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This report discusses the syndrome of amnionic bands, anencephaly, schizencephaly and hydranencephaly, four entities whose pathogenesis includes significant injury to the fetus in utero.

Involvement of the central nervous system by multiple myeloma is rare and has a very poor prognosis. Magnetic resonance imaging of the head and spine plays an important role in diagnosing central nervous system involvement with multiple myeloma. We retrospectively analyzed the imaging features of pathology-proven extraosseous intracranial and intraspinal multiple myeloma from 2002 to 2013. The most common imaging manifestations were extraaxial nonosseous spinal lesions, cranial nerve involvement, and intracranial extraaxial nonosseous lesions. Different sites in the central nervous system may be affected by multiple myeloma, often simultaneously, producing a variety of imaging appearances., (Copyright © 2015 Elsevier Inc. All rights reserved.)

MR imaging features of mitochondrial encephalomyopathies, lactic acidosis, and stroke-like episodes, Kearns-Sayre/Pearson syndrome have been described in the literature. We describe extensive white matter changes with abnormal signal intensity lesions involving the deep gray nuclei and myelinated white matter tracts in an 18-year-old female with a large-scale 7.4 kb mitochondrial DNA deletion and a atypical presentation of Kearns-Sayre syndrome. Restricted diffusion due to status spongiosus at the involved sites is also discussed.

This article presents the first series of MRI-based anatomically labeled sectioned images of the brain of the killer whale (Orcinus orca). Magnetic resonance images of the brain of an adult killer whale were acquired in the coronal and axial planes. The gross morphology of the killer whale brain is comparable in some respects to that of other odontocete brains, including the unusual spatial arrangement of midbrain structures. There are also intriguing differences. Cerebral hemispheres appear extremely convoluted and, in contrast to smaller cetacean species, the killer whale brain possesses an exceptional degree of cortical elaboration in the insular cortex, temporal operculum, and the cortical limbic lobe. The functional and evolutionary implications of these features are discussed., ((c) 2004 Wiley-Liss, Inc.)

Background: Acute intermittent porphyria (AIP), due to half-normal hydroxymethylbilane synthase activity,is characterized by acute life-threatening neurologic attacks whose etiology remains unclear. To date, only 3 patients confirmed to have homozygous dominant AIP (HD-AIP) have been described (hydroxymethylbilane synthase genotypes R167Q/R167Q and R167W/R173Q)., Objective: To investigate the genetic, biochemical, clinical, and neuroradiologic features of a severely affected infant with HD-AIP., Design: Clinical, imaging, and genotype/phenotype studies were performed., Results: The proband, homoallelic for hydroxymethylbilane synthase mutation R167W, had approximately 1% of normal hydroxymethylbilane synthase activity, elevated porphyrins and porphyrin precursors, severe psychomotor delay, and central and peripheral neurologic manifestations. When expressed in vitro, the R167W mutant enzyme had less than 2% of normal activity but was markedly unstable, consistent with the proband's severe phenotype. Mitochondrial respiratory chain enzymes were normal. Neuroradiologic studies revealed a unique pattern of deep cerebral white matter injury, with relative preservation of the corpus callosum, anterior limb of the internal capsule, cerebral gray matter, and infratentorial structures., Conclusions: This severely affected patient with HD-AIP expanded the phenotypic spectrum of HD-AIP. His brain magnetic resonance imaging studies suggested selective cerebral oligodendrocyte postnatal involvement in HD-AIP, whereas most structures developed prenatally were intact. These findings indicate that the neurologic manifestations result from porphyrin precursor toxicity rather than heme deficiency and suggest that porphyrin precursor toxicity is primarily responsible for the acute neurologic attacks in heterozygous AIP and other porphyrias.

This report presents data regarding the brain structure of mountain gorillas (Gorilla beringei beringei) in comparison with other great apes. Magnetic resonance (MR) images of three mountain gorilla brains were obtained with a 3T scanner, and the volume of major neuroanatomical structures (neocortical gray matter, hippocampus, thalamus, striatum, and cerebellum) was measured. These data were included with our existing database that includes 23 chimpanzees, three western lowland gorillas, and six orangutans. We defined a multidimensional space by calculating the principal components (PCs) from the correlation matrix of brain structure fractions in the well-represented sample of chimpanzees. We then plotted data from all of the taxa in this space to examine phyletic variation in neural organization. Most of the variance in mountain gorillas, as well as other great apes, was contained within the chimpanzee range along the first two PCs, which accounted for 61.73% of the total variance. Thus, the majority of interspecific variation in brain structure observed among these ape taxa was no greater than the within-species variation seen in chimpanzees. The loadings on PCs indicated that the brain structure of great apes differs among taxa mostly in the relative sizes of the striatum, cerebellum, and hippocampus. These findings suggest possible functional differences among taxa in terms of neural adaptations for ecological and locomotor capacities. Importantly, these results fill a critical gap in current knowledge regarding great ape neuroanatomical diversity.

MR microscopy at 9.4T depicts the architecture of the brain in exquisite detail, including the individual laminae of the cortex, the individual nuclei of the basal ganglia, the thalami, subthalami and metathalami, and the orientations and relationship among the dominant nuclei and white matter tracts of the brain. The authors believe that these anatomic relations will ultimately be displayed in vivo as clinical MR scanners begin to operate at field strengths of 4.7T, 7T, and 8T. Then, those familiar with this anatomy will be able to interpret patient images with far greater sophistication.

The object of the study was to test the hypotheses that analysis of the anatomic zones affected by single anterior (A), posterior (P), and middle (M) cerebral artery (CA) infarcts, and by dual- and triple-vessel infarcts, will disclose (i) sites most frequently involved by each infarct type (peak sites), (ii) sites most frequently injured by multiple different infarct types (vulnerable zones), and (iii) anatomically overlapping sites in which the relative infarct frequency becomes equal for two or more different infarct types and/or in which infarct frequency shifts greatly between single and multivessel infarcts (potential border zones). Precise definitions of each vascular territory were adopted. CT and MRI studies from 20 ACA, 20 PCA, three dual ACA-PCA, and four triple ACA-PCA-MCA infarcts were mapped onto a standard template (Part I). Relative infarct frequencies in each zone were analyzed within and across infarct types to identify the centers and peripheries of each infarct type, the zones most frequently affected by multiple different infarct types, the zones where relative infarct frequency was equal for different infarcts, and the zones where infarct frequency shifted markedly from single- to multiple-vessel infarcts. Zonal frequency analysis provided quantitative data on the relative infarct frequency in each anatomic zone for each infarct type. It displayed zones of peak infarct frequency for each infarct, zones more vulnerable to diverse types of infarct, peripheral "overlap" zones of equal infarct frequency, and zones where infarct frequency shifted markedly between single- and multiple-vessel infarcts. It is concluded that the hypotheses are correct.

Purpose: To assess the anatomic features and vascular relationships of the proximal portion of the cisternal segment of the trochlear nerve., Materials and Methods: In 30 subjects (60 nerves) and in one patient with right superior oblique myokymia (SOM), the anatomy of the trochlear nerve was depicted with three-dimensional (3D) Fourier transformation constructive interference in steady state (CISS) magnetic resonance (MR) imaging, whereas the adjacent vessels were detected with 3D time-of-flight (TOF) MR imaging before and after gadopentetate dimeglumine administration. The images were evaluated with respect to the identification of the trochlear nerve, the distance between the point of exit (PE) and the midline, the visualized length, the vascular relationships, and the distance between the PE and the point of neurovascular contact., Results: 3D CISS MR imaging depicted the proximal cisternal segment of the trochlear nerve in the transverse, sagittal, and coronal planes in 57 (95%), 51 (85%), and 48 (80%) of 60 nerves, respectively. The distance from the midline to the PE was 3-9 mm, and the maximum visualized length of the trochlear nerve was 1-14 mm. An arterial-trochlear neurovascular contact was seen at the root exit zone (REZ) in eight (14%) nerves and at a mean distance of 3.4 mm distal to the PE in 29 nerves (51%). The patient with SOM had arterial-trochlear neurovascular contact at the REZ., Conclusion: Use of 3D CISS sequences and 3D TOF sequences with or without gadopentetate dimeglumine enables accurate identification of the proximal cisternal segment of the trochlear nerve and its neurovascular relationships.