Madsen, T. E., Khoury, J., Alwell, K., Moomaw, C. J., Rademacher, E., Flaherty, M. L., Woo, D., Mackey, J., De Los Rios La Rosa, F., Martini, S., Ferioli, S., Adeoye, O., Khatri, P., Broderick, J. P., Kissela, B. M., Kleindorfer, D. September 3, 2017

Objective:

Recent data suggest stroke incidence is decreasing over time, but it is unknown whether incidence is decreasing in women and men to the same extent.

Methods:

Within our population of 1.3 million, all incident strokes among residents ≥20 years old were ascertained at all hospitals during July 1993–June 1994 and calendar years 1999, 2005, and 2010.… Read More...

Byun, J.-I., Moon, J., Kim, D.-Y., Shin, H., Sunwoo, J.-S., Lim, J.-A., Kim, T.-J., Lee, W.-J., Lee, H. S., Jun, J.-S., Park, K.-I., Lee, S.-T., Jung, K.-H., Jung, K.-Y., Lee, S. K., Chu, K. September 3, 2017

Objective:

To evaluate the long-term (for up to 3 months) efficacy and safety of single or combined therapy with midodrine and pyridostigmine for neurogenic orthostatic hypotension (OH).

Methods:

This was a randomized, open-label clinical trial. In total, 87 patients with symptomatic neurogenic OH were enrolled and randomized to receive 1 of 3 treatments: midodrine only, pyridostigmine only, or midodrine + pyridostigmine.… Read More...

Giordano, I., Harmuth, F., Jacobi, H., Paap, B., Vielhaber, S., Machts, J., Schöls, L., Synofzik, M., Sturm, M., Tallaksen, C., Wedding, I. M., Boesch, S., Eigentler, A., van de Warrenburg, B., van Gaalen, J., Kamm, C., Dudesek, A., Kang, J.-S., Timmann, D., Silvestri, G., Masciullo, M., Klopstock, T., Neuhofer, C., Ganos, C., Filla, A., Bauer, P., Tezenas du Montcel, S., Klockgether, T. September 3, 2017

Objective:

To define the clinical phenotype and natural history of sporadic adult-onset degenerative ataxia and to identify putative disease-causing mutations.

Methods:

The primary measure of disease severity was the Scale for the Assessment and Rating of Ataxia (SARA). DNA samples were screened for mutations using a high-coverage ataxia-specific gene panel in combination with next-generation sequencing.… Read More...

Koton, S., Rexrode, K. M. September 3, 2017

Stroke poses a heavy burden on health systems, with serious implications for societies and economies. At the global level, stroke is the second leading cause of death and the third most common cause of disability.1 In the United States, about 795,000 strokes occur every year: on average, someone has a stroke every 40 seconds and someone dies as a result of a stroke every 4 minutes.… Read More...

Wardlaw, J. M., Chappell, F. M., Valdes Hernandez, M. d. C., Makin, S. D. J., Staals, J., Shuler, K., Thrippleton, M. J., Armitage, P. A., Munoz-Maniega, S., Heye, A. K., Sakka, E., Dennis, M. S. September 3, 2017

Objective:

To assess factors associated with white matter hyperintensity (WMH) change in a large cohort after observing obvious WMH shrinkage 1 year after minor stroke in several participants in a longitudinal study.

Methods:

We recruited participants with minor ischemic stroke and performed clinical assessments and brain MRI.… Read More...

Hehir, M. K., Hobson-Webb, L. D., Benatar, M., Barnett, C., Silvestri, N. J., Howard, J. F., Howard, D., Visser, A., Crum, B. A., Nowak, R., Beekman, R., Kumar, A., Ruzhansky, K., Chen, I.-H. A., Pulley, M. T., LaBoy, S. M., Fellman, M. A., Greene, S. M., Pasnoor, M., Burns, T. M. September 3, 2017

Objective:

To evaluate the efficacy of rituximab in treatment of anti-muscle-specific kinase (MuSK) myasthenia gravis (MG).

Methods:

This was a multicenter, blinded, prospective review, comparing anti-MuSK-positive patients with MG treated with rituximab to those not treated with rituximab. The primary clinical endpoint was the Myasthenia Gravis Status and Treatment Intensity (MGSTI), a novel outcome that combines the Myasthenia Gravis Foundation of America (MGFA) postintervention status (PIS) and the number and dosages of other immunosuppressant therapies used.… Read More...

Bril, V., Kaminski, H. J. September 3, 2017

A report of the Canadian Task Force on the Periodic Health Examination was the first to propose clinical practice recommendations based on evidence in the medical literature, and proposed a rating scheme.1 From that original publication, the categorization of evidence has evolved, with this journal requiring clinical therapeutic studies to provide the level of evidence provided by a report.… Read More...

Naik, N., Shah, A., Wamelink, M. M. C., van der Knaap, M. S., Hingwala, D. September 3, 2017

Ribose 5 phosphate isomerase deficiency (RPID) is a rare inborn error of metabolism in the pentose phosphate pathway (PPP)1 with a single case reported to date.2,3 The previously reported patient had leukoencephalopathy with a mild peripheral neuropathy. The diagnosis was established by demonstration of highly elevated levels of polyols by magnetic resonance spectroscopy (MRS) and body fluid analysis.… Read More...

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