Cryptococcal meningitis and cerebral vasculitis in a patient with primary intestinal lymphangiectasia: a case report.

Primary intestinal lymphangiectasia (Waldmann's disease) is a rare exudative enteropathy without precisely assessed infectious risk. We report the case of a 49-year-old male patient with meningitis and cerebral vasculitis due to Cryptococcus neoformans complicating Waldmann's disease diagnosed 12 years ago. The treatment combined liposomal amphotericin B, 3 mg/kg daily plus flucytosine 25 mg/kg/6 h, both intravenously during 15 days, then fluconazole 800 mg daily during 8 weeks, and finally 200 mg daily indefinitely. Dexamethasone 0.4 mg/kg daily during the first week was gradually decreased over 2 months. The outcome was good, and the patient is still followed 3 years later without any recurrence.

White Matter Hyperintensities of the Corpus Callosum Are Associated With Clinical Severity in CADASIL.

BACKGROUND: In CADASIL (Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy), clinical severity is not related to the total burden of white matter hyperintensities (WMHs), presumably because of heterogeneous underlying tissue alterations. We aimed to investigate whether WMHs in the corpus callosum (WMHCC) are due to secondary degeneration and related to clinical severity. METHODS: We evaluated data from 228 CADASIL patients included in an ongoing prospective cohort with available 3-dimensional fluid-attenuated inversion recovery magnetic resonance imaging sequences. We analyzed in a blind manner WMHCC and lacunes in presumably connected areas to determine whether WMHCC are related to secondary degeneration. We evaluated the links between WMHCC and the Mattis dementia rating scale and the modified Rankin Scale-widely used measures of global cognitive performances and disability, respectively. Linear regression models were adjusted for age, sex, level of education, brain volume, number of lacunes, and volume of WMH. RESULTS: Among 228 patients, only 105 (46%) had WMHCC while all had WMH in the rest of the white matter. In 74% of cases, WMHCC crossed a presumably connected nearby lacune, which was significantly higher than the expected value if the spatial distributions of WMHCC and nearby lacunes were unrelated (11%; P<0.001). Patients with WMHCC had worse Mattis dementia rating scale (median [P25-P75], 138 [122-142] versus 143 [140-143]; P<0.001) and worse modified Rankin Scale (2 [1-3] versus 1 [0-1]; P<0.001). In adjusted models, Mattis dementia rating scale was significantly associated with WMHCC (estimate, -6.2 [95% CI, -11.8 to -0.1]). CONCLUSIONS: In CADASIL, WMHCC are likely related to secondary degeneration and are independently related to clinical severity, in contrast to the total burden of WMH.

Computed tomography angiography for quantification of cerebral vasospasm following aneurysmal subarachnoid hemorrhage.

PURPOSE: The purpose of this study was to assess the accuracy of computed tomography angiography (CTA) for quantification of cerebral vasospasm following aneurysmal subarachnoid hemorrhage in proximal and middle segments of intracranial arteries. MATERIALS AND METHODS: Twenty consecutive patients (7 men, 13 women; mean age, 47 ± 7 [SD] years; age range: 27-78 years) with aneurysmal subarachnoid hemorrhage who underwent CTA and digital subtracted angiography (DSA) with a 6-hour window at baseline and during vasospasm period were included. Twelve artery segments were analyzed in each patient. Vasospasm was blindly quantified on CTA and digital subtracted angiography (DSA) by two independent readers with discordance > 10% resolved by open data consensus. Inter-reader and inter-test correlations with DSA as reference, and causes of discordant readings were analyzed. The best sensitivity and specificity of CTA for determination of vasospasm ≥ 50% on DSA was determined using receiver operating curve analysis. RESULTS: Two-hundred-and-ten arterial segments were analyzed after exclusion of 30 segments with missing data or metallic artifacts. An inter-reader discordance >10% was observed in 82 segments (82/210; 39% [95% CI: 32-46]). Inter-test discordances >10% were observed respectively in 115 segments (115/210; 55% [95% CI: 49-62]) with the junior reader and in 73 segments (73/210; 35% [95% CI: 29-42]) with the senior reader. They were related to reader error in 55 (55/210; 26% [95% CI: 20-32]) with the junior reader and 13 (13/210; 6% [95% CI: 3-9]) with the senior reader, as well systematic biases in 8 (8/210; 4% [95% CI: 1-6]), and intrinsic limitation in 52 (52/210; 25% [95% CI: 19-31]). Best sensitivity and specificity of CTA were observed for a threshold value of 30% (sensitivity = 88% [95% CI: 78-97%]; specificity = 84% [95% CI: 77-90%]; area under curve = 0.92 [95% CI: 0.86-0.97]). On a patient basis, sensitivity was 100% (specificity = 60% [95% CI: 38-81%]; area under curve = 0.97 [95% CI: 89-100%] for this same threshold. CONCLUSION: Our study shows a moderate accuracy of CTA for the quantification of cerebral vasospasm, mostly related to challenging interpretation and intrinsic limitations. CTA may rule-out angiographic vasospasm ≥ 50% when no segment has vasospasm over than 30%.

Vestibular atelectasis: Myth or reality?

OBJECTIVES/HYPOTHESIS: Because delayed post-contrast three-dimensional fluid-attenuated inversion recovery imaging sequences enable the distinction between the utricle and the saccule, we raised the hypothesis that patients with vestibular atelectasis (VA) could show unilateral collapse of the utricle and the ampullas on imaging. STUDY DESIGN: Retrospective case series. METHODS: We retrospectively reviewed 200 patients who underwent 3 T magnetic resonance imaging (MRI) after intravenous administration of gadolinium. MRI scans were assessed for the presence of VA. The endolymphatic space was considered as collapsed when the utricle and at least two ampullas were not visible or were barely visible. RESULTS: We reported four patients with VA on MRI responsible for atypical clinical presentations of acute vestibular deficit. All patients presented a specific involvement of the pars superior sensory captors (utricle, ampullas), preserving the pars inferior sensory captors (cochlea and saccule). This was confirmed both clinically and on MRI. CONCLUSIONS: Our study is the first to describe in vivo unilateral collapse of the pars superior on delayed postcontrast MRI in patients with a clinical unilateral vestibular loss. LEVEL OF EVIDENCE: 4 Laryngoscope, 129:1689-1695, 2019.

Stent Placement for Disabling Pulsatile Tinnitus Caused by a Lateral Sinus Stenosis: A Retrospective Study.

BACKGROUND: Lateral sinus stenosis is a little-known cause for pulsatile tinnitus (PT). In several small series, stenting has been described as an effective treatment for disabling PT linked with this type of stenosis. OBJECTIVES: To describe the clinical, radiological, and manometric characteristics of patients treated for disabling PT by lateral sinus stenosis. Assessment of the efficacy of stenting for this indication. METHODS: Retrospective study of patients treated for isolated PT by stenting of a lateral sinus stenosis in our institution, between 2009 and 2015. RESULTS: Fourteen patients were included in our study. All of them were women. The median age at the onset of symptoms was 39.0 (21.0) years. The median body mass index was 28.5 (7.0) kg/m 2 . Stenting of the lateral sinus led to the disappearance of PT without recurrence in 13 patients. In one patient, stenting did not modify the noise. In this case, another cause of PT was diagnosed after stent placement. CONCLUSION: Lateral sinus stenosis is a curable cause of venous PT. Other causes of PT must be ruled out before an endovascular treatment is undertaken, due to the frequent asymptomatic nature of Pacchioni granulations in the lateral sinus. Treatment by stenting is effective in all cases, provided that stenosis underlies the PT.

Stenting of the Lateral Sinus in Idiopathic Intracranial Hypertension According to the Type of Stenosis.

BACKGROUND: Over the past decade, stenting of lateral sinus stenosis has been used to treat idiopathic intracranial hypertension. Two types of stenoses have been identified: extrinsic and intrinsic. OBJECTIVE: The aim of this study was to report the results of our use of this procedure to treat patients with extrinsic or intrinsic stenoses in idiopathic intracranial hypertension. METHODS: We retrospectively studied clinical, radiological, and manometric data from patients with idiopathic intracranial hypertension who were treated at our institution between January 2009 and January 2015 by stenting of the lateral sinus. RESULTS: Data were studied from 19 women and 2 men. Average body mass index was 29 kg/m 2 , and the median age at stenting was 33 years. Patients with extrinsic stenoses were younger than those with intrinsic stenoses. Transstenotic gradients measured with patients under general anesthesia were lower than those measured with patients under local anesthesia. In all cases, stenting was effective for papilledema and pulsatile tinnitus. Seventeen patients reporting headaches found that they disappeared completely after stenting. Two complications without long-term effects were reported. CONCLUSION: Irrespective of the type of stenosis, stenting of lateral sinus stenoses is an effective treatment for intracranial hypertension symptoms. At our institution, this treatment has replaced draining of cerebrospinal fluid when treatment with acetazolamide has proved to be ineffective.

Susceptibility-weighted angiography for the detection of high-flow intracranial vascular lesions: preliminary study.

OBJECTIVES: Susceptibility-weighted magnetic resonance imaging (MRI) sequences may demonstrate various signal intensities of draining veins in cases of high-flow vascular malformation (HFVM), including arteriovenous malformation (AVM) and dural arteriovenous fistula (dAVF). Our objective was to evaluate susceptibility-weighted angiography (SWAN) for the detection of HFVM. METHODS: Fifty-eight consecutive patients with a suspected intracranial vascular malformation were explored with SWAN and post-contrast MRI sequences at 3 T. The diagnosis of slow-flow vascular malformation (SFVM), including developmental venous anomaly (DVA) or brain capillary telangiectasia (BCT), was based on MRI. Patients with suspected HFVM underwent digital subtraction angiography (DSA). SWAN images were analysed by three blinded readers according to a three-point scale of the venous signal. RESULTS: Thirty-one patients presented 35 SFVM (26 DVA and 9 BCT) that systematically appeared hypointense on SWAN images. In patients with atypical MRI findings, DSA revealed one patient with an atypical DVA and 26 patients with HFVM (22 AVM and 4 dAVF). SWAN revealed at least one venous hyperintensity in all patients with HFVM. Agreement between readers was excellent. CONCLUSIONS: SWAN appears reliable for characterising blood flow dynamics in brain veins. In clinical practice, SWAN can routinely rule out HFVM in patients with atypical brain veins.