Transverse Venous Sinus Stenosis in Patients With Nasal Cerebrospinal Fluid Leak and Idiopathic Intracranial Hypertension.

OBJECTIVE: Spontaneous nasal cerebrospinal fluid (CSF) leaks are frequently linked to idiopathic intracranial hypertension (IIH). The objectives of our study were: (1) to determine the rate of transverse venous sinus stenosis (TVSS) in patients with spontaneous nasal CSF leak and in patients with IIH without CSF (controls), and (2) to study the correlation between spontaneous nasal CSF leak and brain imaging features. STUDY DESIGN: A multicenter retrospective case-control study. SETTING: Six French tertiary hospitals. METHODS: Patients with spontaneous nasal CSF leaks and patients with IIH without nasal CSF leaks (controls) were included. The transverse venous sinus patency was analyzed by magnetic resonance imaging to identify possible stenosis or hypoplasia. RESULTS: Thirty-two patients with spontaneous nasal CSF leaks and 32 controls were included. TVSS was significantly more frequent in patients with spontaneous nasal CSF leaks than in controls (p = .029). Univariate analysis indicated that TVSS (odds ratio, OR: 4.2; 95% confidence interval, CI [1.352-14.915]; p = .017) and arachnoid granulations (OR: 3; 95% CI [1.065-8.994]; p = .042) were risk factors for spontaneous nasal CSF leak. In multivariate analysis, TVSS and arachnoid granulations were independent risk factors of nasal CSF leak (OR: 5.577, 95% CI [1.485-25.837], p = .016; and OR: 4.35, 95% CI [1.234-17.756], p = .029, respectively). CONCLUSION: This multicenter case-control study shows that TVSS is an independent risk factor for CSF leak in patients with IIH. Stenosis management by interventional radiology may be proposed postoperatively to increase the success of IIH surgical treatment or preoperatively to reduce the need for surgery.

Immune-mediated diseases involving central and peripheral nervous systems.

BACKGROUND AND PURPOSE: In addition to combined central and peripheral demyelination, other immune diseases could involve both the central nervous system (CNS) and peripheral nervous system (PNS). METHODS: To identify immune-mediated diseases responsible for symptomatic combined central/peripheral nervous system involvement (ICCPs), we conducted a multicentric retrospective study and assessed clinical, electrophysiological, and radiological features of patients fulfilling our ICCP criteria. RESULTS: Thirty patients (20 males) were included and followed during a median of 79.5 months (interquartile range [IQR] = 43-145). The median age at onset was 51.5 years (IQR = 39-58). Patients were assigned to one of four groups: (i) monophasic disease with concomitant CNS/PNS involvement including anti-GQ1b syndrome (acute polyradiculoneuropathy + rhombencephalitis, n = 2), checkpoint inhibitor-related toxicities (acute polyradiculoneuropathy + encephalitis, n = 3), and anti-glial fibrillary acidic protein astrocytopathy (subacute polyradiculoneuropathy and meningoencephalomyelitis with linear gadolinium enhancements, n = 2); (ii) chronic course with concomitant CNS/PNS involvement including paraneoplastic syndromes (ganglionopathy/peripheral hyperexcitability + limbic encephalitis, n = 4); (iii) chronic course with sequential CNS/PNS involvement including POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome (polyradiculoneuropathy + strokes, n = 2), histiocytosis (polyradiculoneuropathy + lepto-/pachymeningitis, n = 1), and systemic vasculitis (multineuropathy + CNS vasculitis/pachymeningitis, n = 2); and (iv) chronic course with concomitant or sequential CNS/PNS involvement including combined central and peripheral demyelination (polyradiculoneuropathy + CNS demyelinating lesions, n = 10) and connective tissue diseases (ganglionopathy/radiculopathy/multineuropathy + limbic encephalitis/transverse myelitis/stroke, n = 4). CONCLUSIONS: We diagnosed nine ICCPs. The timing of central and peripheral manifestations and the disease course help determine the underlying immune disease. When antibody against neuroglial antigen is identified, CNS and PNS involvement is systematically concomitant, suggesting a common CNS/PNS antigen and a simultaneous disruption of blood-nerve and blood-brain barriers.