Citations

BACKGROUND AND OBJECTIVES: This study aimed to investigate the clinical presentation, natural history, and long-term outcome of sporadic cerebral cavernous malformations (CCMs) based on initial Zabramski classification. METHODS: A prospective cohort of 285 patients with sporadic CCMs was analyzed. Patients were classified into Zabramski Types I-IV based on diagnostic MRI. Clinical presentation, lesion size, location, and developmental venous anomaly presence were recorded. Prospective symptomatic hemorrhage (SH) (censored at first hemorrhage, surgery, or last follow-up) and functional outcomes were assessed using Kaplan-Meier and Cox regression analyses. Functional outcomes were measured with the modified Rankin Scale (mRS) at baseline, annually, and at the last follow-up. RESULTS: The cohort included 58.9% women and 41.1% men, with a mean age at diagnosis of 44.5 years. Zabramski Types I-IV (n = 113, 125, 40, and 7, respectively) differed significantly in clinical presentation ( P < .001). Type I lesions were symptomatic in 97.3%, Types II and III in 34.4% and 22.5%, respectively, while all Type IV lesions were asymptomatic. Type I lesions had the highest annual hemorrhage rate (13.9% per year) and a 5-year cumulative risk of 50.6%. Types II and III had lower rates (2.9% and 1.8%), whereas no hemorrhages occurred in Type IV lesions. At baseline, 70.8% of Type I patients had mRS ≥2, which decreased to 35.4% at the last follow-up. Type III lesions had favorable outcomes, with 7.5% of patients having mRS ≥2 at the last follow-up. Type IV lesions remained asymptomatic throughout. Severe SH significantly increased the odds of poor outcomes (mRS ≥3; P < .001), whereas Zabramski type was not predictive of outcomes after adjustment. CONCLUSION: Zabramski classification aids in stratifying hemorrhage risk and guiding management in CCMs. Severe SH is a critical determinant of functional outcomes, underscoring the need for comprehensive risk assessments and individualized patient care strategies.

Background There has been growing interest in transradial procedures over the past decade due to substantial reductions in access site complications as well as improved patient recovery. While several radial-specific neurovascular catheters have come to market, all catheters that are compatible with a 7Fr sheath have small inner diameters (IDs) (0.079″–0.082″), which can limit use of modern intermediate catheters. while larger-ID catheters cannot be used with a radial sheath resulting in complications related to radial artery spasm and entrapment. We report our results using a novel 7Fr-sheath-compatible radial guide catheter with an 0.087″ ID (Piraeus 7Fr 87 NeuGlide catheter) for neurovascular procedures. Methods This is a single center retrospective case series of patients undergoing transarterial transradial neuroendovascular procedures using the Piraeus 7Fr 87 NeuGlide catheter. The primary outcome was delivery of the catheter to the target vessel and successful completion of the procedure without the need to convert to femoral access or an alternative guide. Descriptive statistics are reported. Results Forty patients were included. The most common procedure was middle meningeal artery embolization followed by aneurysm embolization. And 97.5% of procedures were completed successfully without conversion to femoral access; 75% were completed without the need of an intermediate guide catheter. One procedure required conversion to femoral access. There were no access site or neurological complications. Conclusion The Piraeus 7Fr 87 NeuGlide catheter is both safe and effective for neurovascular procedures. The larger ID and 7Fr compatible outer diameter allows for the use of standard 5Fr and 6Fr intermediate/aspiration catheters.

BACKGROUND AND OBJECTIVES: Despite many publications about cavernous malformations (CMs), controversy remains regarding diagnostic and management strategies. To update evidence-based guidelines for the clinical management of brain and spinal cord CMs. METHODS: The Alliance to Cure CMs, the patient support group in the United States advocating on behalf of patients and research in CM, convened a multidisciplinary writing group comprising expert CM clinicians to help summarize the existing literature related to the clinical care of CM, focusing on 5 topics: (1) epidemiology and natural history, (2) genetic testing and counseling, (3) diagnostic criteria and imaging standards, (4) neurosurgical considerations, and (5) neurological considerations. Building on prior evidence-based recommendations reflecting literature review through October 2014, the group conducted a systematic review of the more recent literature, identified references for mandatory citation, rated evidence, developed recommendations, and established consensus according to a prespecified protocol. Finally, the writing group outlined remaining knowledge gaps and controversies to guide future research. RESULTS: From 2672 publications published between October 1, 2014, and March 15, 2023, and meeting key word criteria, 234 were selected based on prearticulated criteria for mandatory consideration in evidence-based recommendations. Topic authors used these and other supporting references to summarize current knowledge and arrive at 53 management recommendations, with unanimous consensus based on a Delphi process. These were rated by class (strength of recommendation) and level (quality of evidence) per the American Heart Association/American Stroke Association criteria. Eighteen recommendations were class 1 (34%), class 2 in 31 (58%), and class 3 in 4 (8%). Three were level A (6%), 19 (36%) were level B, and 31 (58%) were level C. CONCLUSION: Current evidence supports prior and new recommendations for the management of CMs, but many reflect moderate classes and low levels, mandating further research to better inform clinical practice.

Abstract Introduction Moyamoya is characterized by idiopathic, progressive, large intracranial arterial steno-occlusion, affecting the anterior circulation of the brain. Common presentations include strokes, seizures, and headaches, which have been associated with poor sleep quality and sleep disorders in the general population. However, to our knowledge, sleep quality has not been previously investigated in Moyamoya disease. The aim of this study was to characterize self-reported sleep quality, sleepiness, sleep disorder diagnoses in patients with Moyamoya syndrome, and to explore possible association between sleep disturbance and quality of life in these patients. Methods We analyzed consecutive patients with Moyamoya evaluated at our institution who completed the Pittsburgh Sleep Quality Index (PSQI) and the Epworth Sleepiness Scale (ESS). We also abstracted the Patient-Reported Outcomes Measurement Information System (PROMIS) to measure person-centered physical, mental and social wellbeing. Results We studied 23 patients with mean age 41.8 ± 12.1 years old having disease duration of 3 ± 4.4 years. 15 (65.2%) had ischemic strokes. Mean total PSQI score was 6.9 ± 2.9, and 17 (73.9%) had poor sleep quality (PSQI&gt;5). Mean sleep efficiency was reduced at 80 ± 17%. Although Median ESS was 6 (IQR 3.25 – 8), most patients (91.3%) reported at least mild daytime dysfunction (Component 7≥1). Mean PSQI component scores (0 – 3 severity, 3 is worse) were 1.29 ± 0.62 (subjective quality), 1.1 ± 0.73 (sleep latency), 1.04 ± 0.83 (sleep duration), 0.87 ± 1.01 (sleep efficiency), 1.22 ± 0.52 (sleep disturbances), 0.3 ± 0.88 (sleep medication), and 1.1 ± 0.52 (daytime dysfunction). The total PSQI score was positively correlated with PROMIS score (r=0.47, p=0.044). Only one patient was diagnosed with obstructive sleep apnea and three with chronic insomnia. Conclusion Poor sleep quality appears to be a frequent problem in patients with Moyamoya disease. Sleep disturbance severity was similar to previous findings in clinical stroke populations. The apparent mismatch between subjective sleep disturbance and sleep disorder diagnoses suggest that sleep disorders are likely underdiagnosed in Moyamoya patients. The relationship between PSQI and PROMIS scores suggests that sleep disturbance may significantly affect quality of life in these patients. Support (if any)

Background: Little is known about the association between intraplaque hemorrhage (IPH) and other features of high-risk carotid atherosclerotic plaques, such as the presence of plaque ulceration, plaque enhancement, and lipid-rich necrotic core (LRNC). This study set out to assess the relationship between IPH and other vulnerable plaque features. Materials and Methods: A retrospective review was done of 102 patients with IPH in one or both of the internal carotid arteries (ICA) on neck MRA between 1/1/2016 and 3/31/2021. IPH was defined as a ≥200% signal intensity of the adjacent sternocleidomastoid muscle on MPRAGE images. All ICA plaques were assessed for the presence or absence of IPH, plaque ulceration, plaque enhancement, and LRNC, as well as IPH volume. IPH volume was measured manually by outlining the region of interest. Results: A total of 102 patients were included, with 88 (86.3%) being male. The average age was 73.5 years (SD = 9.0). Both IPH and LRNC were more commonly seen in the left carotid artery ( p = .018 and p = .047, respectively). For right-sided ICAs, there was a significant association between IPH and LRNC ( p < .0001). Lesions without IPH were more likely to have plaque enhancement than lesions with IPH ( p = .04). For left-sided ICAs, there was also a significant association between the presence of IPH and LRNC ( p < .0001). Conclusions: There is a significant association between the presence of IPH and LRNC. An inverse relationship was found between the presence of IPH and plaque enhancement for right-sided plaques. No associations were found between IPH and plaque ulceration.

Background Flow-diverter stents (FDS) have become the standard of care for a wide range of intracranial aneurysms, but their efficacy/safety in the context of recurrent/recanalized aneurysms following stent-assisted coiling (SAC) is not well established. We evaluate the outcomes of FDS retreatment in a large multicenter cohort. Methods We retrospectively analyzed data from 118 patients across 22 institutions who underwent FDS retreatment for recurrent/persistent aneurysms after SAC (2008–22). The primary outcome was angiographic occlusion status at last follow-up, categorized as complete (100%), near-complete (90–99%), or incomplete (<90%) occlusion. Secondary outcomes included procedural complications and clinical outcomes measured by the modified Rankin Scale (mRS). Results A total of 118 patients (median age 57, 74.6% female) with median follow-up of 15.3 months were identified. Complete occlusion was achieved in 62.5% and near-complete occlusion in 25%. FDS deployment within the pre-existing stent was successful in 98.3% of cases. Major complications occurred in 3.4% of cases, including postoperative aneurysmal rupture with resultant mortality (1.6%) and thromboembolic events with long-term disability (1.6%). Favorable clinical outcomes (mRS 0–2) were observed in 95.1% of patients. Wider aneurysm neck diameter was a significant predictor of incomplete occlusion (adjusted OR (aOR) 1.23 per mm, P=0.044), with male sex trending towards association with non-occlusion (aOR 3.2, P=0.07), while baseline hypertension was associated with complete occlusion (aOR 0.32, P=0.048). Conclusions FDS treatment for recurrent/residual aneurysms after SAC represents a viable treatment option for these challenging cases with acceptable safety and reasonable occlusion rates, although lower than de novo FDS occlusion rates.

BACKGROUND The clinical and radiological evolution of large capillary telangiectasias is largely unknown. OBSERVATIONS A 30-year-old female presented with progressive ataxia and nystagmus. She was found to have a large, diffuse enhancing pontomedullary lesion with associated hypointensity on susceptibility-weighted imaging consistent with a large capillary telangiectasia. In addition, she had a second capillary telangiectasia in the right frontal lobe, hypertrophic olivary degeneration (HOD), and multiple susceptibility lesions throughout the brain. The patient’s condition continued to deteriorate and she became nonambulatory over 8 years, with right-sided hemiparesis and hemiataxia, nystagmus, palatal myoclonus, and a left abducens palsy. Over the follow-up period, serial MR images showed increased cavitation of the lesion and significant brainstem atrophy. LESSONS Capillary telangiectasias are generally small, benign lesions composed of dilated capillaries. However, when large (≥ 1 cm), they can be symptomatic. The authors present the case of a diffuse capillary telangiectasia of the pons with HOD and a progressively declining course due to brainstem atrophy and enlarging cavitation of the lesion. The radiological evolution of an aggressive capillary telangiectasia has not been previously reported. https://thejns.org/doi/10.3171/CASE24746