Fermo, Olga

Cerebrospinal fluid (CSF)–venous fistulas are an increasingly recognized source of spontaneous intracranial hypotension, for which endovascular embolization has emerged as an effective treatment option.1–3 Advances in diagnostic imaging have improved detection of CSF–venous fistulas throughout the cervical, thoracic, and lumbar spine; however, identification of lesions at the cranial and caudal extremes of the spine remains challenging.4 In video 1, we present the novel case of an anterior condylar confluence CSF–venous fistula arising from a hypoglossal canal nerve root sleeve diverticulum. We demonstrate the diagnostic evaluation, preprocedural planning, and transvenous embolization of the fistula using the pressure cooker technique, highlighting key technical considerations for navigating this uncommon venous anatomy. Finally, we review the patient’s postprocedural course and imaging findings demonstrating radiographic resolution of spontaneous intracranial hypotension. Video 1 Endovascular embolization of a cerebrospinal fluid–venous fistula arising from a hypoglossal canal nerve root sleeve diverticulum using a transvenous pressure cooker technique.

Objective: To compare the prevalence and patterns of transverse sinus stenosis (TSS) in patients with lateral skull base spontaneous cerebrospinal fluid (sCSF) leaks and idiopathic intracranial hypertension (IIH), to clarify its role in leak pathogenesis and implications for venous-directed therapies. Study design: Retrospective case-control study. Setting: Single tertiary care institution. Patients: Adults with lateral skull base sCSF leaks confirmed by clinical, laboratory, or surgical findings, and adults with IIH without cranial CSF leaks meeting Modified Dandy criteria. Intervention: Independent magnetic resonance venography evaluation by 2 fellowship-trained neuroradiologists. Main outcome measures: Presence, laterality, and severity of TSS. Results: Eighteen patients with sCSF leaks and 78 patients with IIH were included. The rate of individual TSS, including both unilateral and bilateral cases, was higher in the IIH cohort than in the sCSF leak cohort (bilateral TSS: n=66, 84.6% vs. n=6, 33.3%; P <0.001). Severe bilateral TSS was observed more in the IIH cohort (n=40, 51.3%) compared with the sCSF leak cohort (n=1, 5.6%; P <0.001). Conclusions: Though patients with lateral skull base sCSF leaks have TSS, the significantly lower rate compared with patients with IIH suggests that TSS may play a lesser role in the pathogenesis of lateral skull base defects. These findings indicate that the role of transverse sinus stenting in lateral skull base sCSF leaks remains uncertain and highlight the need to further investigate local remodeling processes when considering the role of venous sinus stenting in this population.

Introduction: Migraine with aura may be the earliest symptom in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Female patients have a higher incidence of migraine with an increased associated disability. In this study, we systematically evaluate the clinical features and management of migraine in female patients with CADASIL in a multi-center health system. Methods: Clinical data was extracted from the electronic medical records of female patients diagnosed with CADASIL and managed at Mayo Clinic Enterprise. Diagnosis was confirmed by a pathogenic NOTCH3 mutation or granular osmiophilic material on skin biopsy. Results: Forty-three female patients with CADASIL were identified. Twenty-two patients (51.2%) had a diagnosis of migraine with aura and eight patients (18.6%) had a diagnosis of migraine without aura. The most common type of aura was visual (81.8%). Nineteen patients (63.3%) required at least one acute medicine and/or one preventive medicine. Acute therapy helped in 18 cases (94.7%) and preventive therapy helped in 13 cases (68.4%). Six patients (20%) tried at least three forms of acute therapy, and eleven patients (36.7%) tried at least three forms of preventive therapy. The presence of visual aura was associated with higher likelihood of need for acute therapy (OR = 3.0, 95% CI = 1.6 - 5.8, P = 0.015). However, the presence of any type of aura was associated with lower likelihood of need for or resistance to preventive therapy (OR = 0.08, 95% CI = 0.01 - 0.57, P = 0.005). Also, headache onset after age of 45 years was associated with higher likelihood of need for or resistance to preventive therapy (OR = 38.0, 95% CI = 2.7 - 528.0, P <0.001). The most common acute therapy was nonsteroidal anti-inflammatory drugs (NSAIDs) (14, 73.7%). Classes of preventive therapy involved anticonvulsants (15, 78.9%), antidepressants (11, 57.9%), Botox injections (7, 36.8%), beta blockers (6, 31.6%), calcium channel blockers (3, 15.8%), and calcitonin gene-related peptide (CGRP) inhibitors (3, 15.8%). Conclusion: Half of female patients with CADASIL had a diagnosis of migraine with aura. Around two thirds of them tried at least one form of acute therapy, most commonly NSAIDs, or preventive therapy, most commonly anticonvulsants. Resistance to preventive therapy was seen in more than 30% of the cases, highlighting the need for advances in migraine management in CADASIL.

Background The majority of patients with spontaneous intracranial hypotension (SIH) are symptomatic. Some patients are discovered incidentally. The proportion of asymptomatic SIH has never been defined. This article reports our institution’s rate of asymptomatic cases among all of our patients with high/intermediate Bern scores on brain MRI, etiology of leak in asymptomatic cases, complications of asymptomatic leaks, and evolution of imaging before and after treatment. Methods We retrospectively analyzed data from the Cerebrospinal Fluid (CSF) Dynamics clinic spanning September 2020 to July 2023. Bern score was calculated from all available brain MRIs in patients with confirmed leaks. Patients with iatrogenic leaks or no brain MRI were excluded. The charts of asymptomatic patients were reviewed to obtain MRI indications, SIH progression, complications, leak type, and treatment outcomes. Results Some 8.6% (7/81) patients with high/intermediate Bern scores were asymptomatic at the time of imaging. Two patients declined myelography. Four of seven asymptomatic patients were found to have CSF-venous fistulas and underwent embolization with radiographic improvement. Complications of asymptomatic leaks in this population included subdural hematoma and superficial siderosis. Conclusions Asymptomatic but clearly present spinal CSF leaks were not uncommon in our group. These results highlight the possibility of a presymptomatic phase in patients with CSF leaks. In our sample, myelography was readily positive for etiology of the leak, and leaks promptly responded to targeted treatment.