Recurrence of cerebrospinal fluid-venous fistulas at different spinal levels following transvenous embolisation or blood/fibrin glue patching.

AIM OF THE STUDY: This study presents cases of recurrent cerebrospinal fluid-venous fistulas (CVFs) de novo at a different spinal level following successful treatment of initial CVFs. The aim was to highlight this rarely described phenomenon and report the clinical and imaging features after initial treatment, providing insights into the dynamics of recurrent CVFs. CLINICAL RATIONALE FOR THE STUDY: Understanding the course of CVFs post-treatment is crucial for optimising patient management, especially when symptoms persist or recur. MATERIAL AND METHODS: We performed a retrospective chart review of all patients with recurrent CVFs at a different level after treatment of their initial CVF at our institution. Clinical and imaging records were reviewed and summarised, including Bern score features on brain magnetic resonance imaging (MRI) before and after treatment. RESULTS: Four patients with five recurrent CVFs were identified. Recurrent or persistent symptoms encouraged subsequent brain MRI scans, which revealed different outcomes: i.e. persistence, or improvement, or complete resolution of abnormal findings. Initial positive responses included improvement of the pachymeningeal enhancement and venous sinus distension. These improvements were reversed when recurrent symptoms arose, which was also correlated with changes in the Bern score. CONCLUSIONS AND CLINICAL IMPLICATIONS: Recognising the factors of CVF recurrence is crucial for comprehensive management. This study underlines the significance of repeated evaluation of persistent or recurring symptoms of CSF leak after treatment for CVFs.

CWH43 Variants Are Associated With Disease Risk and Clinical Phenotypic Measures in Patients With Normal Pressure Hydrocephalus.

Background and Objectives: Variants in the CWH43 gene have been associated with normal pressure hydrocephalus (NPH). We aimed to replicate these findings, identify additional CWH43 variants, and further define the clinical phenotype associated with CWH43 variants. Methods: We determined the prevalence of CWH43 variants by whole-genome sequencing (WGS) in 94 patients with NPH. The odds of having CWH43 variant carriers develop NPH were determined through comparison with 532 Mayo Clinic Biobank volunteers without a history of NPH. For patients with NPH, we documented the head circumference, prevalence of disproportionate enlargement of subarachnoid hydrocephalus (DESH), microvascular changes on MRI quantified by the Fazekas scale, and ambulatory response to ventriculoperitoneal shunting. Results: We identified rare (MAF <0.05) coding CWH43 variants in 15 patients with NPH. Ten patients (Leu533Terfs, n = 8; Lys696Asnfs, n = 2) harbored previously reported predicted loss-of-function variants, and combined burden analysis confirmed risk association with NPH (OR 2.60, 95% CI 1.12-6.03, p = 0.027). Additional missense variations observed included Ile292Thr (n = 2), Ala469Ser (n = 2), and Ala626Val (n = 1). Though not quite statistically significant, in single variable analysis, the odds of having a head circumference above the 75th percentile of normal controls was more than 5 times higher for CWH43 variant carriers compared with that for noncarriers (unadjusted OR 5.67, 95% CI 0.96-108.55, p = 0.057), and this was consistent after adjusting for sex and height (OR 5.42, 95% CI 0.87-106.37, p = 0.073). DESH was present in 56.7% of noncarriers and only 21.4% of carriers (p = 0.016), while sulcal trapping was also more prevalent among noncarriers (67.2% vs 35.7%, p = 0.030). All 8 of the 15 variant carriers who underwent ventriculoperitoneal shunting at our institution experienced ambulatory improvements. Discussion: CWH43 variants are frequent in patients with NPH. Predicted loss-of-function mutations were the most common; we identified missense mutations that require further study. Our findings suggest that congenital factors, rather than malabsorption or vascular dysfunction, are primary contributors to the CWH43-related NPH clinical syndrome.

Cognitive impairment in chronic migraine compared to pseudotumor cerebri.

INTRODUCTION: We aimed to define the prevalence of objective cognitive impairment in a group of chronic migraineurs, and to define how migraineurs with cognitive impairment differed from those without impairment, and in doing so to compare cognitive impairment in chronic migraine to another chronic headache-related disorder already associated with cognitive impairment (i.e. pseudotumor cerebri syndrome). OBJECTIVES: Cognitive impairment in migraine, especially chronic migraine, has been too little studied. Only a few studies have been done, demonstrating that cognitive impairment exists in chronic migraineurs. It is not known how this compares to other headache-related conditions. MATERIAL AND METHODS: We administered a cognitive battery consisting of the National Adult Reading Test, Mini-Mental Status Examination, Digit Span, Boston Naming Test, Rey Auditory Verbal Learning Test, Trail Making Test, Controlled Oral Word Association, and Category Fluency. Cognitive impairment was defined as mild single-domain with one test score, and mild multi- -domain with two scores more than two standard deviations below the mean for age-, gender-, and education-adjusted norms. The data from this study was compared to our previously published population of patients with pseudotumor cerebri syndrome. RESULTS: One hundred prospectively recruited patients with chronic migraine were enrolled. Fifty-seven patients had normal cognitive profiles. Forty-three patients demonstrated mild cognitive impairment, and more than half (n = 24) showed impairment in multiple cognitive domains. Migraineurs with multi-domain impairment had higher pain intensity, shorter duration of disease, were taking narcotics, had more impaired vision-related mental health scores, and worse social health scores. We found an association between objective cognitive impairment and subjective perception of impairment only when controlling for pain. We found no associations with depression and topiramate use. The mean composite cognitive Z score was no different in chronic migraineurs and patients with pseudotumor cerebri. CONCLUSIONS AND CLINICAL IMPLICATIONS: Most chronic migraineurs have normal cognitive profiles, but a large proportion of them do experience mild cognitive impairment, especially in multiple domains. The impairment seen in migraine is similar to that in pseudotumor cerebri syndrome, which has already been associated with mild cognitive impairment. Cognitively impaired migraineurs are different from non-impaired/less impaired migraineurs in several ways, which may be an important factor in influencing their migraine treatment.

Dual microcatheter and coil/balloon pressure cooker technique for transvenous embolization of cerebrospinal fluid-venous fistulas.

Cerebrospinal fluid-venous fistula is increasingly recognized as a cause of spontaneous intracranial hypotension.1 Transvenous embolization is emerging as an efficacious minimally invasive treatment.2-4 The procedure aims to embolize paraspinal and foraminal veins draining the fistula; however, complete embolization may be challenging as numerous small venous tributaries at the foraminal venous plexus, including dorsal muscular branches, may serve as additional routes of cerebrospinal fluid egress.5 To ensure curative embolization, we adopted a dual microcatheter pressure cooker technique, previously used for treatment of brain arteriovenous malformations.6 This allows improved control of embolic material reflux and greater chance of complete embolization of the site of the fistula and all potential venous tributaries. Video 1 demonstrates this technique employed in a typical case using Onyx (Medtronic, Minnesota, USA) to embolize a cerebrospinal fluid-venous fistula at the left L1 neural foramen. neurintsurg;15/6/614/V1F1V1Video 1Technical video demonstrating dual-microcatheter and coil/balloon pressure cooker technique.

Evaluation and management of orthostatic headache in hypermobility disorders.

Headache is a frequent symptom among patients with hypermobility spectrum disorders. This mini review focuses specifically on a challenging aspect of headache evaluation in all patients, but especially those with hypermobility - the orthostatic headache. While the differential for an orthostatic headache is overall limited, patients with hypermobility disorders have risk factors for all of the most commonly encountered orthostatic headache disorders. The most common conditions to produce orthostatic headaches are discussed - spontaneous intracranial hypotension, cervicogenic headache, and postural orthostatic tachycardia syndrome. Less common etiologies of orthostatic headache pertinent to any patient are presented in table format.

Predictors of cognitive impairment in pseudotumor cerebri.

AIMS OF THE STUDY: We aimed to define the cognitive burden of the largest pseudotumor cerebri syndrome (PTCS) population to date, compare objective to subjective cognitive dysfunction, and determine clinical predictors of cognitive dysfunction amongst an array of previously unstudied factors. CLINICAL RATIONALE: Patients with PTCS commonly report cognitive dysfunction, a factor associated with poor quality of life. It is not definitively known whether cognitive impairment is present in these patients, and what features of the syndrome predict impairment. MATERIALS AND METHODS: We administered a cognitive battery consisting of the National Adult Reading Test, Mini-Mental Status Exam, Digit Span, Boston Naming Test, Rey Auditory Verbal Learning Test, Clock Drawing, Trail Making Test, Controlled Oral Word Association, and Category Fluency. Cognitive impairment was defined as mild-single domain with one test score, and mild-multiple domain with two scores, more than two standard deviations below the mean for age-, gender-, and education-adjusted norms. RESULTS: One-hundred and one prospectively recruited PTCS patients were enrolled. The objective testing showed 30 patients had mild-single domain impairment, and 25 had mild-multi domain impairment. More patients without objective cognitive impairment had transverse venous sinus stenosis, but otherwise the groups did not differ. Two measures of headache severity, the Headache Impact Test and pain on the Numeric Rating Scale, were negatively associated with the composite cognitive score, as was ocular pain, vision-related disability, and mental health. Opening pressure and visual function were not associated with objective cognitive impairment. We found no association between subjective and objective cognitive impairment. CONCLUSIONS AND CLINICAL IMPLICATIONS: Patients with PTCS may be cognitively impaired, and this correlates with measures of headache burden. Studies evaluating cognitive impairment before and after remission of the headache disorder would have to be performed to investigate this relationship further. Patients with self-perception of cognitive burden are no more likely to be cognitively impaired.

Impact of emergency department transitions of care on thrombolytic use in acute ischemic stroke.

BACKGROUND AND PURPOSE: In-hospital mortality is higher for certain medical conditions based on the time of presentation to the emergency department. The primary goal of this study was to determine whether patients with acute ischemic stroke who arrived to the emergency department during a nursing shift change had similar rates of thrombolytic use and functional outcomes compared with patients presenting during nonshift change hours. METHODS: A retrospective review of patients with acute ischemic stroke presenting to the emergency department of a primary stroke center from 2005 through 2010. The time to notify the stroke team, perform a head CT scan, and to start intravenous or intra-arterial thrombolysis was assessed. Thrombolysis rates, mortality rate, discharge disposition, change in the National Institutes of Health Stroke Scale, and change in modified Barthel Index at 3 and 12 months were assessed. RESULTS: Of 3133 patients with acute ischemic stroke, 917 met criteria for inclusion. Arrival during nursing shift change, weekends, and July through September had no impact on process times, thrombolysis rates, and functional outcomes. Arrival at night did result in longer time to intra-arterial but not to intravenous thrombolysis, higher mortality rate, and smaller gain in functional status as measured by the modified Barthel Index at 3 months. The degree of emergency department "busyness" also did not influence tissue-type plasminogen activator treatment times. CONCLUSIONS: Presentation during a nursing shift change, a time of transition of care, did not delay thrombolytic use in eligible patients with acute ischemic stroke. Presentation with acute ischemic stroke at night did result in delays of care for patients undergoing interventional therapies.