Journal of neuroimaging : official journal of the American Society of Neuroimaging
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Case Reports
Brain imaging in cerebellar ataxia associated with autoimmune polyglandular syndrome type 2.
Autoimmune polyglandular syndrome (APS) type 2 (Schmidt syndrome) is a disorder characterized by a combination of autoimmune adrenal insufficiency, autoimmune thyroid disease, and type 1 autoimmune diabetes mellitus. We describe the first case of subacute cerebellar syndrome associated with APS type 2. ⋯ Magnetic resonance spectroscopy showed decreased N-acetylaspartate/creatine ratio in the cerebellum and in the pons. Our findings expand the spectrum of neurological deficits in APS type 2 and underlines that cerebellar pathways may be a main target of the disorder.
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Comparative Study
Determination of language dominance: Wada test and fMRI compared using a novel sentence task.
This study aimed to develop a new linguistic based functional magnetic resonance imaging (fMRI)-sentence decision task that reliably detects hemispheric language dominance. ⋯ The sentence task provides robust activations in putative essential language areas and can be used for visual analysis of predefined areas to facilitate interpretation of clinical fMRI.
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To evaluate the time periods of Wallerian degeneration (WD) in which the diffusion parameters of ipsilateral corticalspinal tract (CST) can be used to predict the motor function outcome after brain infarction. ⋯ The rFA and ipsilateral FA values after 2 weeks of stroke onset correlate with the motor function outcome.
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The second most frequently affected organ in mitochondrial disorders (MIDs) is the central nervous system (CNS). One of the most frequent CNS abnormalities on imaging is the affection of the white matter (WMLs) for which the term, leukoencephalopathies in mitochondrial disorders (LEM), is proposed. The morphology of LEM on imaging is quite variable even within the same type of MID and the same family. ⋯ WMLs in MIDs frequently give rise to misinterpretation, particularly if the mitochondrial defect is not evident in organs other than the CNS or if the presence of WMLs does not induce consideration of a MID as a differential. The diagnosis of a LEM requires the diagnosis of an MID, the detection of WMLs on imaging, and the exclusion of all possible differentials. Because the presence of LEM has an impact on the prognosis of an MID, all MID patients should undergo cerebral imaging even in the absence of clinical CNS manifestations.
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Case Reports
Potential role of PMD-TCD monitoring in the management of hemodynamically unstable intracranial stenosis.
A 54-year-old woman started to loose vision 2 days prior to admission and also experienced left-sided headache, nausea, emesis, and disorientation. Magnetic resonance imaging (MRI) revealed bilateral posterior cerebral artery and cerebellar infarctions. Transcranial power motion Doppler (PMD-TCD) showed blunted flow signal in the proximal basilar artery (BA) suggestive for a high-grade stenosis also seen on magnetic resonance angiography (MRA). ⋯ PMD-TCD findings were suggestive for an intraluminal thrombus that moved from the proximal to the distal basilar artery, presumably further contributing to brain stem hypoperfusion and neurological deterioration. To achieve a compromise between lower blood pressure and maintenance of brain perfusion, hypervolemic hemodilution with intravenous dextran-40 was initiated. Patient's symptoms resolved to baseline and MRI showed no new parenchymal lesions.