evidence suggesting Eagle syndrome. This case emphasizes the importance of considering ICAD in the differential diagnosis of lower cranial nerve palsies, even in the absence of stroke or typical neurological signs.

Abstract Romidepsin is an important therapeutic option for patients with peripheral T-cell lymphoma (PTCL). However, the timing of romidepsin administration remains controversial. The objective of this study was to characterize the safety and efficacy of romidepsin as consolidation therapy after gemcitabine, dexamethasone, and cisplatin (GDP) therapy (GDPR). This study of patients treated between March 2019 and March 2021 was registered with the Japan Registry of Clinical Trials (registration number: jRCT0000000519). If complete response, partial response, or stable disease was confirmed after 2–4 GDP cycles, romidepsin was administered every 4 weeks until 1 year. The outcomes of patients participating in this prospective study (PTCL-GDPR) who were receiving GDPR between 2000 and 2015 before starting this trial were retrospectively reviewed. Seven patients with relapsed/refractory (R/R) PTCL [T-follicular helper phenotype (n = 1) and angioimmunoblastic T-cell lymphoma (AITL, n = 6)] were included in PTCL-GDPR. The outcomes of eight patients with R/R PTCL not otherwise specified and seven patients with AITL were retrospectively reviewed. After a median follow-up of 34, 63, and 65 months in patients in PTCL-GDPR and the retrospective cohorts with PTCL-NOS and AITL, respectively, the 2-year OS rates were 71%, 100%, and 100%, respectively, and the overall response rates after treatment were 57%, 100%, and 100%, respectively. Common adverse events in patients in PTCL-GDPR included hematological toxicities such as neutropenia, which improved with supportive treatment. There were no treatment-related mortalities. GDPR might be safe and effective in elderly transplant-ineligible patients with R/R PTCL, and further investigation is warranted.

Autologous stem cell transplantation (ASCT) remains an important therapeutic strategy for multiple myeloma; however, a proportion of patients fail to mobilize a sufficient number of peripheral blood stem cells (PBSCs) to proceed to ASCT. In the present study, we aimed to clarify the characteristics and outcomes of poor mobilizers. Clinical data on poorly mobilized patients who underwent PBSC harvest for almost 10 years were retrospectively collected from 44 institutions in the Japanese Society of Myeloma (JSM). Poor mobilizers were defined as patients with less than 2 × 106/kg of CD34+ cells harvested at the first mobilization. The proportion of poor mobilization was 15.1%. A sufficient dataset including overall survival (OS) was evaluable in 258 poor mobilizers. Overall, 92 out of 258 (35.7%) poor mobilizers did not subsequently undergo ASCT, mainly due to an insufficient number of PBSCs. Median OS from apheresis was longer for poor mobilizers who underwent ASCT than for those who did not (86.0 vs. 61.9 mon., p = 0.02). OS from the diagnosis of poor mobilizers who underwent ASCT in our cohort was similar to those who underwent ASCT in the JSM database (3y OS rate, 86.8% vs. 85.9%). In this cohort, one‐third of poor mobilizers who did not undergo ASCT had relatively poor survival. In contrast, the OS improved in poor mobilizers who underwent ASCT. However, the OS of extremely poor mobilizers was short irrespective of ASCT.

Background: Superselective shunt occlusion (SSSO) for cavernous sinus dural arteriovenous fistula (CSDAVF) avoids the risk of cranial nerve palsy, unlike entire sinus packing, but requires paying attention to recurrence. Distinguishing between true and paradoxical worsening of postoperative ophthalmic symptoms using a less-invasive modality is often difficult. Here, we report a case of true worsening of neuro-ophthalmic symptom by recurrent CSDAVF detected by venous-arterial spin labeling (ASL) on magnetic resonance imaging.Case Description: A 55-year-old woman with neither contributory medical history nor previous head trauma presented with neuro-ophthalmic symptoms and pulsatile tinnitus. Digital subtraction angiography (DSA) revealed CSDAVF with multiple shunted pouches. She underwent successful transvenous SSSO, but neuroophthalmic symptom worsened after SSSO and venous-ASL revealed increased signal intensity in the right superior orbital vein (SOV). DSA confirmed recurrent CSDAVF and additional transvenous embolization was performed. Neuro-ophthalmic symptoms and venous-ASL hyperintensity on SOV improved postoperatively.Conclusion: Venous-ASL is noninvasive and seems useful for detecting true worsening of neuro-ophthalmic symptoms of recurrent CSDAVF.

Several studies have demonstrated that the dynamic factor at the mobile segment affects the severity of myelopathy in patients with cervical ossification of the posterior longitudinal ligament (C-OPLL), and posterior decompression supplemented with posterior instrumented fusion at the mobile segment provides good neurological improvement. However, there have been few reports of changes in range of motion at the mobile segment (segmental ROM) after laminoplasty (LP). The aim of this study was thus to retrospectively investigate changes in segmental ROM after LP and the impacts of these changes on neurological improvement in patients with C-OPLL.A total of 51 consecutive patients who underwent LP for C-OPLL since May 2010 and were followed for at least 2 years after surgery were included in this study. Neurological status was assessed using the Japanese Orthopaedic Association (JOA) score before surgery and at 2-year follow-up. Segmental ROM at the responsible level for myelopathy was measured preoperatively and at 2-year follow-up using lateral flexion-extension radiographs of the cervical spine.The mean JOA score improved significantly from 10.7 points preoperatively to 13.5 points at 2 years after surgery (mean recovery rate, 45.0%). The mean segmental ROM decreased significantly from 6.5 degrees before surgery to 3.2 degrees at 2 years after surgery. In the good clinical outcome group (recovery rate of the JOA score ≥50%; n = 22), the mean segmental ROM decreased significantly from 5.8 degrees preoperatively to 3.0 degrees postoperatively. It also decreased significantly from 7.1 degrees to 3.4 degrees in the poor clinical outcome group (recovery rate of the JOA score <50%; n = 29).This study showed that segmental ROM was stabilized after LP in most patients with C-OPLL. Neither preoperative nor postoperative segmental ROM showed significant differences between the good and poor clinical outcome groups and neither a postoperative increase nor decrease of segmental ROM significantly affected the recovery rate of the JOA score.

Cosmetics are used to improve physical appearance, but the benefits may be limited to people without visual impairment. The importance of attractiveness among blind persons has not been assessed. We investigated the influence of makeup on brain activity of blind persons using functional magnetic resonance imaging (fMRI). Participants were 7 blind females (BFs) who learned to fully apply makeup and 9 mostly age-matched normally sighted females (NSFs). Brain activity was measured using fMRI before and after application of makeup and during a makeup image task in each state. In the default mode network at rest, there was no difference between the BFs and NSFs. However, a lateral visual network to the opposite side was observed in the NSFs, whereas no such network was noted in the BFs. A weak network was noted in the BFs in the occipital fusiform gyrus and temporal occipital fusiform cortex, and an extensive visual area network defect was noted. Also, activity after makeup application was significantly higher in the nucleus accumbens, pallidum, and hippocampus. Activity in the right middle cingulate gyrus, right cerebral white matter, and right anterior cingulate gyrus was higher before makeup in both BFs and NSFs, and the activity was significantly higher and more extensive in the BFs. In conclusion, applying makeup is a personally rewarding activity, even for BFs, as it strongly activates the reward system and the reward/memory system network, even in the absence of a visual area network.

We report a case of in-stent stenosis as a complication at 6 months after the deployment of Pipeline Flex. This case necessitated retreatment for parent artery occlusion. A 55-year-old woman with right-side visual disorder was referred to our hospital for the deployment of Pipeline Flex. Cerebral digital subtraction angiography (DSA) demonstrated a large right-side paraclinoid aneurysm in combination with severe internal carotid artery (ICA) stenosis just beyond the aneurysm. We deployed Pipeline Flex under general anesthesia. After deployment, we performed angioplasty through the Pipeline. Six months after deployment, this patient exhibited exacerbation of visual disorder. Follow-up DSA revealed in-stent stenosis at 6 months after the deployment of Pipeline Flex. Therefore, we performed parent artery occlusion. Right-side visual disorder was improved in this patient. If Pipeline is deployed for patients with ICA stenosis just beyond an aneurysm, we need to be aware of in-stent stenosis after deployment.