Primary melanotic tumors of the nervous system: a consecutive case series.

BACKGROUND AND PURPOSE: Primary melanotic tumors of the nervous system (PMTNS) are thought to be an exceedingly rare group of tumors not captured by tumor registries. We aimed to determine relative incidence, clinical presentation, diagnostic findings, patient management, and outcome. METHODS: We retrospectively searched the database of the Section of Neuro-Oncology at the Yale Cancer Center for patients with primary or metastatic melanotic lesions of the nervous system. For patients with PMTNS, we recorded demographic data, clinical presentation, histopathological and imaging findings, therapy, and outcome. RESULTS: A total of 116 patients with melanotic lesions were identified, including four patients with PMTNS. The relative incidence of PMTNS was therefore calculated as 3.4%. Histology of PMTNS patients revealed melanocytoma in three patients and psammomatous melanotic schwannoma in one patient. Symptoms were non-specific and attributed to tumor mass effect. Magnetic resonance imaging showed hyperintensity on pre-contrast T1-weighted imaging, hypointensity on T2-weighted imaging, and homogenous contrast enhancement in all PMTNS patients. Definitive diagnosis was based on tissue analysis, with detection of melanin-containing cells on conventional histology and S100-positivity on immunohistochemistry. Molecular analysis for GNAQQ209L mutation assisted in establishing diagnosis when only small amounts of tissue were available. Aggressive surgical treatment showed favorable outcomes in all cases; radiation therapy was used for residual or relapsed disease. The median follow-up was 7.5 ± 5 years, and all patients were alive on the day of database closure. CONCLUSION: Primary melanotic tumors of the nervous system are rare nervous system tumors. Outcome appears excellent, and complete surgical resection may form the basis for favorable outcome. Radiation therapy may represent a therapeutic approach for residual or relapsed disease.

Delayed leukoencephalopathy with stroke-like presentation in chemotherapy recipients.

BACKGROUND: A transient leukoencephalopathy mimicking cerebrovascular accident has been described as a complication of chemotherapy, most commonly in recipients of intrathecal methotrexate for childhood leukaemia. Recently published neuroimaging data suggest a common pathophysiology associated with a variety of chemotherapy agents and modes of administration. METHODS: We reviewed the medical literature for single reports and case series of patients presenting with stroke-like episodes while receiving systemic or intrathecal chemotherapy. We only included studies providing detailed neuroimaging data. Patients with cerebrovascular accidents were excluded. RESULTS: We identified 27 reports of toxic leukoencephalopathy in patients treated with methotrexate (intrathecal, systemic), 5-fluorouracil and its derivative carmofur, and capecitabine. Diffusion weighted imaging (DWI) of all patients revealed well demarcated hyperintense lesions within the subcortical white matter of the cerebral hemispheres and the corpus callosum, corresponding to areas of decreased proton diffusion on apparent diffusion coefficient (ADC) maps (available in 21/27 patients). Lesions exceeded the confines of adjacent vascular territories. Complete resolution of symptoms within 1-4 days was accompanied by normalisation of ADC abnormalities. However, fluid attenuated inversion recovery (FLAIR) sequences frequently revealed persistent white matter abnormalities. CONCLUSIONS: Several pathophysiological models of delayed leukoencephalopathy after exposure to intrathecal or systemic chemotherapy have been proposed. DWI findings in this cohort are indicative of cytotoxic oedema within cerebral white matter and lend support to an at least partially reversible metabolic derangement as the basis for this syndrome.

Cortical dysfunction in schizophrenia during auditory word and tone working memory demonstrated by functional magnetic resonance imaging.

BACKGROUND: Verbal learning and memory deficits are among the most severe cognitive deficits observed in schizophrenia. We have demonstrated that such deficits do not extend to working memory for tones in a substantial number of patients even when verbal working memory is impaired. In this study we used functional magnetic resonance imaging to study the neural basis of this dissociation of auditory verbal and nonverbal working memory in individuals with schizophrenia. METHODS: While undergoing functional magnetic resonance imaging, 12 schizophrenic patients and 12 matched control subjects performed auditory Word Serial Position Task and Tone Serial Position Task. RESULTS: Both tasks produced activation in frontal cortex and temporal and parietal lobes of the cerebrum in both groups. While robust activation was observed in the left inferior frontal gyrus (areas 6, 44, and 45) in the control group during the Word Serial Position Task, activation in the patient group was much reduced in these areas and failed to show the same task-specific activation as in controls. Reduced activation in patients was not confined to the inferior frontal gyrus, but also extended to a medial area during the Tone Serial Position Task and to premotor and anterior temporal lobe areas during both tasks. CONCLUSIONS: These findings support the hypothesis that abnormalities in cortical hemodynamic response in the inferior frontal gyrus underlie the verbal working memory deficit in schizophrenia. The relationship of verbal working memory deficits to other cognitive functions suggests that abnormal functioning in the speech-related areas may reflect a critical substrate of a broad range of cognitive dysfunctions associated with schizophrenia.

Abnormal ventral temporal cortical activity during face discrimination among individuals with autism and Asperger syndrome.

BACKGROUND: Recognition of individual faces is an integral part of both interpersonal interactions and successful functioning within a social group. Therefore, it is of considerable interest that individuals with autism and related conditions have selective deficits in face recognition (sparing nonface object recognition). METHOD: We used functional magnetic resonance imaging (fMRI) to study face and subordinate-level object perception in 14 high-functioning individuals with autism or Asperger syndrome (the autism group), in comparison with 2 groups of matched normal controls (normal control group ] [NC1] and normal control group 2 [NC2]) (n = 14 for each). Regions of interest (ROIs) were defined in NC1 and then applied in comparisons between NC2 and the autism group. Regions of interest were also defined in NC2 and then applied to comparisons between NC1 and the autism group as a replication study. RESULTS: In the first set of comparisons, we found significant task x group interactions for the size of activation in the right fusiform gyrus (FG) and right inferior temporal gyri (ITG). Post hoc analyses showed that during face (but not object) discrimination, the autism group had significantly greater activation than controls in the right ITG and less activation of the right FG. The replication study showed again that the autism group used the ITG significantly more for processing faces than the control groups, but for these analyses, the effect was now on the left side. Greater ITG activation was the pattern found in both control groups during object processing. CONCLUSIONS: Individuals with autism spectrum disorders demonstrate a pattern of brain activity during face discrimination that is consistent with feature-based strategies that are more typical of nonface object perception.

Preliminary evidence of improved verbal working memory performance and normalization of task-related frontal lobe activation in schizophrenia following cognitive exercises.

OBJECTIVE: The authors' goal was to evaluate the possibility of treating brain and behavioral aspects of verbal memory dysfunction in patients with schizophrenia through cognitive exercises. METHOD: Eight patients did daily verbal memory exercises that became progressively more difficult over a 10-week training period. Memory performance and regional brain activations during a verbal memory task were assessed before and after these exercises. RESULTS: Verbal but not nonverbal memory performance improved after training; three patients made substantial gains, and five showed little change. Performance gains were correlated with increases in task-related activation of the left inferior frontal cortex. One patient given 5 extra weeks of training 6 weeks after the initial training period showed maintenance of initial performance gains 6 weeks after training, further improvement after the second period of training, and normalization of task-related activation of the left inferior frontal cortex. CONCLUSIONS: Verbal memory deficits can be ameliorated by memory exercises in some patients with schizophrenia. Performance improvements are associated with increased task-related activation of the same brain region that is activated during verbal memory tasks in healthy individuals.

Functional magnetic resonance imaging of cocaine craving.

OBJECTIVE: Identification of brain activity associated with craving is important for understanding the neurobiology of addiction. METHOD: Brain activity was measured in cocaine addicts and healthy subjects by functional magnetic resonance imaging (fMRI) while the subjects watched videotapes designed to elicit happy feelings, sad feelings, or the desire to use cocaine. The subjects indicated the onset of drug craving or emotional response, allowing comparison of groups before and after such feelings. RESULTS: Robust activation of the anterior cingulate was evident in patients watching cocaine-cue tapes but not in patients watching happy or sad tapes or in healthy subjects under any condition. Anterior cingulate activation preceded the reported onset of craving and was evident in patients who did not report craving. In contrast, patients showed less activation than healthy subjects during the cocaine-cue tapes in areas of the frontal lobes. After the reported onset of craving, cocaine-dependent subjects showed greater activity than healthy subjects in regions that are more active in healthy subjects when they watch sad tapes than when they watch happy tapes, suggesting a physiologic link between cocaine-cue responses and normal dysphoric states. Dynamic aspects of regional brain activations, but not the location of activations, were abnormal in cocaine-dependent subjects watching sad tapes, suggesting more general affective dysregulation. Patients showed low activation of sensory areas during initial viewing of all videotapes, suggesting generalized alteration in neuroresponsiveness. CONCLUSIONS: Cocaine cues lead to abnormally high cingulate and low frontal lobe activation in cocaine addicts. Addicts also show more general abnormalities in affect-related brain activation.

51Cr-release assay adapted to a 96-well format sample reading.

The application of the Matrix 96, a direct beta counter, to monitor cell-mediated lympholysis assays (CML) was investigated. Until now, the gamma rays emitted from 51Cr, released in the supernatant of the sample following lysis of targets by effector cells, were read in gamma counters using individual tubes for each sample. The Matrix 96 has been designed to count 96 wells simultaneously for assays performed in 96-well microplates. Aliquots of supernatants were spotted on a 96-well disposable metal spotting plate and dried prior to reading in the Matrix 96. A tight correlation was observed between the counts obtained in the Matrix 96 and a gamma counter, which indicates that the detection of electron capture beta particles emitted from 51Cr was as accurate as reading gamma rays from the same isotope. Both methods confirmed the minimal level of cytotoxicity mediated by unstimulated lymphocytes and the high levels of nonspecific cytolytic activity mediated by lymphokine-activated killer (LAK) cells against several tumor cell lines.

Network analysis of brain activations in working memory: behavior and age relationships.

Forty-six middle-aged female subjects were scanned using functional Magnetic Resonance Imaging (fMRI) during performance of three distinct stages of a working memory task-encoding, rehearsal, and recognition-for both printed pseudowords and visual forms. An expanse of areas, involving the inferior frontal, parietal, and extrastriate cortex, was active in response to stimuli during both the encoding and recognition periods. Additional increases during memory recognition were seen in right prefrontal regions, replicating a now-common finding [for reviews, see Fletcher et al. (1997) Trends Neurosci 20:213-218; MacLeod et al. (1998) NeuroImage 7:41-48], and broadly supporting the Hemispheric Encoding/Retrieval Asymmetry hypothesis [Tulving et al. (1994) Proc Natl Acad Sci USA 91:2016-2020]. Notably, this asymmetry was not qualified by the type of material being processed. A few sites demonstrated higher activity levels during the rehearsal period, in the absence of any new stimuli, including the medial extrastriate, precuneus, and the medial temporal lobe. Further analyses examined relationships among subjects' brain activations, age, and behavioral scores on working memory tests, acquired outside the scanner. Correlations between brain scores and behavior scores indicated that activations in a number of areas, mainly frontal, were associated with performance. A multivariate analysis, Partial Least Squares [McIntosh et al. (1996) NeuroImage 3:143-157, (1997) Hum Brain Map 5:323-327], was then used to extract component effects from this large set of univariate correlations. Results indicated that better memory performance outside the scanner was associated with higher activity at specific sites within the frontal and, additionally, the medial temporal lobes. Analysis of age effects revealed that younger subjects tended to activate more than older subjects in areas of extrastriate cortex, medial frontal cortex, and the right medial temporal lobe; older subjects tended to activate more than younger subjects in the insular cortex, right inferior temporal lobe, and right inferior frontal gyrus. These results extend recent reports indicating that these regions are specifically involved in the memory impairments seen with aging.

Contribution of the anteromedial temporal lobes to the evaluation of facial emotion.

Amygdala damage can result in impairments in evaluating facial expressions largely specific to fear. In contrast, right-hemisphere cortical lesions result in a more global deficit in facial emotion evaluation. This study addressed these 2 contrasting findings by investigating amygdala and adjacent cortical contributions to the evaluation of facial emotion in 12 patients with right and 11 patients with left unilateral anteromedial temporal lobectomy (RTL and LTL, respectively) and 23 normal controls. RTL but not LTL patients revealed impaired intensity ratings that included but were not exclusive to fear, with the most severe deficits confined to expressions related to affective states of withdrawal-avoidance. This suggests that affective hemispheric specializations in cortical function may extend to subcortical limbic regions. In addition, the right amygdala and adjacent cortex may be part of a neural circuit representing facial expressions of withdrawal.

Cranial nerve enhancement in the Guillain-Barré syndrome.

We report a case of Guillain-Barré syndrome with enhancement of multiple cranial nerves seen with postcontrast MR imaging. Clinical symptoms and electrodiagnostic studies reflected abnormalities of some but not all of the enhancing cranial nerves.

Limbic lobe embryology and anatomy: dissection and MR of the medial surface of the fetal cerebral hemisphere.

PURPOSE: To facilitate understanding of limbic lobe anatomy by showing embryologic transformations of the medial surface of the cerebral hemisphere. METHODS: Brains from fetal specimens ranging from 13 to 24 weeks of gestational age were dissected. Photographs were made of the medial surface of the cerebral hemisphere. MR images of different fetal specimens of similar age were made for comparison of MR anatomy with dissected material. RESULTS: At 13 weeks, the entire inner limbic arch of the hippocampal formation is visible on the medial surface of the cerebral hemisphere. The hippocampal sulcus extends from frontal lobe to temporal lobe. At 16 weeks, the outer neocortical limbic arch of the subcallosal area, cingulate gyrus, and parahippocampus gyrus is present. Growth of the corpus callosum is associated with reduction in size of the hippocampal formation in the frontal lobe. The sulcus of the corpus callosum is the remnant of the anterior part of the hippocampal sulcus. At 18 weeks, growth of the parahippocampal gyrus begins to conceal the hippocampal formation. The supracallosal gyrus (indusium griseum), hidden from view by the corpus callosum, and the paraterminal gyrus are remnants of the previously larger hippocampal formation. CONCLUSIONS: Analysis of fetal specimens in different developmental stages with dissection and MR provides insight into embryologic transformations responsible for the complex anatomy of the limbic lobe.

Embryology of the human fetal hippocampus: MR imaging, anatomy, and histology.

PURPOSE: To identify changes in the embryology of the hippocampus responsible for its adult anatomy. METHODS: Ten human fetal specimens ranging from 13 to 24 weeks' gestational age were examined with MR imaging. Dissections and histologic sections of 10 different specimens of similar ages were compared with MR imaging findings. RESULTS: At 13 to 14 weeks' gestation, the unfolded hippocampus, on the medial surface of the temporal lobe, surrounds a widely open hippocampal sulcus (hippocampal fissure). At 15 to 16 weeks, the dentate gyrus and cornu ammonis have started to infold. The hippocampal sulcus remains open. The parahippocampal gyrus is larger and more medially positioned. The CA1, CA2, and CA3 fields of the cornu ammonis are arranged linearly. The dentate gyrus has a narrow U shape. By 18 to 20 weeks, the hippocampus begins to resemble the adult hippocampus. The dentate gyrus and cornu ammonis have folded into the temporal lobe. The hippocampus and subiculum approximate each other across a narrow hippocampal sulcus. The CA1-3 fields form an arc and the CA4 field has increased in size within the widened arch of the dentate gyrus. CONCLUSION: MR imaging of fetuses provides a developmental basis for understanding hippocampal anatomy.

MR of the head and neck: comparison of fast spin-echo and conventional spin-echo sequences.

PURPOSE: To compare conspicuousness of head and neck lesions on fast spin-echo sequences and conventional spin-echo sequences. METHODS: Forty consecutive patients with 61 head and neck lesions were evaluated. Lesion conspicuousness was qualitatively compared on conventional spin-echo and fast spin-echo sequences, using both spin-density and T2-weighted images. Thirty-six lesions had surgical or pathologic confirmation, and 25 were assigned a presumptive diagnosis based on clinical evaluation and imaging findings seen on conventional spin-echo T1- and T2-weighted sequences. Forty lesions were related to neoplasms; 21 lesions consisted of infectious, vascular, or inflammatory abnormalities. RESULTS: Fast spin-echo sequences provided improved lesion conspicuousness in 91% of spin-density images, in 77% of T2-weighted images, and in 84% of the combined spin-density and T2-weighted images. CONCLUSION: By providing shorter imaging times and equal or superior lesion conspicuousness, long-repetition-time fast spin-echo sequences can replace long-repetition-time conventional spin-echo sequences in evaluation of the head and neck.

Cerebral activation during multiplication: a functional MR imaging study of number processing.

BACKGROUND AND PURPOSE: Current models of brain function propose that number processing involves the interaction of different neuronal networks. Our purpose was to use functional MR (fMR) imaging to elucidate the brain regions engaged by multiplication. METHODS: Eighteen adults underwent fMR imaging while performing matching, multiplication, and control tasks. For each task, three or four single-digit or low-value double-digit numbers were presented serially followed by a 12-second delay. A target stimulus then appeared and subjects made a judgement by pressing a button box that recorded responses. During the matching task, subjects judged whether the target stimulus matched one of the previous numbers. During the multiplication task, subjects judged whether the target stimulus was the product of the previous numbers. For the control task, the numbers were always zeros, and the subjects responded to a target stimulus that was always four zeros. Composite statistical parametric maps of the time course of activation comparing the control task with the matching and multiplication tasks, respectively, were generated and the significance of signal changes was estimated by randomization of statistical parametric maps. RESULTS: The matching and multiplication tasks resulted in activation (P < .005) in the medial superior frontal gyrus; the anterior cingulate gyrus; the intraparietal sulci, bilaterally; the right superior frontal sulcus bilaterally; the middle, inferior and precentral frontal gyri (left greater than right); the left basal ganglia; and the right lateral and inferior occipital gyri. There was a larger area of early activation in the right middle frontal gyrus during the matching task compared with the multiplication task, and there was a longer interval of activation in the left middle frontal gyrus during the multiplication task (10 seconds) than in the matching task (6 seconds). CONCLUSION: Multiplication and memory of numbers share an integrated network of brain regions. The left frontal lobe, an area also involved in memory and language processes, appears to play an important role in multiplication.

Exceptional multiplicity of cerebral arteriovenous malformations associated with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome).

PURPOSE: To describe the clinical and imaging features of seven patients with hereditary hemorrhagic telangiectasia and an exceptional number of cerebral arteriovenous malformations (AVMs). METHODS: One hundred thirty-six patients from a dedicated hereditary hemorrhagic telangiectasia clinic were screened systematically for cerebral AVMs by means of MR imaging. Thirty-one were found to have abnormalities suggestive of a vascular malformation. Eighteen of these 31 patients subsequently underwent diagnostic cerebral angiography. RESULTS: Of the 18 patients who had cerebral angiography, all were found to have at least one AVM and seven were found to have three or more AVMs. The number of cerebral AVMs detected ranged from three to nine. At angiography, the AVMs varied in size from 3 to 25 mm in maximal dimension and consisted of a poorly defined plexiform nidus that typically had a single arterial feeding pedicle and a single draining vein. The two largest AVMs (20- and 25-mm nidus, respectively) contained intranidal aneurysms. Treatment included embolization, surgical excision, or follow-up management. CONCLUSIONS: Multiple cerebral AVMs are associated with hereditary hemorrhagic telangiectasia and further highlight the uniqueness of central nervous system involvement by this systemic angiodysplasia. MR imaging can underestimate the number and size of cerebral AVMs; therefore, catheter angiography is necessary to establish the extent of central nervous system involvement in this disorder.

Focal cortical dysplasia of Taylor, balloon cell subtype: MR differentiation from low-grade tumors.

PURPOSE: To test the hypothesis that focal cortical dysplasia of Taylor (FCDT) can be distinguished from low-grade tumors by means of clinical and MR findings. METHODS: We examined 10 clinical and 19 MR imaging variables in patients who underwent surgery for intractable epilepsy over an 8-year period. The 54 patients with low-grade glial neoplasms were compared with the eight patients who had balloon cell FCDT. RESULTS: Statistically significant differences were seen with respect to eight of the MR variables and none of the clinical variables. MR findings suggesting dysplasia rather than tumor included the presence of gray matter thickening associated with a homogeneous hyperintense signal in the subcortical white matter that tapers as it extends to the lateral ventricle. A frontal lobe location favors dysplasia, while a temporal lobe (especially medial temporal lobe) location is more suggestive of a neoplasm. CONCLUSION: Several MR features help distinguish balloon cell FCDT from neoplasms, especially cortical thickening and a tapered signal to the ventricle. This distinction is important for surgical planning, as the decision to operate and the extent of surgical resection often depend on the presence or absence of neoplastic tissue.

Functional MR imaging of regional brain responses to pleasant and unpleasant odors.

BACKGROUND AND PURPOSE: Odors can elicit a range of behaviors and emotions. Our purpose was to identify regional activation of the human cerebral cortex in response to pleasant (positive hedonic value) and unpleasant (negative hedonic value) odors. METHODS: Thirteen neurologically normal adults underwent functional MR imaging of frontal and anterior temporal brain regions with a gradient-echo echo-planar technique. Eleven candidate regions of interest (ROIs) were identified on the first half of the data set based on t-map comparisons of signal intensities during administration of clementine (pleasant odor), isovaleric acid (unpleasant odor), and clear air (control odor). These ROIs were applied to the second half of the data set, and the number of voxels activated with the odorants was compared with the number of voxels activated during clear air trials, using independent t-tests. RESULTS: Clementine activated five cortical areas: Brodmann's area (BA) 8, BA 32 (lateralized to left), BA 46/9, BA 6 (lateralized to right), and the insula. Isovaleric acid activated four of the five regions without lateralization; no BA 8 activity was seen. Clementine produced more activity than isovaleric acid in the left insula, and isovaleric acid produced more activity than clementine in the left BA 6. No activation was detected in the orbitofrontal cortex or in the medial temporal lobes. Subjects rated clementine, isovaleric acid, and clear air as being pleasant, unpleasant, and neutral, respectively. CONCLUSION: Activation in frontal regions may represent brain processes linked to olfactory networks. There may be regional specialization based on odorant hedonic values.

Regional distribution of MR findings in hippocampal sclerosis.

PURPOSE: To investigate the distribution of MR findings in the hippocampus and amygdala in patients with hippocampal sclerosis. METHODS: We blindly evaluated MR scans for atrophy and signal changes occurring in the amygdala, hippocampal head, hippocampal body, and hippocampal tail in 57 consecutive patients with hippocampal sclerosis proved by pathologic analysis. RESULTS: Regional atrophy or signal change was present in limbic structures. Atrophy was detected in 52 patients, occurring in the amygdala in 7 (12%), hippocampal head in 29 (51%), hippocampal body in 50 (88%), and hippocampal tail in 35 (61%). Hyperintense signal on long-repetition-time images was observed in 49 patients and involved the amygdala in 2 (4%), hippocampal head in 22 (39%), hippocampal body in 46 (81%), and hippocampal tail in 28 (49%). Thirty patients (53%) had abnormal MR findings distributed through the entire ipsilateral hippocampus, 25 (44%) had regional rather than widespread involvement of limbic structures, and 2 (3%) had no MR abnormalities. CONCLUSION: Signal and volume changes associated with hippocampal sclerosis affect the entire hippocampus in most patients. However, a substantial number of patients have MR abnormalities that are regional, involving only portions of the hippocampus and amygdala. The most frequently affected region was the hippocampal body. These findings can have important implications for surgery and quantitative image analysis, if the seizure generator is related to MR changes.

MR of hereditary hemorrhagic telangiectasia: prevalence and spectrum of cerebrovascular malformations.

PURPOSE: Our goal was to describe the prevalence and types of cerebral vascular malformations (CVMs) seen with MR imaging in patients with hereditary hemorrhagic telangiectasia (HHT). METHODS: We reviewed retrospectively the brain MR images of 184 consecutive patients with HHT. Catheter angiography was performed in 17 patients with CVMs detected on MR images. RESULTS: MR imaging revealed 63 CVMs in 42 patients. Classic arteriovenous malformations (n = 10) had a conspicuous network of vessels with flow voids and enlarged adjacent pial vessels. Apparent venous malformations (n = 5) were best seen after administration of contrast material as a prominent vessel coursing through normal brain parenchyma. Indeterminate vascular malformations (n = 48) had a spectrum of appearances characterized by variable combinations of heterogeneous signal intensity, enhancement, or hemosiderin. Angiography in 17 patients revealed 47 CVMs. Forty-six were arteriovenous malformations (AVMs), including 25 CVMs not seen with MR imaging and 21 CVMs that by MR criteria included 8 AVMs and 13 indeterminate vascular malformations. Angiography confirmed 1 venous malformation seen with MR imaging but failed to detect 3 indeterminate lesions revealed by MR imaging. CONCLUSION: MR imaging of a large cohort of consecutive patients with HHT revealed a CVM prevalence of 23% (42/184). Most CVMs (48/63) have an atypical appearance for vascular malformations on MR images. Angiographic correlation suggests that MR imaging underestimates the prevalence of CVMs and that the majority of indeterminate CVMs, despite their variable MR appearance, are AVMs.

The cerebellum's role in reading: a functional MR imaging study.

BACKGROUND AND PURPOSE: Long considered to have a role limited largely to motor-related functions, the cerebellum has recently been implicated as being involved in both perceptual and cognitive processes. Our purpose was to determine whether cerebellar activation occurs during cognitive tasks that differentially engage the component processes of word identification in reading. METHODS: Forty-two neurologically normal adults underwent functional MR imaging of the cerebellum with a gradient-echo echo-planar technique while performing tasks designed to study the cognitive processing used in reading. A standard levels-of-processing paradigm was used. Participants were asked to determine whether pairs of words were written in the same case (orthographic processing), whether pairs of words and non-words rhymed with each other, respectively (phonologic assembly), and whether pairs of words belonged to the same category (semantic processing). Composite maps were generated from a general linear model based on a randomization of statistical parametric maps. RESULTS: During phonologic assembly, cerebellar activation was observed in the middle and posterior aspects of the posterior superior fissure and adjacent simple lobule and semilunar lobule bilaterally and in posterior aspects of the simple lobule, superior semilunar lobule, and inferior semilunar lobule bilaterally. Semantic processing, however, resulted in activation in the deep nuclear region on the right and in the inferior vermis, in addition to posterior areas active in phonologic assembly, including the simple, superior semilunar, and inferior semilunar lobules. CONCLUSION: The cerebellum is engaged during reading and differentially activates in response to phonologic and semantic tasks. These results indicate that the cerebellum contributes to the cognitive processes integral to reading.