Nasr, Deena M.

Background and Purpose: The diagnosis of spontaneous spinal cord infarction (SCI) is limited by the lack of diagnostic biomarkers and MRI features that often overlap with those of other myelopathies, especially acute myelitis. We investigated whether the ratio between serum neurofilament light chain levels and MRI T2-lesion area (neurofilament light chain/area ratio—NAR) differentiates SCI from acute myelitis of similar severity. Methods: We retrospectively identified Mayo Clinic patients (January 1, 2000–December 31, 2019) with (1) SCI, (2) AQP4 (aquaporin 4)-IgG or MOG (myelin oligodendrocyte glycoprotein)-IgG-associated myelitis at disease clinical presentation, or (3) idiopathic transverse myelitis from a previously identified population-based cohort of patients seronegative for AQP4-IgG and MOG-IgG. Serum neurofilament light chain levels (pg/mL) were assessed at the Verona University (SIMOA, Quanterix) in a blinded fashion on available stored samples obtained ≤3 months from myelopathy presentation. For each patient, the largest spinal cord lesion area (mm 2 ) was manually outlined by 2 independent raters on sagittal T2-weighted MRI images, and the mean value was used to determine NAR (pg/[mL·mm 2 ]). Results: Forty-eight patients were included SCI, 20 (definite, 11; probable, 6; possible, 3); acute myelitis, 28 (AQP4-IgG-associated, 17; MOG-IgG-associated, 5; idiopathic transverse myelitis, 6). The median expanded disability status scale score (range) at myelopathy nadir were 7.75 (2–8.5) and 5.5 (2–8), respectively. Serum neurofilament light chain levels (median [range] pg/mL) in patients with SCI (188 [14.3–2793.4]) were significantly higher compared with patients with AQP4-IgG-associated myelitis (37 [0.8–6942.9]), MOG-IgG-associated myelitis (45.8 [4–283.8]), and idiopathic transverse myelitis (15.6 [0.9–217.8]); P =0.01. NAR showed the highest accuracy for identification of SCI versus acute myelitis with values ≥0.35 pg/(mL·mm 2 ) yielding 86% specificity and 95% sensitivity (area under the curve=0.93). The positive and negative likelihood ratios were 6.67 and 0.06, respectively. NAR remained independently associated with SCI after adjusting for age, gender, immunotherapy before sampling, and days from myelopathy symptoms onset to sampling ( P =0.0007). Conclusions: NAR is a novel and promising clinical biomarker for differentiation of SCI from acute myelitis.

Background and Purpose: Few community-based studies investigating young adult strokes exist. The aim of this study was to determine the diagnostic yield of head and neck CTA in evaluation of anterior circulation ischemic stroke in young adults. Materials and Methods: This retrospective review is limited to patients between the ages of 18-50 presenting to the Mayo Clinic Rochester emergency department or referred from smaller institutions in adjacent counties. Patients with posterior circulation infarcts, amaurosis fugax, central retinal artery occlusion or transient ischemic attacks were excluded. The presence of carotid dissection, webs, plaque, or other high-risk lesions were recorded. Results: A total of 136 patients met inclusion criteria. Mean degree of carotid stenosis ipsilateral to infarct was 14.1%, compared to 6.5% on the contralateral side (p = .006). Ipsilateral mean carotid wall thickness measured .79 mm, compared to contralateral thickness of .80 mm (p = .51). Ipsilateral low-density plaque was observed in 22 patients (16.2%), and 21 patients (15.4%) on the contralateral side (p = .87). Ipsilateral calcified plaque was observed in 37 patients (27.2%), and 29 patients (21.3%) on the contralateral side (p = .31). 20 (14.7%) ipsilateral dissections were noted, compared to 4 (2.9%) contralateral dissections (p = .001). 3 (2.2%) ipsilateral carotid webs were identified, compared to 0 contralateral webs (p = .25). Conclusion: Carotid dissection was the most common cause of anterior circulation infarct identified on cervical CTA in this population. Carotid webs and atherosclerosis were not common findings.

Background To assess if leukoaraiosis severity is associated with outcome in patients with acute ischemic stroke (AIS) following endovascular thrombectomy, and to propose a leukoaraiosis-related modification to the ASPECTS score. Methods A retrospective review was completed of AIS patients that underwent mechanical thrombectomy for anterior circulation large vessel occlusion. The primary outcome measure was 90-day mRS. A proposed Leukoaraiosis-ASPECTS (“L-ASPECTS”) was calculated by subtracting from the traditional ASPECT based on leukoaraiosis severity (1 point subtracted if mild, 2 if moderate, 3 if severe). L-ASEPCTS score performance was validated using a consecutive cohort of 75 AIS LVO patients. Results 174 patients were included in this retrospective analysis: average age: 68.0±9.1. 28 (16.1%) had no leukoaraiosis, 66 (37.9%) had mild, 62 (35.6%) had moderate, and 18 (10.3%) had severe. Leukoaraiosis severity was associated with worse 90-day mRS among all patients (P=0.0005). Both L-ASPECTS and ASPECTS were associated with poor outcomes, but the area under the curve (AUC) was higher with L-ASPECTS (P<0.0001 and AUC=0.7 for L-ASPECTS; P=0.04 and AUC=0.59 for ASPECTS). In the validation cohort, the AUC for L-ASPECTS was 0.79 while the AUC for ASPECTS was 0.70. Of patients that had successful reperfusion (mTICI 2b/3), the AUC for traditional ASPECTS in predicting good functional outcome was 0.80: AUC for L-ASPECTS was 0.89. Conclusions Leukoaraiosis severity on pre-mechanical thrombectomy NCCT is associated with worse 90-day outcome in patients with AIS following endovascular recanalization, and is an independent risk factor for worse outcomes. A proposed L-ASPECTS score had stronger association with outcome than the traditional ASPECTS score.

OBJECTIVESpinal epidural arteriovenous fistulas (SEDAVFs) constitute a rare but treatable cause of vascular myelopathy and are a different subtype from the more common Type I spinal dural AVFs. The purpose of this study was to review a consecutive series of SEDAVFs from a single institution and report on the clinical presentations, functional status, and treatment outcomes.METHODSThe authors identified all SEDAVFs treated at their institution from 2005 to 2015. SEDAVFs were defined as spinal AVFs in which the fistulous connection occurred in the epidural venous plexus. The clinical presentation, functional status, immediate treatment outcomes, and long-term neurological outcomes were analyzed.RESULTSTwenty-four patients with SEDAVFs were included in this study. The patients' mean age at presentation was 66.9 years. The most common presenting symptoms were pain and numbness (22 patients, 91.7%), followed by lower-extremity weakness (21 patients, 87.5%). The mean duration of symptoms prior to diagnosis was 11.8 months. Eighteen patients (75.0%) were treated with endovascular therapy alone, 4 (16.7) with surgery, and 2 (8.3%) with a combination of techniques. There was 1 major treatment-related complication (4.2%). Fifteen patients (62.5%) had improvement in disability, and 12 patients (54.5%) had improvement in sensory symptoms.CONCLUSIONSSEDAVFs often present with lower-extremity motor dysfunction and sensory symptoms. With the availability of newer liquid embolic agents, these lesions can be effectively treated with endovascular techniques. Surgery is also effective at treating these lesions, especially in situations where endovascular embolization fails or is not safe and in patients presenting with mass effect from compressive varices.

Internal carotid artery pseudoaneurysm is an uncommon complication of retropharyngeal and parapharyngeal abscess in children. Treatment of the pseudoaneurysm has evolved in recent years from surgical ligation to endovascular techniques. Neurologic sequelae most commonly consist of Horner’s syndrome with cerebral ischemia being uncommon. The clinical course of a 2-year-old boy with retropharyngeal abscess complicated by internal carotid artery pseudoaneurysm, is described and the literature is reviewed. A conventional angiogram confirmed the presence of a large pseudoaneurysm with no anterograde flow distal to the pseudoaneurysm and substantial collateral flow across the circle of Willis, with filling of the left anterior and middle cerebral arteries via the anterior and posterior communicating arteries. Endovascular occlusion resulted in nonfilling of the left internal carotid artery, pseudoaneurysm, and left internal jugular vein at the base of the skull. Following the procedure, the patient developed transient mild right hemiparesis associated with frontal lobe ischemia.

Background Although rare, spinal arteriovenous malformations (AVMs) are thought to be more prevalent in the hereditary Hereditary Hemorrhagic Telangiectasia (HHT) population. Methods We report a case of a spinal AVM in a 37-year-old female with HHT treated with endovascular embolization. In addition, we report findings from a systematic review of the literature on the clinical characteristics, angioarchitecture, and clinical outcomes of HHT patients with spinal AVMs. Results The patient is a 37 year-old female with definite HHT who presented with a one-year history of progressive gait difficulty. The spinal fistula was incidentally detected on chest computed tomography (CT). Spinal angiography demonstrated a large perimedullary arteriovenous fistula was supplied by a posterolateral spinal artery. The fistula was treated with detachable coils. The patient made a complete neurological recovery. Our systematic review yielded 25 additional cases of spinal AVMs in HHT patients. All fistulae were perimedullary (100.0%). Treatments were described in 24 of the 26 lesions. Endovascular-only treatment was performed in 16 cases (66.6%) and surgical-only treatment was performed in five cases (20.8%). Complete or near-complete occlusion rates were 86.7% (13/15) for endovascular treated cases, 100.0% (4/4) for surgery and 66.6% (2/3) for combined treatments. Overall, 80.0% of patients (16/20) reported improvement in function following treatment, 100.0% (5/5) in the surgery group and 84.6% (11/13) reported improvement in the endovascular group. Conclusions Spinal fistulae in HHT patients are usually type IV perimedullary fistulae. Both endovascular and surgical treatments appeared to be effective in treating these lesions. However, it is clear that endovascular therapy has become the preferred treatment modality.