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Nicolae Sarbu, Robert Y Shih, Robert V Jones, Iren Horkayne-Szakaly, Laura Oleaga, James G Smirniotopoulos
White matter diseases include a wide spectrum of disorders that have in common impairment of normal myelination, either by secondary destruction of previously myelinated structures (demyelinating processes) or by primary abnormalities of myelin formation (dysmyelinating processes). The pathogenesis of many white matter diseases remains poorly understood. Demyelinating disorders are the object of this review and will be further divided into autoimmune, infectious, vascular, and toxic-metabolic processes. Autoimmune processes include multiple sclerosis and related diseases: tumefactive demyelinating lesions, Balo concentric sclerosis, Marburg and Schilder variants, neuromyelitis optica (Devic disease), acute disseminated encephalomyelitis, and acute hemorrhagic leukoencephalopathy (Hurst disease)...
September 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Sabine Montaut, Ivica Blažević, Mirko Ruščić, Patrick Rollin
The glucosinolates (GLs) present in seed extracts of Brassica elongata Ehrh., B. botteri Vis and B. cazzae Ginzb. & Teyber from Croatia were identified by LC-MS. 4-Hydroxyindol-3-ylmethyl GL (3) was the major GL in the seeds of B. elongata, along with the four minor GLs 2-(R)-hydroxy-3-butenyl- (1), 3-butenyl- (2), 4-pentenyl- (4) and indol-3-ylmethyl (5). The seeds of B. botteri (Vis island) and B. cazzae (Sušac island) contained 2 as the major GL as well as 1, 3, 5 and 4-methoxyindol-3-ylmethyl GL (6). However, the GLs in B...
August 2, 2016: Natural Product Research
Clea Staelens, Kurt Audenaert, Hannelore Tandt, Veroniek Van Driessche, Gilbert Lemmens
No abstract text is available yet for this article.
October 2016: Clinical Neurology and Neurosurgery
B Grygiel-Górniak, M Puszczewicz, E Czaplicka
Susac syndrome is an endotheliopathy affecting the arterioles of the brain, retina, and inner ear. Many cases of Susac syndrome are underdiagnosed, mainly at the early stages of the disease, while prompt diagnosis enables a speedy recovery. Immediate treatment can halt disease progression and even prevent future disability. We report a case of Susac syndrome, describe the difficulties in the diagnosis of this case, and include a detailed history of a 35-year-old man via the presentation of extensive laboratory work-up and imaging studies...
July 2016: European Review for Medical and Pharmacological Sciences
Carl Ellenberger
This chapter can guide the use of imaging in the evaluation of common visual syndromes: transient visual disturbance, including migraine and amaurosis fugax; acute optic neuropathy complicating multiple sclerosis, neuromyelitis optica spectrum disorder, Leber hereditary optic neuropathy, and Susac syndrome; papilledema and pseudotumor cerebri syndrome; cerebral disturbances of vision, including posterior cerebral arterial occlusion, posterior reversible encephalopathy, hemianopia after anterior temporal lobe resection, posterior cortical atrophy, and conversion blindness...
2016: Handbook of Clinical Neurology
Daniel Kondziella
Rare causes of ischaemic stroke comprise a plethora of diagnoses of cardioembolic, inflammatory and genetic origin. The differential diagnosis is challenging but important because these disorders (e.g. monogenetic disorders such as COL4A1 mutations) often affect young individuals, they typically require sophisticated diagnostics (e.g. fluorescence angiography in Susac's syndrome) and they are increasingly treatable (e.g. enzyme replacement for Fabry's disease). This review discusses practical considerations and recent diagnostic and therapeutic advances in uncommon causes of ischaemic stroke...
June 27, 2016: Ugeskrift for Laeger
Kimberly D Blankshain, Heather E Moss
BACKGROUND: Medical research registries (MRR) are organized systems used to collect, store, and analyze patient information. They are important tools for medical research with particular application to the study of rare diseases, including those seen in neuro-ophthalmic practice. EVIDENCE ACQUISITION: Evidence for this review was gathered from the writers' experiences creating a comprehensive neuro-ophthalmology registry and review of the literature. RESULTS: MRR are typically observational and prospective databases of de-identified patient information...
September 2016: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
Juan B Yepez, Felipe A Murati, Michele Pettito, J Fernando Arevalo
PURPOSE: To report the use of intravitreal triamcinolone for Susac Syndrome in one patient. METHODS: Case report. RESULTS: A 23-year-old woman presented with presented with sudden visual loss in the left eye with associated neurologic symptoms. Ophthalmic examination and fluorescein angiography showed multiple areas of branch retinal artery occlusion, bilaterally. Magnetic resonance imaging showed dot-like, diffusion-restricted lesions in the corpus callosum, and audiometry showed low-frequency sensory hearing loss, compatible with Susac Syndrome...
June 7, 2016: Retinal Cases & Brief Reports
Elie Gertner, Michael H Rosenbloom
BACKGROUND: Susac syndrome (retinocochleocerebral vasculopathy) is an autoimmune endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear. It presents with encephalopathy, branch retinal artery occlusions, and hearing loss. The condition is often under recognized because the clinical symptoms may present at different times and physicians may be unfamiliar with the syndrome. Peripheral findings would be helpful in early diagnosis. There are numerous treatment regimens proposed with varying effectiveness...
2016: Journal of Medical Case Reports
Renate Engisch, David S Titelbaum, Lara Chilver-Stainer, Frauke Kellner-Weldon
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...
June 2016: Multiple Sclerosis: Clinical and Laboratory Research
Jaume Sastre-Garriga
Magnetic resonance imaging detection of leptomeningeal enhancement has long been considered a red flag for a diagnosis of multiple sclerosis. However, recent studies seem to suggest that leptomeningeal enhancement can be detected in up to 25% of patients with multiple sclerosis. The case reported here suggest a distinct set of features of leptomeningeal enhancement in a patient with Susac's syndrome which may still be helpful in the differential diagnosis between Susac's syndrome and multiple sclerosis.
June 2016: Multiple Sclerosis: Clinical and Laboratory Research
Nikisha Kothari, Ajay E Kuriyan
No abstract text is available yet for this article.
July 1, 2016: JAMA Neurology
Pedro Serrano, Samit K Dutta, Andrew Proudfoot, Biswaranjan Mohanty, Lukas Susac, Bryan Martin, Michael Geralt, Lukasz Jaroszewski, Adam Godzik, Marc Elsliger, Ian A Wilson, Kurt Wüthrich
For more than a decade, the Joint Center for Structural Genomics (JCSG; www . jcsg . org) worked toward increased three-dimensional structure coverage of the protein universe. This coordinated quest was one of the main goals of the four high-throughput (HT) structure determination centers of the Protein Structure Initiative (PSI; www . nigms . nih . gov/Research/specificareas/PSI). To achieve the goals of the PSI, the JCSG made use of the complementarity of structure determination by X-ray crystallography and nuclear magnetic resonance (NMR) spectroscopy to increase and diversify the range of targets entering the HT structure determination pipeline...
May 7, 2016: FEBS Journal
Kshitiz Kumar, Bhavini Jariwala, Pallavi Raj, Amar Agarwal
PURPOSE: To report a case of Susac syndrome with ocular complications in the chronic stage of the disease. METHODS: Case report. RESULTS: A 37-year-old male patient of Indian origin presented with painless blurring of vision in the right eye. Ophthalmoscopic examination revealed vitreous hemorrhage in the right eye and NVE in both eyes well-documented angiographically. Old medical records revealed a previous attack of bilateral BRAO as a complete triad of Susac syndrome 10 years before, with an uncomplicated clinical course...
April 29, 2016: Seminars in Ophthalmology
Aniruddha Agarwal, Mohamed Soliman, Salman Sarwar, Mohammad Ali Sadiq, Diana V Do, Quan Dong Nguyen, Yasir Jamal Sepah
PURPOSE: To report spectral-domain optical coherence tomography (SD-OCT) features in patients diagnosed with Susacs syndrome. METHODS: Clinical report of two cases. RESULTS: Spectral-domain optical coherence tomography was performed in two patients diagnosed with Susacs syndrome. Both the patients had normal macular perfusion on fluorescein angiography (FA). However, SD-OCT revealed bilateral, temporal macular atrophy with disorganization and thinning of the retinal layers...
April 14, 2016: Retinal Cases & Brief Reports
Danielle R Bullock, Sean Rivera, Robert M Beardsley, Erick D Bothun, Robert M Rennebohm, Richard K Vehe, Patricia M Hobday
No abstract text is available yet for this article.
July 2016: Arthritis & Rheumatology
Michael C Abt, Charlie G Buffie, Bože Sušac, Simone Becattini, Rebecca A Carter, Ingrid Leiner, James W Keith, David Artis, Lisa C Osborne, Eric G Pamer
Antibiotic administration can disrupt the intestinal microbiota and down-regulate innate immune defenses, compromising colonization resistance against orally acquired bacterial pathogens. Vancomycin-resistant Enterococcus faecium (VRE), a major cause of antibiotic-resistant infections in hospitalized patients, thrives in the intestine when colonization resistance is compromised, achieving extremely high densities that can lead to bloodstream invasion and sepsis. Viral infections, by mechanisms that remain incompletely defined, can stimulate resistance against invading bacterial pathogens...
February 24, 2016: Science Translational Medicine
Alena M Gallegos, Huizhong Xiong, Ingrid M Leiner, Bože Sušac, Michael S Glickman, Eric G Pamer, Jeroen W J van Heijst
The T cell antigen receptor (TCR) is unique in that its affinity for ligand is unknown before encounter and can vary by orders of magnitude. How the immune system regulates individual T cells that display very different reactivity to antigen remains unclear. Here we found that activated CD4(+) T cells, at the peak of clonal expansion, persistently downregulated their TCR expression in proportion to the strength of the initial antigen recognition. This programmed response increased the threshold for cytokine production and recall proliferation in a clone-specific manner and ultimately excluded clones with the highest antigen reactivity...
April 2016: Nature Immunology
Lindsay N Ramey, Edrick Lopez, Timothy Young
A 43-yr-old man presented with headaches, forgetfulness, and personality changes. Based on imaging, there was concern for acute disseminated encephalomyelitis. He improved with steroids and immunoglobulin. When symptoms recurred, magnetic resonance imaging showed new scattered gray and white matter lesions, many within the corpus callosum. Angiogram showed multiple branch retinal artery occlusions, pathognomonic for Susac syndrome. He was treated with steroids, intravenous immunoglobulin, and cytoxan and was sent to acute rehabilitation...
April 2016: American Journal of Physical Medicine & Rehabilitation
F London, D Pothalil, T P Duprez, C J M Sindic
Susac syndrome is a rare vasculopathy of unknown aetiology, affecting prominently young women and electively targeting brain (encephalopathy), retina (visual field defects), and cochlea (hearing loss), of which optimal treatment has yet to be established. We report clinical, CSF and MRI features together with the long-term outcome in a monocentric series of eight consecutive patients with unusual sex ratio (5 male; 3 female), to define the best diagnostic/therapeutic strategy. Six patients presented with the classical clinical triad within less than 6 months after symptoms onset; two did not suffer from sensorineural hearing loss...
January 19, 2016: Acta Neurologica Belgica
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