84 research outputs found

    Value of prominent flow voids without cord edema in the detection of spinal arteriovenous fistulae

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    Purpose: To determine the prevalence of spinal dural arteriovenous fistulae (SDAVF) in patients presenting with prominent vascular flow voids on imaging without other imaging findings suggestive of SDAVF. Methods: We retrospectively identified patients from January 1, 2005 to March 1, 2012 who underwent spinal angiography for suspected SDAVF with prominent vascular flow voids on prior imaging. We excluded patients with other major spinal pathology or other imaging findings of SDAVF including cord hyperintensity, enhancement, or expansion. We calculated the proportion of patients with positive findings for SDAVF on angiography and evaluated the prevalence of SDAVF for this finding alone and in correlation with clinical findings. Results: 18 patients underwent spinal angiography for prominent flow voids on imaging without other spinal pathology or imaging findings of SDAVF. Three had a SDAVF detected on angiography. The prevalence of SDAVF in this population was low, only 17% (95% CI 6-39%). All of the patients with positive angiography findings had myelopathy, increasing the prevalence to 100% if the additional clinical finding of myelopathy was present. Conclusions: Prominent flow voids without other imaging findings suggestive of SDAVF is poorly predictive of the presence of a SDAVF, unless myelopathy is present clinically. © 2014 Alhilali et al

    Clinical and Neuroimaging Manifestations of Erdheim–Chester Disease: A Review

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    ABSTRACT Erdheim–Chester disease (ECD) is a rare disorder characterized by accumulation of non‐Langerhans cell histiocytes in multiple organs. The clinical manifestations are protean and vary from asymptomatic focal disease to potentially fatal multisystem disorder. The commonest presentation is symmetric osterosclerotic lesions of lower extremity long bones; other organs, including cardiovascular, nervous, and endocrine system may be affected. Central nervous system involvement can occur in up to 50% cases and is associated with poor prognosis. The disease pathogenesis involves organ involvement secondary to histiocytic infiltration and systemic inflammation driven by Th1 cytokine activation. The recent discovery of activating mutations in proto‐oncogene B‐rapidly accelerated fibrosarcoma (BRAF) V600E and other genes involved in mitogen‐activated protein kinase (MAPK) pathways has led to redefinition of ECD as a myeloid neoplastic disorder. The diagnosis requires histochemical and molecular analysis of histiocytes in tissue biopsies in patients with compatible clinical and imaging features. The treatment options include interferon‐alpha, anakinra, and immunosuppressive therapies. Better understanding of disease pathogenesis has led to development of novel targeted and effective therapies including BRAF and MEK inhibitors. The rarity of the disease and variable clinical features and course often results in diagnostic errors and delays. Rare primary neurological presentation can occur mimicking CNS inflammatory, neoplastic, or demyelinating disorders. We report an unusual case of ECD presenting with progressive encephalopathy and ataxia along with multifocal brainstem and cerebellar lesions. A comprehensive review of clinical and neuroimaging features and immunohistochemical and molecular characteristic of ECD are presented along with review of neuroimaging findings in two previously reported cases

    Anomalous Extraocular Muscles: A Case Series of Orbital Bands Connecting the Superior Rectus to Inferior Rectus

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    Orbital bands are rarely reported abnormal connections of tissue that bridge extraocular muscles and/or the globes. A series of 7 cases of orbital bands that bridged the superior and inferior rectus muscles were reviewed. The bands in this small series were discovered as incidental findings, with no relation to the patients' symptoms and, therefore, may have been of no clinical significance

    Sacral morel-lavallée lesion: a not-so-rare diagnosis

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    Morel-Lavallée lesions are closed soft tissue degloving injuries with a propensity to become infected, arising in the lumbosacral region or even the scalp, common anatomical locations in neuroradiological studies. The radiologist must recognize this entity, its traumatic etiology, and treatment options. Our patient's Morel-Lavallée lesion was evaluated with ultrasound and MRI, demonstrating a predominantly hemorrhagic lesion successfully managed by aspiration
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