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Background: Fabry disease is an X-linked lysosomal storage disorder caused by mutations in GLA, which encodes the enzyme α-galactosidase A (α-Gal A). Although the prevalence of Fabry disease in patients with stroke has been reported to range from 0% to 4%, few cohort studies have examined Japanese stroke patients. We aimed to clarify the prevalence of Fabry disease and the frequency of GLA mutations among patients with young-onset stroke in Japan.Methods: From April 2015 to December 2016, we enrolled patients with young-onset (≤60 years old) ischemic stroke or intracerebral hemorrhage. We measured α-Gal A activity and the concentration of globotriaosylsphingosine in plasma. Genetic evaluations were performed in patients with low α-Gal A activity or high concentrations of globotriaosylsphingosine.Results: Overall, 516 patients (median age of onset, 52 years old; 120 women) were consecutively enrolled in this study. Five patients (4 men and 1 woman) had low α-Gal A activity, and no patients were detected with the screen for plasma globotriaosylsphingosine levels. The genetic analysis did not identify a causative mutation responsible for classic Fabry disease in any of the patients, but 2 patients (.4%) carried the p.E66Q in GLA.Conclusions: No patient with Fabry disease was detected in our young-onset stroke cohort. [ABSTRACT FROM AUTHOR]

A new version of an air abrasion device, which expelled abrasive sodium bicarbonate powder, was fabricated to remove carious tissue. This device had a motor-driven mechanism to control the amount of powder expelled. The purpose of this study was to estimate the abrading efficacy of this device, in particular the new jet nozzle, for removal of carious dentin. Powder was supplied to the handpiece by a rotary gear attached to the powder control motor. Two types of jet nozzles, a cylinder-type and a bugle-type, were fabricated and evaluated. The duct of bugle-type nozzle had an isthmus to increase the air pressure on the powder and to spread it out in a cone-shaped flow. Results obtained showed that the bugle-type jet nozzle exhibited a more prominent abrasive capability. Compared to the cylinder-type nozzle, it was thus more effective in removing the carious dentin which remained in the undercut region of the cavities. [ABSTRACT FROM AUTHOR]

Objectives: The present study aimed to examine the effectiveness of proton magnetic resonance spectroscopy (1HMRS) in determining the progression of neurological symptoms resulting in acute ischemic stroke in patients with lenticulostriate artery (LSA) infarction.Materials and Methods: 1HMRS was performed within 72 h after neurological symptom onset. Voxel of interest was placed in tissue that included the pyramidal tract and identified diffusion weighted echo planar spin-echo sequence (DWI) coronal images. Infarct volume in DWI was calculated using the ABC/2 method. 1HMRS data (tNAA, tCr, Glx, tCho, and Ins) were analyzed using LCModel. Progressive neurological symptoms were defined as an increase of 1 or more in the NIHSS score. Patients who underwent 1HMRS after progressive neurological symptoms were excluded.Results: In total, 77 patients were enrolled. Of these, 19 patients had progressive neurological symptoms. The patients with progressive neurological symptoms were significantly more likely to be female and had higher tCho/tCr values, higher rates of axial slices ≥ 3 slices on DWI, higher infarct volume on DWI, higher maximum diameter of infarction of axial slice on DWI, and higher SBP on admission compared to those without. Multivariable logistic analysis revealed that higher tCho/tCr values were independently associated with progressive neurological symptoms after adjusting for age, sex, and initial DWI infarct volume (tCho/tCr per 0.01 increase, OR 1.26, 95% CI 1.03-1.52, P = 0.022).Conclusions: Increased tCho/tCr score were associated with progressive neurological symptoms in patients with LSA ischemic stroke. Quantitative evaluation of 1HMRS parameters may be useful for predicting the progression of neurological symptoms. [ABSTRACT FROM AUTHOR]

A 63-year-old woman with end-stage renal disease on maintenance hemodialysis discontinued her medication for rheumatoid arthritis with prednisolone and azathioprine. One month later, she was admitted because of consciousness disturbance and right hemiparesis. Diffusion-weighted brain magnetic resonance imaging (MRI) revealed multiple hyperintensities in her left frontal and parietal lobes. She also developed high fever and left neck pain. Carotid ultrasonography showed calcified plaque with vessel wall swelling at the bifurcation of the left common carotid artery (LCCA) and surrounding hypoechoic soft tissue. The tissue was identified as an isodense lesion on noncontrast computed tomography (CT) and as a high-intensity lesion on fat-saturated T2-weighted MRI. From her symptoms and radiological findings, she was diagnosed with carotidynia. Cervical MRI also showed that the LCCA was transposed to a retropharyngeal location, suggesting a moving carotid artery. Carotid ultrasonography revealed that the LCCA moved to and from the retropharyngeal position with swallowing and was thus being compressed by the hyoid bone. After corticosteroid therapy was initiated with 30 mg of prednisolone, her symptoms and radiological findings improved. To our knowledge, this is the first report of a case of cerebral embolism due to carotidynia. The repetitive compressions by the hyoid bone during swallowing were presumed to have provoked shear stress and inflammation of the carotid vessel wall, which was aggravated by discontinuation of steroid therapy in our case. These mechanical and inflammatory stresses might cause dysfunction of endothelial cells, hypercoagulation, platelet hyperaggregation, and vulnerability and rupture of carotid plaques, and may subsequently result in embolic strokes. [ABSTRACT FROM AUTHOR]