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Presence of decreased Intraepidermal Nerve Fiber Density consistent with Small Fiber Neuropathy in Patients with Central Post-Stroke Pain (P1.050)
CONCLUSIONS: We have demonstrated evidence of decreased ENFD in a small series of patients with CPSP. These findings do not imply causality; however, they do bring up several important questions for further consideration. Does the central nervous system injury associated with stroke result in small fiber neuropathy in certain patients? Are patients with asymptomatic, previously undiagnosed small fiber neuropathy at increased risk for developing CPSP following a stroke? Do these findings have any implication for treatment? Further studies including prospective analyses are necessary to better understand these findings for b...
Source: Neurology - April 8, 2015 Category: Neurology Authors: Yushvayev-Cavalier, Y., Amory, C., Bernardini, G., Argoff, C. Tags: Cerebrovascular Disease and Interventional Neurology: The Spectrum of Small Vessel Cerebrovascular Disease Source Type: research

Bilateral vertebral artery dissection, agenesis of both ICAs, and connective tissue aberrations
A 35-year-old woman presented with acute signs of stroke (appendix e-1 on the Neurology® Web site at www.neurology.org). After initial CT with angiography, MRI with time-of-flight angiography confirmed agenesis of both internal carotid arteries (ICA; figure 1A). It revealed bilateral vertebral artery (VA) dissections and ischemias in both middle artery territories (figure 1, A–E). Skin biopsy microscopy (figure 2) was consistent with ultrastructural connective tissue syndrome (uCTD), for which no further evidence was found apart from mild hypermobility of the finger joints. The underlying uCTD with structural ins...
Source: Neurology - April 8, 2013 Category: Neurology Authors: Lill, C. M., Gunther-Kunkel, K., Hoch, H., Paul, F., Grond-Ginsbach, C., Hausser, I., Zipp, F. Tags: Stroke in young adults, MRI, All Cerebrovascular disease/Stroke, Carotid artery dissection NEUROIMAGES Source Type: research

Misdiagnosis of CADASIL: A single Medical Center experience (P1.251)
Conclusions:CADASIL is the most prevalent monogenic cerebral small-vessel arteriopathy. Despite wide availability of genetic testing, misdiagnosis and delay diagnosis are common. We encourage for enhanced awareness of CADASIL among patients with migraines with aura, recurrent TIAs or subcortical ischemic strokes, and mood disorder, in order to provide appropriate multidisciplinary treatment, psychological support and genetic counselingStudy Supported by: n/aDisclosure: Dr. Ortiz-Garcia has nothing to disclose. Dr. Orjuela has nothing to disclose. Dr. Sweis has nothing to disclose. Dr. Biller has received personal compensat...
Source: Neurology - April 17, 2017 Category: Neurology Authors: Ortiz-Garcia, J., Orjuela, K., Sweis, R., Biller, J. Tags: Genetic Stroke Syndromes, Biomarkers, and Translational/Basic Research Source Type: research

Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopthy (CADASIL) in Argentina (P2.123)
ConclusionsTo the best of our knowledge, this is the first cases series of patients with CADASIL in Argentina. Our findings are similar to those reported in the literature for other regions.Study Supported by:Disclosure: Dr. Hawkes has nothing to disclose. Dr. Wilken has nothing to disclose. Dr. Bruno has nothing to disclose. Dr. Pujol Lereis has nothing to disclose. Dr. Povedano has nothing to disclose. Dr. Taratuto has nothing to disclose. Dr. Taratuto has nothing to disclose. Dr. Ameriso has nothing to disclose.
Source: Neurology - April 9, 2014 Category: Neurology Authors: Hawkes, M., Wilken, M., Bruno, V., Pujol Lereis, V., Povedano, G., Lubieniecki, F., Taratuto, A. L., Ameriso, S. Tags: Cerebrovascular Disease and Interventional Neurology: Behavioral, Cognitive, and Miscellaneous Source Type: research

Fluctuating Mental Status and Fever of Unknown Origin (P1.030)
Conclusion: Central nervous system involvement as the initial manifestation of microscopic polyangiitis is rare and can result in a challenging clinical picture including recurrent ischemic strokes.Disclosure: Dr. Daniel has nothing to disclose. Dr. Brink has nothing to disclose. Dr. Hosley has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Daniel, A., Brink, J., Hosley, C. Tags: Cerebrovascular Disease and Interventional Neurology: Case Reports Source Type: research

A Case Of Hemophagocytic Lymphohistiocytosis Syndrome In A Patient With Multiple Sclerosis On Fingolimod Therapy. (P2.206)
CONCLUSIONS:As far as we know, this syndrome has never been reported in fingolimod treated patients. EBV reactivation may have been the triggering factor of HLH in our patient. Whether fingolimod contributed to HLH, through a direct mechanism, or indirectly favoring EBV reactivation, remains unknown. Our case highlights the diagnostic challenge of HLH and the need of monitoring and reporting serious adverse events in patients exposed to new IMD. Study Supported by:Disclosure: Dr. Abreu has received personal compensation for activities with Bayer Pharmaceuticals Corporation, Novartis, and Biogen Idec. Dr. Peixoto has nothin...
Source: Neurology - April 9, 2014 Category: Neurology Authors: Abreu, P., Peixoto, C., Carvalho, C., Santos, L., Sarmento, A., Jose Sa, M. Tags: MS and CNS Inflammatory Disease: Treatment Safety Source Type: research

Generalized Dilative Arteriopathy without Myopathy: A New Phenotype of Pompe Disease (P6.256)
Conclusion. This case demonstrates that LOPD may present as an isolated generalized dilative arteriopathy with repeated kidney and brain infarcts, and no myopathy. Pompe disease should be systematically screened in patients with generalized dilative arteriopathy.Disclosure: Dr. Echaniz-Laguna has received personal compensation for activities with Sanofi-Aventis Pharmaceuticals, Inc. Dr. Bataillard has nothing to disclose. Dr. Quenardelle has nothing to disclose.
Source: Neurology - April 9, 2014 Category: Neurology Authors: Echaniz-Laguna, A., Bataillard, M., Quenardelle, V. Tags: Cerebrovascular Disease and Interventional Neurology: Case Reports Source Type: research

Mystery Case: Cutaneous lesions in KRIT1-associated cerebral cavernous malformations
An asymptomatic 65-year-old man was seen because of a family history of cerebral cavernous malformations (CCM) and a positive test for the KRIT1/CCM1 (7q21.2) gene mutation. MRI of the brain showed multiple CCM (figure 1). The patient was noted to have innumerable skin lesions over the face, trunk, and limbs (figure 2). Cutaneous lesions occur in 9% of patients with familial CCM, particularly with KRIT1 mutations.1 These include red macules, nodular venous malformations, and hyperkeratotic cutaneous capillary-venous malformations.1 Skin biopsy shows collections of abnormal, dilated thin-walled blood vessels. Familial CCM s...
Source: Neurology - September 29, 2014 Category: Neurology Authors: Brownlee, W. J., Roxburgh, R. Tags: Other cerebrovascular disease/ Stroke, Other neurocutaneous disorders RESIDENT AND FELLOW SECTION Source Type: research

Brachial Plexus Neuritis as Presenting Manifestation of Disseminated Coccidioidomycosis. (P6.323)
CONCLUSIONS:This is the first reported case of coccidioidomycosis presenting with PNS involvement. Both remote inflammatory changes and direct local fungal invasion noted in this case highlight the wide spectrum of clinico-pathological presentations seen in coccidioidomycosis infection. As this fungal disease is identified with increasing frequency, clinicians should be aware of its atypical manifestations. Study Supported by: Disclosure: Dr. Shah has nothing to disclose. Dr. Liao has nothing to disclose. Dr. Shanina has nothing to disclose. Dr. Smith has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Shah, R., Liao, B., Shanina, E., Smith, G. Tags: Fungi, Parasites, and Other Infectious Disorders Source Type: research