Susac's syndrome: Leptomeningeal enhancement on 3D FLAIR MRI.

BACKGROUND: Contrast-enhanced (ce) fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR MRI) has recently been shown to identify leptomeningeal pathology in multiple sclerosis. OBJECTIVE: To demonstrate leptomeningeal enhancement on three-dimensional (3D) FLAIR in a case of Susac's syndrome. METHODS: Leptomeningeal enhancement was correlated with clinical activity over 20 months and compared to retinal fluorescein angiography. RESULTS: The size, number, and location of leptomeningeal enhancement varied over time and generally correlated with symptom severity. The appearance was remarkably similar to that of retinal vasculopathy. CONCLUSION: Ce 3D FLAIR may aid in diagnosis and understanding of pathophysiology in Susac's syndrome and may serve as a biomarker for disease activity.

Early and delayed neurological manifestations of cardiac myxomas.

Cardiac myxoma can embolize and cause early and delayed sequelae including stroke, growth into intracranial fusiform aneurysms and cerebral tumors with risk of hemorrhage and mass effect. Here, we report the rare coincidence of all these manifestations in a 63-year-old man who presented with cognitive and behavioral changes, and seizures 9 months after an embolic stroke from the heart tumor. C-reactive protein (CRP) was elevated at the time of stroke and cardiac myxoma diagnosis but was normal at late neurologic manifestation with isolated myxoma-related intracranial tumors and aneurysms. Low-dose whole-brain radiotherapy can be helpful to diminish cerebral myxoma tumors and fusiform aneurysms despite reported increased risk of aneurysm rupture.

Leptomeningeal Enhancement on 3D-FLAIR MRI in Multiple Sclerosis: Systematic Observations in Clinical Practice.

BACKGROUND AND PURPOSE: Meningeal inflammation is implicated in cortical demyelination and disability progression in multiple sclerosis (MS). Gadolinium (Gd)-enhanced 3-dimensional (3D) FLAIR (fluid-attenuated inversion recovery) magnetic resonance imaging (MRI) can identify leptomeningeal enhancement (LME) in MS. Further characterization is needed to determine if LME is an imaging biomarker for meningeal inflammation. We sought to characterize the natural history of LME in the community setting, including persistence/resolution, effect of disease-modifying therapy, scanner variability, timing of acquisition, and imaging pitfalls that may lead to misinterpretation. METHODS: A total of 341 MRI exams with Gd-enhanced 3D-FLAIR were reviewed in MS and non-MS patients to determine frequency of enhancement by MS subtype and association with therapy. A phantom was used to assess scanner variability. Two MS patients with seven LME were imaged at four postinjection time points to generate time-intensity curves. Imaging pitfalls were compiled. RESULTS: A total of 16.6% (40/241) of MS patients revealed LME compared to 8% (8/100) in non-MS patients (P = .04). There was no association with MS subtype, therapy, or disease activity. Detection using General Electric's version of 3D-FLAIR (29%) was greater than with Siemen's 3D-FLAIR (12%) at 1.5T (Tesla) (P < .001). Lesions were generally stable but resolved in 2 patients following high-dose steroids. LME kinetics were heterogeneous, even within patients, without uniform optimal time for acquisition. Enhancement curves exhibited three different variations, similar to the two-compartment model. Imaging pitfalls included enhancements of uncertain biologic significance, cortical veins and anatomic structures, and imaging artifacts. CONCLUSIONS: Awareness of LME characteristics, variability with imaging parameters, and imaging pitfalls will facilitate determining the potential role as an imaging biomarker for meningeal inflammation.

Subtle Imaging Findings Aid the Diagnosis of Adolescent Hereditary Spastic Paraplegia and Ataxia.

PURPOSE: Hereditary spastic paraplegia (HSP) and hereditary spastic ataxia (HSA) are a heterogeneous group of genetic disorders characterized by progressive lower limb spasticity resulting from pyramidal tract dysfunction. By identifying critical imaging findings within the clinical context of spasticity, radiologists are uniquely positioned to recommend specific genetic testing, and thus facilitate diagnosis. METHODS: We present two examples of HSP and HSA that had gone clinically unrecognized for years, and in which magnetic resonance imaging played a critical role in the diagnosis. RESULTS: Radiologists' awareness of HSP and HSA, combined with a critical review of the clinical history and characteristic imaging findings led to specific genetic testing and a definitive diagnosis. CONCLUSION: Awareness of HSP and HSA among radiologists will expedite more accurate diagnosis, explanation of patient symptoms, recommendation for syndrome-specific treatment, and family planning considerations.