MR angiographic findings of aberrant right subclavian artery with agenesis of the left common carotid artery and anomalous origin of the vertebral arteries.

INTRODUCTION: Congenital aortic arch anomalies and variants have been extensively characterized in the medical literature. Proper identification of these anomalies is important when surgical or percutaneous interventions are indicated. CASE PRESENTATION: We present a case of a 48-year old male who presented to the emergency department with altered mental status. Magnetic resonance angiography (MRA) findings revealed an aberrant right subclavian artery (ARSA), early bifurcation of the right common carotid artery (CCA) with anomalous origin of the right vertebral artery (VA) from the right common carotid artery bifurcation, anomalous left vertebral artery originating from the aortic arch, and absent left common carotid artery with independent origins of the left external carotid artery (ECA) and internal carotid artery (ICA). No other abnormalities were identified, and the patient demonstrated no symptoms attributable to his vascular anomalies. CONCLUSION: To our knowledge, this unique combination of anomalies has never been reported in the literature. With an understanding of embryological pathways, even exceedingly rare anomalies like this one can be explained.

Detection of subthreshold atrophy in crossed cerebellar degeneration via two-compartment mathematical modeling of cell density in DWI: A proof of concept study.

Crossed cerebellar diaschisis (CCD) refers to transneuronal degeneration of the corticopontocerebellar pathway, resulting in atrophy of cerebellum contralateral to supratentorial pathology. CCD is traditionally diagnosed on nuclear medicine studies. Our aim is to apply a biexponential diffusion model, composed of intracellular and extracellular compartments, to the detection of subthreshold CCD on DWI, with the calculated fraction of the intracellular compartment as a proposed measure of cell density. At a voxel-by-voxel basis, we solve for intracellular and extracellular coefficients in each side of the cerebellum and compare the distribution of coefficients between each hemisphere. We demonstrate, in all six CCD cases, a significantly lower contribution of the intracellular compartment to the cerebellar hemisphere contralateral to supratentorial pathology (p < 0.01). In a separate, proof-of-concept case of pontine stroke, we also demonstrate reduced intracellular coefficients in bilateral cerebellar hemispheres, excluding middle cerebellar peduncles (p < 0.01). Our findings are consistent with a decreased intracellular fraction, presumably a surrogate for reduced cellular density in corticopontocerebellar degeneration, despite normal-appearing scans. Our approach allows detection of subthreshold structural changes and offers the additional advantage of applicability to most clinical cases, where only three DWI beta values are available.

An unusual case of cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy with occipital lobe involvement.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant angiopathy caused by a mutation in the notch 3 gene on chromosome 19. Clinically, patients may be asymptomatic or can present with recurrent ischemic episodes and strokes leading to dementia, depression, pseudobulbar palsy, and hemi- or quadraplegia. Additional manifestations that have been described include migraine (mostly with aura), psychiatric disturbances, and epileptic seizures. Neuroimaging is essential to the diagnosis of CADASIL. On imaging CADASIL is characterized by symmetric involvement by confluent lesions located subcortically in the frontal and temporal lobes as well as in the insula, periventricularly, in the centrum semiovale, in the internal and external capsule, basal ganglia, and brain stem; with relative sparing of the fronto-orbital and the occipital subcortical regions. We describe a 49 year old male with CADASIL with absence of temporal lobe findings on MRI but predominant lesions within the periventricular white matter, occipital lobes with extension into the subcortical frontal lobes, corpus callosum and cerebellar white matter. Although CADASIL characteristically presents with anterior temporal lobe involvement, these findings may be absent and our case addresses the atypical imaging findings in CADASIL.

Complex imaging features of accidental cerebral intraventricular gadolinium administration.

Gadolinium diethylenetriamine pentaacetic acid (Gd-DTPA) is a contrast agent commonly used for enhancing MRI. In this paper, the authors report on 2 cases of postoperative inadvertent administration of Gd-DTPA directly into a ventriculostomy tubing side port that was mistaken for intravenous tubing. Both cases demonstrated a low signal on MRI throughout the ventricular system and dependent portions of the subarachnoid spaces, which was originally believed to be CSF with areas of T1 shortening in the nondependent portions of the subarachnoid spaces, and misinterpreted as basal leptomeningeal enhancement and meningitis. The authors propose that the appearance of profound T1 hypointensity within the ventricles and diffuse susceptibility artifact along the ependyma is pathognomonic of intraventricular Gd-DTPA and should be recognized.

Parameter optimization for quantitative signal-concentration mapping using spoiled gradient echo MRI.

Rationale and Objectives. Accurate signal to tracer concentration maps are critical to quantitative MRI. The purpose of this study was to evaluate and optimize spoiled gradient echo (SPGR) MR sequences for the use of gadolinium (Gd-DTPA) as a kinetic tracer. Methods. Water-gadolinium phantoms were constructed for a physiologic range of gadolinium concentrations. Observed and calculated SPGR signal to concentration curves were generated. Using a percentage error determination, optimal pulse parameters for signal to concentration mapping were obtained. Results. The accuracy of the SPGR equation is a function of the chosen MR pulse parameters, particularly the time to repetition (TR) and the flip angle (FA). At all experimental values of TR, increasing FA decreases the ratio between observed and calculated signals. Conversely, for a constant FA, increasing TR increases this ratio. Using optimized pulse parameter sets, it is possible to achieve excellent accuracy (approximately 5%) over a physiologic range of concentration tracer concentrations. Conclusion. Optimal pulse parameter sets exist and their use is essential for deriving accurate signal to concentration curves in quantitative MRI.

A medley of midbrain maladies: a brief review of midbrain anatomy and syndromology for radiologists.

The midbrain represents the uppermost portion of the brainstem, containing numerous important nuclei and white matter tracts, most of which are involved in motor control, as well as the auditory and visual pathways. Notable midbrain nuclei include the superior and inferior colliculus nuclei, red nucleus, substantia nigra, oculomotor nuclear complex, and trochlear nucleus. In addition, white matter tracts include the brachium conjunctivum, medial and lateral lemniscus, spinothalamic tracts, and the fiber tracts within the cerebral peduncles. Although neurologically vital, many of these small midbrain nuclei and white matter tracts are not easily individually identified on neuroimaging. However, given their diverse functions, midbrain pathology often leads to distinct clinical syndromes. A review and understanding of the location and relationships between the different midbrain nuclei and fiber tracts will allow more precise correlation of radiologic findings with patient pathology and symptomatology. Particular syndromes associated with midbrain pathology include the Weber, Claude, Benedikt, Nothnagel, and Parinaud syndromes. The oculomotor and trochlear cranial nerves also reside at this level. An understanding of their functions as well as their projected courses from the midbrain towards the eye allows identification of distinct locations which are particularly vulnerable to pathology.

Dominant posterior-variant alien hand syndrome after acute left parietal infarction.

Alien hand syndrome (AHS) is a complex clinical disorder in which patients develop a sense of estrangement from, and loss of volitional control of, an affected limb and non-purposeful complex motor actions of the same limb. Several forms of AHS exist, including frontal, callosal, and posterior types with a series of different associated neuroanatomical lesions and clinical symptoms. Most commonly, the lesions associated with AHS occur in the frontal lobes and corpus callosum. Rarely, lesions in the parietal lobes may be associated with AHS, and most often occur within the non-dominant hemisphere. We describe a 57-year-old patient who developed symptoms of posterior AHS after an acute infarction in the left (presumably dominant) parietal lobe. A review of the different clinical features of AHS and the underlying mechanisms is also presented.

Midbrain ataxia: an introduction to the mesencephalic locomotor region and the pedunculopontine nucleus.

OBJECTIVE: Although gait ataxia is usually associated with cerebellar lesions, we review a less familiar cause. We present three patients with dorsal midbrain lesions and correlate these presentations with recent findings in the functional anatomy of the midbrain. CONCLUSION: We suggest that these lesions involve a well-studied but generally unfamiliar area of the dorsal midbrain known as the mesencephalic locomotor region. More specifically, we hypothesize that involvement of the pedunculopontine nucleus, a major component of the mesencephalic locomotor region, may be at least partially responsible for producing midbrain ataxia.

Sonographic NASCET index: a new doppler parameter for assessment of internal carotid artery stenosis.

BACKGROUND AND PURPOSE: Established Doppler parameters for carotid stenosis assessment do not reflect North American Symptomatic Carotid Endarterectomy Trial (NASCET)-style methodology. We derived a Doppler parameter, termed sonographic NASCET index (SNI), and hypothesized that the SNI would provide greater angiographic correlation and better accuracy in predicting stenosis of 70% or greater than that of currently used peak systolic velocity (PSV) measurements. METHODS: Inclusion criteria of angiographically proved carotid stenoses of 40-95% and measured proximal and distal internal carotid artery Doppler PSV values were established. Occlusions and near occlusions were specifically excluded. Doppler and angiographic data meeting the inclusion criteria from 32 carotid bifurcations were identified; actual angiographic stenoses ranged 40-89%. SNI values were calculated for each vessel. PSV and SNI were correlated with angiography by using linear regression analysis. Accuracies of SNI and PSV in predicting stenosis of 70% or greater were compared at two thresholds. RESULTS: Correlation between SNI and angiography was superior to that between PSV and angiography (r2=0.64 vs 0.38). PSV and SNI values that corresponded to 70% angiographic stenosis were 345 cm/s and 45.5, respectively. Accuracy of PSV of 345 cm/s or greater in predicting stenosis of 70% or greater was 78%, compared with 88% for SNI of 45.5 or greater. The SNI value that corresponded to a PSV threshold of 250 cm/s was 33. Accuracy of PSV of 250 cm/s or greater in predicting stenosis of 70% or greater was 81%, compared with 88% for SNI of 33 or greater. CONCLUSION: Correlation between SNI and angiography was greater than that between PSV and angiography. Accuracy of SNI in predicting stenosis of 70% or greater was also superior to that of PSV at two thresholds. These results suggest that SNI may be a better predictor of high-grade carotid stenosis than is PSV.

Accuracy of contrast-enhanced MR angiography in predicting angiographic stenosis of the internal carotid artery: linear regression analysis.

BACKGROUND AND PURPOSE: We sought to assess whether contrast-enhanced MR angiography is able to predict the degree of angiographic stenosis of the internal carotid artery within a clinically acceptable margin of error, thereby decreasing the need for angiography. In addition, we sought to assess whether adding ultrasound peak systolic velocity (PSV) as an additional regressor improves the accuracy of prediction. METHODS: A retrospective review of our institution's records for a 4-year period was conducted to identify all patients who had undergone evaluation of their carotid arteries using digital subtraction angiography, contrast-enhanced MR angiography, and ultrasonography. All internal carotid artery stenoses ranging from 10% to 90% at carotid angiography were selected (n = 22). Measurements were then obtained based on the North American Symptomatic Carotid Endarterectomy Trial style by using the digital subtraction angiograms and contrast-enhanced MR angiograms in a blinded fashion. The correlation between digital subtraction angiography data and contrast-enhanced MR angiography data was assessed by conducting linear regression analysis. Multiple regression analysis was then conducted to determine whether the inclusion of ultrasound PSV as an additional regressor increased the accuracy of prediction. RESULTS: The correlation between the degree of stenosis measured by digital subtraction angiography and that measured by contrast-enhanced MR angiography was r = 0.967. The 95% confidence interval for the line of means showed low errors bounds, ranging as low as +/-2.8%. The 95% confidence interval for individual prediction of angiographic stenosis based on a given contrast-enhanced MR angiographic measurement, however, was significantly larger, being no less than +/-13.6%. With the inclusion of PSV, the adjusted correlation was r = 0.965. CONCLUSION: A clear linear relationship exists between digital subtraction angiographic and contrast-enhanced MR angiographic measurements of carotid stenosis. Increasing severity of stenosis as measured by contrast-enhanced MR angiography corresponds to increasing severity at angiography. Although the predictive value of contrast-enhanced MR angiography is excellent in the mean, it is less reliable for predicting the degree of angiographic stenosis in the individual patient, showing rather wide confidence intervals. Furthermore, the inclusion of PSV as an additional regressor does not improve the predictive accuracy beyond that of contrast-enhanced MR angiography alone.