Impaired Glymphatic and Meningeal Lymphatic Functions in Patients with Chronic Migraine.

OBJECTIVE: This study was undertaken to investigate migraine glymphatic and meningeal lymphatic vessel (mLV) functions. METHODS: Migraine patients and healthy controls (HCs) were prospectively recruited between 2020 and 2023. Diffusion tensor image analysis along the perivascular space (DTI-ALPS) index for glymphatics and dynamic contrast-enhanced magnetic resonance imaging parameters (time to peak [TTP]/enhancement integral [EI]/mean time to enhance [MTE]) for para-superior sagittal (paraSSS)-mLV or paratransverse sinus (paraTS)-mLV in episodic migraine (EM), chronic migraine (CM), and CM with and without medication-overuse headache (MOH) were analyzed. DTI-ALPS correlations with clinical parameters (migraine severity [numeric rating scale]/disability [Migraine Disability Assessment (MIDAS)]/bodily pain [Widespread Pain Index]/sleep quality [Pittsburgh Sleep Quality Index (PSQI)]) were examined. RESULTS: In total, 175 subjects (112 migraine + 63 HCs) were investigated. DTI-ALPS values were lower in CM (median [interquartile range] = 0.64 [0.12]) than in EM (0.71 [0.13], p = 0.005) and HCs (0.71 [0.09], p = 0.004). CM with MOH (0.63 [0.07]) had lower DTI-ALPS values than CM without MOH (0.73 [0.12], p < 0.001). Furthermore, CM had longer TTP (paraSSS-mLV: 55.8 [12.9] vs 40.0 [7.6], p < 0.001; paraTS-mLV: 51.2 [8.1] vs 44.0 [3.3], p = 0.002), EI (paraSSS-mLV: 45.5 [42.0] vs 16.1 [9.2], p < 0.001), and MTE (paraSSS-mLV: 253.7 [6.7] vs 248.4 [13.8], p < 0.001; paraTS-mLV: 252.0 [6.2] vs 249.7 [1.2], p < 0.001) than EM patients. The MIDAS (p = 0.002) and PSQI (p = 0.002) were negatively correlated with DTI-ALPS index after Bonferroni corrections (p < q = 0.01). INTERPRETATION: CM patients, particularly those with MOH, have glymphatic and meningeal lymphatic dysfunctions, which are highly clinically relevant and may implicate pathogenesis for migraine chronification. ANN NEUROL 2024;95:583-595.

Magnetic resonance radiomics-derived sphericity correlates with seizure in brain arteriovenous malformations.

OBJECTIVES: Angioarchitectural analysis of brain arteriovenous malformations (BAVMs) is qualitative and subject to interpretation. This study quantified the morphology of and signal changes in the nidal and perinidal areas by using MR radiomics and compared the performance of MR radiomics and angioarchitectural analysis in detecting epileptic BAVMs. MATERIALS AND METHODS: From 2010 to 2020, a total of 111 patients with supratentorial BAVMs were retrospectively included and grouped in accordance with the initial presentation of seizure. Patients' angiograms and MR imaging results were analyzed to determine the corresponding angioarchitecture. The BAVM nidus was contoured on time-of-flight MR angiography images. The perinidal brain parenchyma was contoured on T2-weighted images, followed by radiomic analysis. Logistic regression analysis was performed to determine the independent risk factors for seizure. ROC curve analysis, decision curve analysis (DCA), and calibration curve were performed to compare the performance of angioarchitecture-based and radiomics-based models in diagnosing epileptic BAVMs. RESULTS: In multivariate analyses, low sphericity (OR: 2012.07, p = .04) and angiogenesis (OR: 5.30, p = .01) were independently associated with a high risk of seizure after adjustment for age, sex, temporal location, and nidal volume. The AUC for the angioarchitecture-based, MR radiomics-based, and combined models was 0.672, 0.817, and 0.794, respectively. DCA confirmed the clinical utility of the MR radiomics-based and combined models. CONCLUSIONS: Low nidal sphericity and angiogenesis were associated with high seizure risk in patients with BAVMs. MR radiomics-derived tools may be used for noninvasive and objective measurement for evaluating the risk of seizure due to BAVM. CLINICAL RELEVANCE STATEMENT: Low nidal sphericity was associated with high seizure risk in patients with brain arteriovenous malformation and MR radiomics may be used as a noninvasive and objective measurement method for evaluating seizure risk in patients with brain arteriovenous malformation. KEY POINTS: • Low nidal sphericity was associated with high seizure risk in patients with brain arteriovenous malformation. • The performance of MR radiomics in detecting epileptic brain arteriovenous malformations was more satisfactory than that of angioarchitectural analysis. • MR radiomics may be used as a noninvasive and objective measurement method for evaluating seizure risk in patients with brain arteriovenous malformation.

Endovascular Management of Post-Irradiated Carotid Blowout Syndrome in Patients with Lower Neck Cancers.

OBJECTIVE: Lower neck cancers (LNCs) include specific tumour types and have some different vascular supply or collaterals from other head and neck cancers. This prospective study evaluated the outcome of endovascular management of post-irradiated carotid blowout syndrome (PCBS) in patients with LNC by comparing reconstructive management (RE) and deconstructive management (DE). METHODS: This was a single centre, prospective cohort study. Patients with LNC complicated by PCBS between 2015 and 2021 were enrolled for RE or DE. RE was performed by stent graft placement covering the pathological lesion and preventive external carotid artery (ECA) embolisation without balloon test occlusion (BTO). DE was performed after successful BTO by permanent coil or adhesive agent embolisation of the internal carotid artery (ICA) and ECA to common carotid artery, or ICA occlusion alone if the pathological lesion was ICA only. Cross occlusion included the proximal and distal ends of the pathological lesion in all patients. Re-bleeding events, haemostatic period, and neurological complications were evaluated. RESULTS: Fifty-nine patients (mean age 58.5 years; 56 male) were enrolled, including 28 patients undergoing RE and 31 patients undergoing DE. Three patients originally grouped to DE were transferred to RE owing to failed BTO. The results of RE vs. DE were as follows: rebleeding events, 13/28 (46%) vs. 10/31 (32%) (p = .27); haemostatic period, 9.4 ± 14.0 months vs. 14.2 ± 27.8 months (p = .59); neurological complication, 4/28 (14%) vs. 5/31 (16%) (p = .84); and survival time, 11.8 ± 14.6 months vs. 15.1 ± 27.5 months (p = .61). CONCLUSION: No difference in rebleeding risk or neurological complications was observed between the DE and RE groups. RE could be used as a potential routine treatment for PCBS in patients with LNC.

Percutaneous transluminal angioplasty and stenting of post-irradiated stenosis of subclavian artery: A matched case-control study.

BACKGROUND: Although radiotherapy is common for head/neck and chest cancers (HNCC), it can result in post-irradiation stenosis of the subclavian artery (PISSA). The efficacy of percutaneous transluminal angioplasty and stenting (PTAS) to treat severe PISSA is not well-clarified. AIMS: To compare the technical safety and outcomes of PTAS between patients with severe PISSA (RT group) and radiation-naïve counterparts (non-RT group). METHODS: During 2000 and 2021, we retrospectively enrolled patients with severe symptomatic stenosis (>60%) of the subclavian artery who underwent PTAS. The rate of new recent vertebrobasilar ischaemic lesions (NRVBIL), diagnosed on diffusion-weight imaging (DWI) within 24 h of postprocedural brain MRI; symptom relief; and long-term stent patency were compared between the two groups. RESULTS: Technical success was achieved in all 61 patients in the two groups. Compared with the non-RT group (44 cases, 44 lesions), the RT group (17 cases, 18 lesions) had longer stenoses (22.1 vs 11.1 mm, P = 0.003), more ulcerative plaques (38.9% vs 9.1%, P = 0.010), and more medial- or distal-segment stenoses (44.4% vs 9.1%, P<0.001). The technical safety and outcome between the non-RT group and the RT group were NRVBIL on DWI of periprocedural brain MRI 30.0% vs 23.1%, P = 0.727; symptom recurrence rate (mean follow-up 67.1 ± 50.0 months) 2.3% vs 11.8%, P = 0.185; and significant in-stent restenosis rate (>50%) 2.3% vs 11.1%, P = 0.200. CONCLUSION: The technical safety and outcome of PTAS for PISSA were not inferior to those of radiation-naïve counterparts. PTAS for PISSA is an effective treatment for medically refractory ischaemic symptoms of HNCC patients with PISSA.

Noninvasive investigations of human glymphatic dynamics in a diseased model.

OBJECTIVES: To explore human glymphatic dynamics in a diseased model via a noninvasive technique. METHODS: Patients with reversible vasoconstriction syndrome (RCVS) presenting with blood-brain barrier disruption, i.e., para-arterial gadolinium leakage on 3-T 3-dimensional isotropic contrast-enhanced T2-fluid-attenuated inversion recovery (CE-T2-FLAIR) magnetic resonance imaging (MRI), were prospectively enrolled. Consecutive 9-min-CE-T2-FLAIR for 5-6 times (early panel) after intravenous gadolinium-based contrast agent (GBCA) administration and one time-varying deferred scan of noncontrast T2-FLAIR (delayed panel) were performed. In Bundle 1, we measured the calibrated signal intensities (cSIs) of 10 different anatomical locations. In Bundle 2, brain-wide measurements of para-arterial glymphatic volumes, means, and medians of the signal intensities were conducted. We defined mean (mCoIs) or median (mnCoIs) concentration indices as products of volumes and signal intensities. RESULTS: Eleven subjects were analyzed. The cSIs demonstrated early increase (9 min) in perineural spaces: (cranial nerve [CN] V, p = 0.008; CN VII + VII, p = 0.003), choroid plexus (p = 0.003), white matter (p = 0.004) and parasagittal dura (p = 0.004). The volumes, mCoIs, and mnCoIs demonstrated increasing rates of enhancement after 9 to 18 min and decreasing rates after 45 to 54 min. The GBCA was transported centrifugally and completely removed within 961-1086 min after administration. CONCLUSIONS: The exogenous GBCA leaked into the para-arterial glymphatics could be completely cleared around 961 to 1086 min after administration in a human model of BBB disruption. The tracer enhancement started variously in different intracranial regions but was eventually cleared centrifugally to brain convexity, probably towards glymphatic-meningeal lymphatics exits. CLINICAL RELEVANCE STATEMENT: Glymphatic clearance time intervals and the centrifugal directions assessed by a noninvasive approach may have implications for clinical glymphatic evaluation in the near future. KEY POINTS: • This study aimed to investigate the human glymphatic dynamics in a noninvasive diseased model. • The intracranial MR-detectable gadolinium-based contrast agents were removed centrifugally within 961 to 1086 min. • The glymphatic dynamics was demonstrable by enhancing MRI in an in vivo diseased model noninvasively.

Clinical and imaging findings for the evaluation of large Rathke's cleft cysts and cystic craniopharyngiomas.

PURPOSE: Large Rathke's cleft cysts (LRCCs) and cystic craniopharyngiomas (CCPs) arise from the same embryological origin and may have similar MR presentations. However, the two tumors have different management strategies and outcomes. This study was designed to evaluate the clinical and imaging findings of LRCCs and CCPs, aiming to evaluate their pretreatment diagnosis and outcomes. METHODS: We retrospectively enrolled 20 patients with LRCCs and 25 patients with CCPs. Both tumors had a maximal diameter of more than 20 mm. We evaluated the patients' clinical and MR imaging findings, including symptoms, management strategies, outcomes, anatomic growth patterns and signal changes. RESULTS: The age of onset for LRCCs versus CCPs was 49.0 ± 16.8 versus 34.2 ± 22.2 years (p = .022); the following outcomes were observed for LRCCs versus CCPs: (1) postoperative diabetes insipidus: 6/20 (30%) versus 17/25 (68%) (p = .006); and (2) posttreatment recurrence: 2/20 (10%) versus 10/25 (40%) (p = .025). The following MR findings were observed for LRCCs versus CCPs: (1) solid component: 7/20 (35%) versus 21/25 (84%) (p = .001); (2) thick cyst wall: 2/20 (10%) versus 12/25 (48%) (p = .009); (3) intracystic septation: 1/20 (5%) versus 8/25 (32%) (p = .030); (4) snowman shape: 18/20 (90%) versus 1/25 (4%) (p < .001); (5) off-midline extension: 0/0 (0%) versus 10/25 (40%) (p = .001); and (6) oblique angle of the sagittal long axis of the tumor: 89.9° versus 107.1° (p = .001). CONCLUSIONS: LRCCs can be differentiated from CCPs based on their clinical and imaging findings, especially their specific anatomical growth patterns. We suggest using the pretreatment diagnosis to select the appropriate surgical approach and thus improve the clinical outcome.

Advances in Boron Neutron Capture Therapy (BNCT) for Recurrent Intracranial Meningioma.

Meningiomas are the most frequently diagnosed primary intracranial tumors in adults. Surgical resection is preferred if the meningioma is accessible; for those that are not suitable for surgical resection, radiotherapy should be considered to improve local tumor control. However, recurrent meningiomas are challenging to treat, as the recurrent tumor might be located in the previously irradiated area. Boron Neutron Capture Therapy (BNCT) is a highly selective radiotherapy modality in which the cytotoxic effect focuses mainly on cells with increased uptake of boron-containing drugs. In this article, we describe four patients with recurrent meningiomas treated with BNCT in Taiwan. The mean boron-containing drug tumor-to-normal tissue uptake ratio was 4.125, and the tumor mean dose was 29.414 GyE, received via BNCT. The treatment response showed two stable diseases, one partial response, and one complete response. We also introduce and support the effectiveness and safety of BNCT as an alternative salvage treatment for recurrent meningiomas.

Intracranial Vertebrobasilar Artery Dissection with Silent Rapid Progression.

PURPOSE: Intracranial vertebrobasilar artery dissection (iVBD) is a potentially lethal disease, and progression of the dissected vessels is not uncommon. Our report is aimed at providing further clinical experience of the timing of follow-up vascular imaging or endovascular intervention in iVBD patients. CASE REPORT: We report a case of iVBD with silent rapid progression. The 48-year-old woman presented as transient right limbs weakness. Brain MRI showed a small acute infarct over the left cerebellum, and MRA revealed a short segment of dissection over the left distal vertebral artery extending to proximal basilar artery. With no new clinical symptoms and signs, follow-up of vascular imaging within 1 week showed progressive critical narrowing of the dissected vertebrobasilar arteries. The blood flow of the vertebrobasilar system was restored by endovascular stenting. CONCLUSION: iVBD might progress without clinical manifestations. Early follow-up of vascular imaging should be considered in the patients with high risk for progression.

Quantified flow and angioarchitecture show similar associations with hemorrhagic presentation of brain arteriovenous malformations.

INTRODUCTION: The purpose of our study was to elucidate the impact of brain arteriovenous malformation (BAVM) flow and wall shear stress (WSS) on angioarchitecture and to evaluate their association with hemorrhagic presentations. MATERIALS AND METHODS: Forty-one patients with BAVMs were evaluated by phase-contrast MR angiography. Volume flow rate and WSS were quantified. Angioarchitectural features such as location, angiogenesis, venous stenosis, venous ectasia, venous phlebitis, venous rerouting, exclusive deep vein and venous sac were evaluated by two neuroradiologists. The correlation between BAVM flow and size was evaluated with Spearman correlation coefficients. Differences of size, flow, and WSS between the hemorrhagic and non-hemorrhagic groups, the seizure and non-seizure groups, and between the different groups based on angioarchitecture were evaluated with Mann-Whitney U tests. Accuracy in predicting hemorrhage was evaluated with receiver operating characteristic curves. RESULT: BAVM flow was highly correlated with volume (ρ = 0.77). Higher flow was more commonly associated with angiogenesis, venous ectasia, venous rerouting, and venous phlebitis. Flow and angioarchitecture showed similar efficacy in differentiating hemorrhagic from non-hemorrhagic BAVMs. WSS did not demonstrate differences across any clinical groups. CONCLUSION: Flow quantification and angioarchitecture analysis of BAVMs showed similar efficacy as evaluated by associations with hemorrhagic presentation. High flow affects both arterial and venous angioarchitecture, reflecting the nature of low vascular resistance in BAVMs.

Percutaneous transluminal angioplasty and stenting of post-irradiation stenosis of the vertebral artery.

BACKGROUND: The outcomes of percutaneous transluminal angioplasty and stenting (PTAS) in patients with medically refractory post-irradiation stenosis of the vertebral artery (PISVA) have not been clarified. AIM: This retrospective study evaluated the safety and outcomes of PTAS in patients with severe PISVA compared with their radiation-naïve counterparts (non-RT group). METHODS: Patients with medically refractory severe symptomatic vertebral artery stenosis and undergoing PTAS between 2000 and 2021 were classified as the PISVA group or the non-RT group. The periprocedural neurological complications, periprocedural brain magnetic resonance imaging, the extent of symptom relief, and long-term stent patency were compared. RESULTS: As compared with the non-RT group (22 cases, 24 lesions), the PISVA group (10 cases, 10 lesions) was younger (62.0 ± 8.6 vs 72.4 ± 9.7 years, P = 0.006) and less frequently had hypertension (40.0% vs 86.4%, P = 0.013) and diabetes mellitus (10.0% vs 54.6%, P = 0.024). Periprocedural embolic infarction was not significantly different between the non-RT group and the PISVA group (37.5% vs 35.7%, P = 1.000). At a mean follow-up of 72.1 ± 58.7 (3-244) months, there was no significant between-group differences in the symptom recurrence rate (0.00% vs 4.55%, P = 1.000) and in-stent restenosis rate (10.0% vs 12.5%, P = 1.000). CONCLUSION: PTAS of severe medically refractory PISVA is effective in the management of vertebrobasilar ischemic symptoms in head and neck cancer patients. Technical safety and outcome of the procedure were like those features in radiation-naïve patients.

Influence of angioplasty and stenting on intracranial artery stenosis: preliminary results of high-resolution vessel wall imaging evaluation.

OBJECTIVES: To investigate whether the imaging changes on high-resolution vessel wall imaging (HR-VWI) in patients before and after percutaneous transluminal angioplasty and stenting (PTAS) contribute to predicting the clinical outcome. METHODS: The study included 24 severe intracranial artery stenosis (SICAS) patients undergoing PTAS with Wingspan Stent between 2018 and 2020 and had a 1-year follow-up. Three HR-VWI sessions (preprocedural, early [within 24 h], and delayed postprocedural [134.7 ± 27.1 days)]) in each subject were performed with 3-Tesla MRI. We evaluated periprocedural HR-VWI changes in patients with and without recurrent cerebral ischemic symptoms (RCIS) within 1-year follow-up. RESULTS: On CE-T1WI of the patients without RCIS, a significant decrease in enhanced area was observed on early postprocedural (0.04 ± 0.02 cm2, p = 0.001) and delayed postprocedural (0.04 ± 0.02 cm2; p = 0.001) HR-VWI compared to preprocedural (0.07 ± 0.02 cm2) HR-VWI. Patients with RCIS demonstrated no significant loss of enhanced area on CE-T1WI of early postprocedural HR-VWI (p = 0.180). Significant decreases in calibrated T1 signals were observed in both presence (1.77 ± 0.70 vs. 0.79 ± 0.52; p = 0.018) and absence (1.42 ± 0.62 vs. 0.83 ± 0.40; p = 0.001) of RCIS in early postprocedural HR-VWI. CONCLUSION: The preliminary results showed the presence of reduced contrast enhancement immediately after PTAS may indicate less recurrent stroke events within 1 year. Further studies are necessary to confirm the phenomena in a longer observation period. KEY POINTS: • Early postprocedural high-resolution vessel imaging (HR-VWI) within 24 h can effectively predict a 1-year outcome following intracranial stenting. • For stenotic lesions after stenting without reduced contrast enhancement on HR-VWI within 24 h may need closer clinical surveillance for potentially higher risk of stroke events within 1 year.

Long-term cognitive and multimodal imaging outcomes after carotid artery stenting vs intensive medication alone for severe asymptomatic carotid stenosis.

BACKGROUND: Severe carotid stenosis is associated with cognitive impairment, which may be attributed to asymptomatic microembolism and/or chronic hypoperfusion. We aim to evaluate the long-term cognitive and brain connectivity outcomes of carotid artery stenting (CAS) for asymptomatic ≥70% stenosis of the extracranial internal carotid artery (ICA). METHODS: We conducted a non-randomized controlled study to compare intensive medical therapy alone (Med) or in combination with carotid artery stenting for the composite vascular events, neuropsychological, and multimodal magnetic resonance perfusion imaging and diffusion tensor imaging outcomes. RESULTS: Sixty-nine patients were followed for a mean of 2.3 years (31 Med, 38 CAS) and 11 patients had composite vascular events of all-cause death, ischemic stroke, or myocardial infarction (6 Med vs 5 CAS). Forty-six asymptomatic subjects completed neuropsychological and multimodality imaging follow-ups (23 Med, 23 CAS). Compared to the Med group, the CAS group had a modest improvement of 12-item delayed verbal memory (8.9 ± 2.4 to 9.8 ± 2.7 vs 9.0 ± 2.1 to 8.9 ± 2.3, p = 0.04), but not in global cognition, attention or executive function, which was associated with increased structural connectivity of fractional anisotropy at the ipsilateral deep white matter. Importantly, the memory improvement was correlated with the perfusion increment at the ipsilateral middle cerebral artery territory. CONCLUSION: For asymptomatic extracranial carotid steno-occlusion, successful carotid revascularization in addition to intensive medical treatment may potentially benefit cognitive reserve and connectivity strength which are partly attributed to restoration of non-critical hypoperfusion.

Percutaneous transluminal angioplasty and stenting of post-irradiated stenosis of subclavian artery.

BACKGROUND/PURPOSE: The therapeutic efficacy of percutaneous transluminal angioplasty and stenting (PTAS) of post-irradiated stenosis of subclavian artery (PISSA) was not well clarified. This retrospective study was designed to evaluate the technical safety and outcome of the patients of severe symptomatic PISSA accepted PTAS. METHODS: Between 2000 and 2019, 16 cases with 17 lesions of symptomatic and medically refractory PISSA accepted PTAS were included. We evaluated their technical success, peri-procedural complications and diffusion-weight imaging (DWI) of brain magnetic resonance imaging (MRI), results of symptom relief, and long-term stent patency. RESULTS: The stenosis of the 17 stenotic lesions were 81.2 ± 11.1%. The most common symptom of the 16 patients was dizziness (14/16, 87.5%). All successfully accepted PTAS without neurological complication and had symptom relief after PTAS (17/17, 100%). Of the 12 patients accepted pre-procedural and early post-procedural MRI follow-up, 2 patients had an asymptomatic tiny acute embolic infarct in the territory of vertebrobasilar system. In a 51.9 ± 54.9 months follow-up, all patients had no severe restenosis and no recurrent vertebrobasilar ischemic symptoms. CONCLUSION: For patients with PISSA and medically refractory ischemic symptoms, PTAS can be an effective alternative management.

Application of Flat-Panel Volume Computed Tomography to Evaluate Cerebral Hemorrhage After Mechanical Thrombectomy of Acute Embolic Stroke of the Anterior Circulation.

OBJECTIVE: The aim of the study was to evaluate cerebral hemorrhage (CH) and contrast media leakage (CML or commonly synonymous with "contrast staining") differentiation on flat-panel volume computed tomography (FPVCT) after intra-arterial mechanical thrombectomy. METHODS: We evaluated patients with hyperattenuation on FPVCT after intra-arterial mechanical thrombectomy between 2018 and 2021 by multiple parameters on CT angiography, FPVCT, CT, and/or magnetic resonance imaging. RESULTS: The CH (n = 43) versus CML (n = 24) groups revealed: (1) regional anatomical characteristics (preserved and distorted): 7 of 43 (9.6%) and 36 of 43 (83.7%) versus 22 of 24 (91.7%) and 2 of 24 (8.3%, P < 0.001); (2) thrombus in proximal two-thirds versus distal one-thirds M1 segment of middle cerebral artery (preserved and distorted): 17 of 21 (81.0%) and 4 of 21 (19.0%) versus 5 of 11 (45.5%) and 6 of 11 (54.5%, P = 0.040); and (3) average density ratio: 1.83 ± 0.65 versus 1.35 ± 0.13 (P = 0.004). CONCLUSIONS: Contrast media leakage can be differentiated from CH by preserved regional anatomical characteristics and relatively low average density ratio on FPVCT. Patients with CML who have embolism in proximal two thirds of M1 segment are more likely to develop hyperattenuation with preserved regional anatomy.

Effect of early radiotherapy initiation and high-dose chemotherapy on the prognosis of pediatric atypical teratoid rhabdoid tumors in different age groups.

PURPOSE: The optimal treatment strategy for pediatric atypical teratoid rhabdoid tumor (ATRT) is inconclusive. This study evaluated the prognostic value of early radiotherapy (RT) and high-dose chemotherapy with autologous stem cell rescue (HDC/ASCR) in pediatric ATRT. METHODS: This pooled analysis included ATRT patients treated at our institution and from other studies who were identified by a search of the PubMed electronic database. The effect of patient demographics and treatment profiles on progression-free survival (PFS) and overall survival (OS) were analyzed using Cox regression. RESULTS: Overall, 34 patients from our institution and 436 patients from 35 published studies were included. In multivariable analysis, patients with gross total resection (GTR), early RT (time to RT interval < 2 months), and HDC/ASCR had both better PFS [hazard ratio (HR) 0.46, p[Formula: see text] 0.001; HR 0.64, p = 0.011; and HR 0.51, p = 0.005, respectively] and OS (HR 0.55, p = 0.002; HR 0.48, p = 0.004; and HR 0.42, p < 0.001, respectively). For patients aged < 3 years, both RT and HDC/ASCR were significant favorable factors for PFS (HR 0.32 and 0.46, respectively) and OS (HR 0.40 and 0.36, respectively), while early RT was not prognostic. For patients aged ≥ 3 years, early RT was significantly associated with better PFS (HR 0.51) and HDC/ASCR did not affect PFS, and neither was related to OS. CONCLUSION: Both early RT initiation and HDC/ASCR were important components in the treatment of pediatric ATRT. However, the optimal treatment strategies might differ by age.

A single-center study of treatment outcomes of pediatric basal ganglia germinoma in Taiwan.

PURPOSE: A basal ganglia (BG) germinoma is a rare tumor, and the optimal treatment remains unknown. We evaluated the clinical outcomes of treatment of BG germinoma in pediatric patients in Taiwan. METHODS: We retrospectively reviewed the medical records of 34 children with BG germinoma who were treated with radiotherapy (RT) at Taipei Veterans General Hospital between 1989 and 2016. The median follow-up time is 8.3 years (1.8-25.2 years). Survival was analyzed using the Kaplan-Meier estimate. Univariate Cox proportional-hazards models were used to identify the potential risk factors. RESULTS: Only four patients (11.8%) experienced recurrence and all successfully underwent salvage therapy. One patient (2.97%) died due to suspected radiotherapy (RT)-related sarcoma in the scalp. The 2-, 3-, and 5-year DFS rates were 91.2%, 88.2%, and 79.4%, respectively; the 2-, 3-, and 5-year OS rates were 97.1%, 94.1%, and 82.4%, respectively. Focal RT showed low DFS in the Kaplan-Meier survival curves (P = .028) compared with non-focal RT (whole ventricle, whole brain, or cranial spinal area). In the univariate Cox proportional-hazards model, there was a significant difference in DFS between focal and non-focal RT (P = .03). There is no difference in DFS and OS between BG germinoma patients and non-BG germinoma patients. CONCLUSIONS: We found an excellent DFS and OS in pediatric patients with BG germinoma treated with RT. Whole ventricle irradiation is recommended for good tumor control and low treatment-related toxicity. BG germinoma patients showed similar treatment results as germinoma patients in other common sites.

Characterization of Heterozygous HTRA1 Mutations in Taiwanese Patients With Cerebral Small Vessel Disease.

BACKGROUND AND PURPOSE: Homozygous and compound heterozygous mutations in the high temperature requirement serine peptidase A1 gene (HTRA1) cause cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy. However, heterozygous HTRA1 mutations were recently identified to be associated with autosomal dominant cerebral small vessel disease (SVD). The present study aims at investigating the clinical features, frequency, and spectrum of HTRA1 mutations in a Taiwanese cohort with SVD. METHODS: Mutational analyses of HTRA1 were performed by Sanger sequencing in 222 subjects, selected from a cohort of 337 unrelated patients with SVD after excluding those harboring a NOTCH3 mutation. The influence of these mutations on HTRA1 protease activities was characterized. RESULTS: Seven novel heterozygous mutations in HTRA1 were identified, including p.Gly120Asp, p.Ile179Asn, p.Ala182Profs*33, p.Ile256Thr, p.Gly276Ala, p.Gln289Ter, and p.Asn324Thr, and each was identified in 1 single index patient. All mutations significantly compromise the HTRA1 protease activities. For the 7 index cases and another 2 affected siblings carrying a heterozygous HTRA1 mutation, the common clinical presentations include lacunar infarction, intracerebral hemorrhage, cognitive decline, and spondylosis at the fifth to sixth decade of life. Among the 9 patients, 4 have psychiatric symptoms as delusion, depression, and compulsive behavior, 3 have leukoencephalopathy in anterior temporal poles, and 2 patients have alopecia. CONCLUSIONS: Heterozygous HTRA1 mutations account for 2.08% (7 of 337) of SVD in Taiwan. The clinical and neuroradiological features of HTRA1-related SVD and sporadic SVD are similar. These findings broaden the mutational spectrum of HTRA1 and highlight the pathogenic role of heterozygous HTRA1 mutations in SVD.

Verification of bleeding points in carotid blowout syndrome using guidewire manipulation.

PURPOSE: Carotid blowout syndrome (CBS) is a catastrophic complication of aggressive treatment of head and neck cancer. Early detection of bleeding points with embolization is a life-saving procedure; however, some bleeding points may be difficult to identify. Our aim was to determine whether guidewire manipulation (GWM) could be used to verify bleeding point locations in patients with CBS. METHODS: Of the 92 patients with CBS referred for embolization in a 5-year period, 14 men and one woman (mean age 58 years) had bleeding points at locations that could not be definitely determined. We used GWM to verify the presence of these bleeding points. We assessed the anatomy of the ruptured arteries, technical details of GWM, and the angiographic and clinical outcomes. RESULTS: Bleeding points were difficult to detect because of the presence of small arterial pouches (n = 6) or multiple small arterial pouches (n = 9) in the unilateral or bilateral carotid arteries. Bleeding point locations were accurately identified using GWM in the internal carotid artery (n = 7), carotid bulb (n = 4), or common carotid artery (n = 4). Balloon-assisted GWM was applied in one patient. Fiber coils (n = 15) and/or liquid adhesives (n = 2) were used to occlude the affected artery. Endovascular management was technically successful in all patients and resulted in immediate cessation of hemorrhage without recurrence in a mean 22-month clinical follow-up. CONCLUSION: GWM is a simple and effective method for verifying bleeding points in ruptured arteries and preventing erroneous occlusion by embolization.

Intra-Arterial Thrombolytic Therapy Is Not a Therapeutic Option for Filler-Related Central Retinal Artery Occlusion.

Cosmetic facial filler-related central retinal artery occlusion (CRAO) is a devastating complication of facial hyaluronic acid (HA) injection and can be managed by intra-arterial thrombolytic therapy (IATT). The authors report on a 20-year-old woman who developed unilateral CRAO due to facial HA injection and who, despite prompt IATT, lost vision. A review of the related literature found 14 other female patients who developed cosmetic facial filler-related CRAO and accepted IATT management. In no case was vision loss clinically improved. IATT is not an effective preventive treatment of dermal filler-associated CRAO. The authors suggest careful preprocedural patient selection to prevent this complication.

Vascular wall imaging in reversible cerebral vasoconstriction syndrome - a 3-T contrast-enhanced MRI study.

BACKGROUND: Limited histopathology studies have suggested that reversible cerebral vasoconstriction syndromes (RCVS) does not present with vascular wall inflammation. Previous vascular imaging studies have had inconsistent vascular wall enhancement findings in RCVS patients. The aim of this study was to determine whether absence of arterial wall pathology on imaging is a universal finding in patients with RCVS. METHODS: We recruited patients with RCVS from Taipei Veterans General Hospital prospectively from 2010 to 2012, with follow-up until 2017 (n = 48). We analyzed the characteristics of vascular wall enhancement in these patients without comparisons to a control group. All participants received vascular wall imaging by contrasted T1 fluid-attenuated inversion recovery with a 3-T magnetic resonance machine. The vascular wall enhancement was rated as marked, mild or absent. RESULTS: Of 48 patients with RCVS, 22 (45.8%) had vascular wall enhancement (5 marked and 17 mild). Demographics, clinical profiles, and cerebral artery flow velocities were similar across patients with versus without vascular wall enhancement, except that patients with vascular wall enhancement had fewer headache attacks than those without (p = 0.04). Follow-up imaging completed in 14 patients (median interval, 7 months) showed reduced enhancement in 9 patients, but persistent enhancement in 5. CONCLUSION: Almost half of our RCVS patients exhibited imaging enhancement of diseased vessels, and it was persistent for approximately a third of those patients with follow-up imaging. Both acute and persistent vascular wall enhancement may be unhelpful for differentiating RCVS from central nervous system vasculitis or subclinical atherosclerosis.