Andrew J. Degnan

Narrowing of Meckel's cave and cavernous sinus and enlargement of the optic nerve sheath in Pseudotumor Cerebri.

Objective: Pseudotumor cerebri (PTC) is a clinical entity of uncertain etiology associated with several subtle findings on magnetic resonance imaging (MRI) including posterior flattening of the globes, enlargement of the optic nerve sheath (ONS), empty sella sign. We aimed to characterize the incidence of and significance of 2 novel MRI findings: narrowing of Meckels cave and of cavernous sinus. Methods: Forty-six patients with a condition diagnosed as PTC based on clinical history were retrospectively reviewed, and their MRI studies were assessed for previously reported imaging findings associated with PTC. The maximal diameters of the cavernous sinuses, Meckels caves, and ONSs were measured along with those of age-matched controls on axial T2-weighted images. Results: The Meckels caves and cavernous sinuses are significantly (P < 0.01) narrowed in patients (mean diameters: 0.41 and 0.25 cm) versus controls (0.54 and 0.36 cm), respectively. The ONS was enlarged in patients with a mean diameter of 0.65 cm versus 0.54 cm (P < 0.01). Meckels cave narrowing and ONS enlargement seem to be better indicators of PTC than cavernous sinus narrowing, with sensitivities of 78.3% and 86.9% and specificities of 84.8% and 76.1% versus 60.9% and 76.1%, respectively. Conclusions: This finding of narrowed Meckels caves in PTC may be clinically useful as a novel imaging finding seen on routine MRI studies. Optic nerve sheath enlargement is also confirmed as an important finding in PTC.

Evaluation of Ultrasmall Superparamagnetic Iron Oxide-Enhanced MRI of Carotid Atherosclerosis to Assess Risk of Cerebrovascular and Cardiovascular Events: Follow-Up of the ATHEROMA Trial

moderate carotid stenosis. The study randomly assigned patients into two treatment arms, low(10 mg) and high-dose (80 mg) atorvastatin therapy, over 12 weeks, and assessed the dose-response interaction [10] . The purpose of this preliminary evaluation of long-term follow-up is to report on the ability of initial USPIO-enhanced MRI to predict subsequent cerebrovascular and cardiovascular morbidity and mortality.

Independent component analysis of resting state activity in pediatric obsessive-compulsive disorder.

Obsessive‐compulsive disorder (OCD) is an often severely disabling illness with onset generally in childhood or adolescence. Little is known, however, regarding the pattern of brain resting state activity in OCD early in the course of illness. We therefore examined differences in brain resting state activity in patients with pediatric OCD compared with healthy volunteers and their clinical correlates. Twenty‐three pediatric OCD patients and 23 healthy volunteers (age range 9–17), matched for sex, age, handedness, and IQ completed a resting state functional magnetic resonance imaging exam at 3T. Patients completed the Childrens Yale Brown Obsessive Scale. Data were decomposed into 36 functional networks using spatial group independent component analysis (ICA) and logistic regression was used to identify the components that yielded maximum group separation. Using ICA we identified three components that maximally separated the groups: a middle frontal/dorsal anterior cingulate network, an anterior/posterior cingulate network, and a visual network yielding an overall group classification of 76.1% (sensitivity = 78.3% and specificity = 73.9%). Independent component expression scores were significantly higher in patients compared with healthy volunteers in the middle frontal/dorsal anterior cingulate and the anterior/posterior cingulate networks, but lower in patients within the visual network. Higher expression scores in the anterior/posterior cingulate network correlated with greater severity of compulsions among patients. These findings implicate resting state fMRI abnormalities within the cingulate cortex and related control regions in the pathogenesis and phenomenology of OCD early in the course of the disorder and prior to extensive pharmacologic intervention. Hum Brain Mapp 35:5306–5315, 2014.

Early Metabolic Development of Posteromedial Cortex and Thalamus in Humans Analyzed Via In Vivo Quantitative Magnetic Resonance Spectroscopy

The posteromedial cortex (PMC) including the posterior cingulate, retrosplenial cortex, and medial parietal cortex/precuneus is an epicenter of cortical interactions in a wide spectrum of neural activity. Anatomic connections between PMC and thalamic components have been established in animal studies, but similar studies do not exist for the fetal and neonatal period. Magnetic resonance spectroscopy (MRS) allows for noninvasive measurement of metabolites in early development. Using single‐voxel 3‐T MRS, healthy term neonates (n = 31, mean postconception age 41.5 weeks ± 3.8 weeks) were compared with control children (n = 23, mean age 9.4 years ± 5.1 years) and young adults (n = 10, mean age 24.1 years ± 2.6 years). LCModel‐based calculations compared metabolites within medial parietal gray matter (colocalizing to the PMC), posterior thalamus, and parietal white matter voxels. Common metabolic changes existed for neuronal−axonal maturation and structural markers in the PMC, thalamus, and parietal white matter with increasing NAA and glutamate and decreasing myoinositol and choline with age. Key differences in creatine and glucose metabolism were noted in the PMC, in contrast to the thalamic and parietal white matter locations, suggesting a unique role of energy metabolism. Significant parallel metabolite developmental changes of multiple other metabolites including aspartate, glutamine, and glutathione with age were present between PMC and parietal white matter but not between PMC and thalamus. These findings offer insight into the metabolic architecture of the interface between structural and functional topology of brain networks. Further investigation unifying metabolic changes with functional and anatomic pathways may further enhance the understanding of the PMC in posterior default mode network development. J. Comp. Neurol. 522:3717–3732, 2014.

Anatomic olfactory structural abnormalities in congenital smell loss: magnetic resonance imaging evaluation of olfactory bulb, groove, sulcal, and hippocampal morphology.

Background and Purpose There are 2 groups of patients with congenital smell loss: group 1 (12% of the total), in which patients exhibit a familial smell loss in conjunction with severe anatomical, somatic, neurological, and metabolic abnormalities such as hypogonadotropic hypogonadism; and a larger group, group 2 (88% of the total), in which patients possess a similar degree of smell loss but without somatic, neurological, or anatomical abnormalities or hypogonadism. Both groups are characterized by similar olfactory dysfunction, and both have been reported to have absent or decreased olfactory bulbs and grooves, which indicates some overlap in olfactory pathophysiology and anatomy. The purpose of this study was to evaluate patients with congenital smell loss, primarily among group 2 patients, comparing brain magnetic resonance imaging (MRI) results in patients with types of hyposmia. Methods Forty group 2 patients were studied by measurements of taste (gustometry) and smell (olfactometry) function and by use of MRI in which measurements of olfactory bulbs, olfactory sulcus depth, olfactory grooves, and hippocampal anatomy were performed. Anatomical results were compared with similar studies in group 1 patients and in 22 control subjects with normal sensory function. Results Olfactometry was abnormal in all patients with no patient reporting ever having normal olfaction. No patient had a familial history of smell loss. On MRI, all exhibited at least 1 abnormality in olfactory system anatomy, including absence or decreased size of at least 1 olfactory bulb, decreased depth of an olfactory sulcus, and abnormalities involving hippocampal anatomy with hippocampal malrotations. One patient had bilateral bulb duplication. Normal subjects with normal smell and taste function exhibited some but very few or significant neuroanatomical changes on MRI. Conclusions Although both groups have similar abnormalities of smell function, group 2 patients demonstrate anatomical anomalies in olfactory structures that are neither as common nor as severe as in group 1 patients. Group 2 patients can have a wide range of olfactory anatomical abnormalities.

Advances in noninvasive imaging for evaluating clinical risk and guiding therapy in carotid atherosclerosis

Managing asymptomatic carotid atherosclerosis with a view to preventing ischemic stroke is a challenging task. As the annual risk of stroke in untreated asymptomatic patients on average is less than the risk of surgical intervention, the key question is how to identify those asymptomatic individuals whose risk of stroke is elevated and who would benefit from surgery, while sparing low-risk asymptomatic patients from the risks of surgical intervention. The advent of a multitude of noninvasive carotid imaging techniques offers an opportunity to improve risk stratification in patients and to monitor the response to medical therapies; assessing efficacy at individual and population levels. As part of this, plaque measurement techniques (using ultrasound, computed tomography or MRI) may be employed in monitoring plaque/component regression and progression. Novel imaging applications targeted to plaque characteristics, inflammation and neovascularization, including contrast-enhanced ultrasound and MRI, dynamic contrast-enhanced MRI, and fluorodeoxyglucose-PET, are also being explored. Ultimately, noninvasive imaging and other advances in risk stratification aim to improve and individualize the management of patients with carotid atherosclerosis.

Alterations of resting state networks and structural connectivity in relation to the prefrontal and anterior cingulate cortices in late prematurity.

Late preterm birth is increasingly recognized as a risk factor for cognitive and social deficits. The prefrontal cortex is particularly vulnerable to injury in late prematurity because of its protracted development and extensive cortical connections. Our study examined children born late preterm without access to advanced postnatal care to assess structural and functional connectivity related to the prefrontal cortex. Thirty-eight preadolescents [19 born late preterm (34–36 6/7 weeks gestational age) and 19 at term] were recruited from a developing community in Brazil. Participants underwent neuropsychological testing. Individuals underwent three-dimensional T1-weighted, diffusion-weighted, and resting state functional MRI. Probabilistic tractography and functional connectivity analyses were carried out using unilateral seeds combining the medial prefrontal cortex and the anterior cingulate cortex. Late preterm children showed increased functional connectivity within regions of the default mode, salience, and central-executive networks from both right and left frontal cortex seeds. Decreased functional connectivity was observed within the right parahippocampal region from left frontal seeding. Probabilistic tractography showed a pattern of decreased streamlines in frontal white matter pathways and the corpus callosum, but also increased streamlines in the left orbitofrontal white matter and the right frontal white matter when seeded from the right. Late preterm children and term control children scored similarly on neuropsychological testing. Prefrontal cortical connectivity is altered in late prematurity, with hyperconnectivity observed in key resting state networks in the absence of neuropsychological deficits. Abnormal structural connectivity indicated by probabilistic tractography suggests subtle changes in white matter development, implying disruption of normal maturation during the late gestational period.

Altered Structural and Functional Connectivity in Late Preterm Preadolescence: An Anatomic Seed-Based Study of Resting State Networks Related to the Posteromedial and Lateral Parietal Cortex

Objective Late preterm birth confers increased risk of developmental delay, academic difficulties and social deficits. The late third trimester may represent a critical period of development of neural networks including the default mode network (DMN), which is essential to normal cognition. Our objective is to identify functional and structural connectivity differences in the posteromedial cortex related to late preterm birth. Methods Thirty-eight preadolescents (ages 9–13; 19 born in the late preterm period (≥32 weeks gestational age) and 19 at term) without access to advanced neonatal care were recruited from a low socioeconomic status community in Brazil. Participants underwent neurocognitive testing, 3-dimensional T1-weighted imaging, diffusion-weighted imaging and resting state functional MRI (RS-fMRI). Seed-based probabilistic diffusion tractography and RS-fMRI analyses were performed using unilateral seeds within the posterior DMN (posterior cingulate cortex, precuneus) and lateral parietal DMN (superior marginal and angular gyri). Results Late preterm children demonstrated increased functional connectivity within the posterior default mode networks and increased anti-correlation with the central-executive network when seeded from the posteromedial cortex (PMC). Key differences were demonstrated between PMC components with increased anti-correlation with the salience network seen only with posterior cingulate cortex seeding but not with precuneus seeding. Probabilistic tractography showed increased streamlines within the right inferior longitudinal fasciculus and inferior fronto-occipital fasciculus within late preterm children while decreased intrahemispheric streamlines were also observed. No significant differences in neurocognitive testing were demonstrated between groups. Conclusion Late preterm preadolescence is associated with altered functional connectivity from the PMC and lateral parietal cortex to known distributed functional cortical networks despite no significant executive neurocognitive differences. Selective increased structural connectivity was observed in the setting of decreased posterior interhemispheric connections. Future work is needed to determine if these findings represent a compensatory adaptation employing alternate neural circuitry or could reflect subtle pathology resulting in emotional processing deficits not seen with neurocognitive testing.

Neuroimaging of Rapidly Progressive Dementias, Part 1: Neurodegenerative Etiologies

SUMMARY: Most dementias begin insidiously, developing slowly and generally occurring in the elderly age group. The so-called rapidly progressive dementias constitute a different, diverse collection of conditions, many of which are reversible or treatable. For this reason, prompt identification and assessment of acute and subacute forms of dementia are critical to effective treatment. Numerous other entities within this category of presenile rapid-onset dementias are untreatable such as the prion-related diseases. Neuroimaging aids in the diagnosis and evaluation of many of these rapidly progressive dementias, which include myriad conditions ranging from variations of more common neurodegenerative dementias, such as Alzheimer disease, dementia with Lewy bodies, and frontotemporal dementia; infectious-related dementias such as acquired immune deficiency syndrome dementia; autoimmune and malignancy-related conditions; to toxic and metabolic forms of encephalopathy. This first of a 2-part review will specifically address the ability of MR imaging and ancillary neuroimaging strategies to support the diagnostic evaluation of rapidly progressive dementias due to neurodegenerative causes.

Neuroimaging of Rapidly Progressive Dementias, Part 2: Prion, Inflammatory, Neoplastic, and Other Etiologies

SUMMARY: Most dementias begin insidiously, developing slowly and generally occurring in the elderly age group. The so-called rapidly progressive dementias constitute a different, diverse collection of conditions, many of which are reversible or treatable. For this reason, accurate identification and assessment of acute and subacute forms of dementia are critical to effective treatment; neuroimaging aids greatly in narrowing the diagnosis of these conditions. This second installment of a 2-part review of rapidly progressive dementias examines the use of imaging in an assortment of other etiologies in the differential diagnosis, from prion disease and neoplastic-related conditions to rare metabolic and other conditions such as Wernicke encephalopathy. In these clinical conditions, MR imaging has the potential to narrow this broad differential diagnosis and, at times, can definitively aid in the diagnosis of certain conditions on the basis of typical imaging patterns.