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Diffusion tensor imaging (DTI) of the cerebral white matter has proven to be a promising tool with regard to demonstration of developmental changes during childhood and older age and of pathologic alterations by calculation of parameter maps of mean diffusivity (MD) and fractional anisotropy (FA) as well as to image the positions of the major fiber tracts by tractography. After a methodological introduction, the experiences with DTI collected in Mainz, Germany, during the last 5 years are presented. Special emphasis is on preoperative imaging of the corticospinal tract and optic radiation and the computation of FA-based templates (atlases) which can be used for observer-independent evaluation of FA and MD group differences and supra- and infratentorial probabilistic tractography.

Purpose To look for the presence and age-dependence of late structural alterations of otherwise normal-appearing cerebral gray and white matter after radiation and chemotherapy in adult survivors of acute lymphoblastic leukemia (ALL) during childhood. Materials and Methods In a group of 13 adult survivors 17–37 years old, who had been treated by total brain radiation (18–24 Gy) and chemotherapy 16–28 years ago, prospective MR examinations including diffusion tensor imaging (DTI) were performed. Evaluation included volumetry, calculation of mean diffusivity (MD) and fractional anisotropy (FA), and comparison of results to an age-matched control group. Results DTI showed significantly reduced FA values in the temporal lobes (difference of 0.069 units, P < 0.001), hippocampi (difference of 0.033 units, P < 0.001), and thalami (difference of 0.046 units, P = 0.001), which were accompanied by significant white matter volume loss (difference of 92 cm3, P < 0.001). Significant elevations of MD were limited to the temporal white matter (difference of 42 × 10−6 mm2/s, P = 0.005). Global and frontal white matter MD correlated negatively to increasing age of the survivors (P < 0.01). Conclusion With regard to structural white matter alterations, adult long-term survivors of childhood ALL, who had received total brain radiation and chemotherapy, apparently show the same overall age dependence as controls. Follow-up studies are needed for confirmation. J. Magn. Reson. Imaging 2008;27:1250–1255. © 2008 Wiley-Liss, Inc.

Animal data suggest that the gene neuregulin-1 (NRG1) is involved in neuronal myelination. A haplotype (deCODE) in the 5' end region of the gene was described to double the risk for schizophrenia in an Icelandic population (Stefansson, H., Sigurdsson, E., Steinthorsdottir, V., Bjornsdottir, S., Sigmundsson, T., Ghosh, S., Brynjolfsson, J., Gunnarsdottir, S., Ivarsson, O., Chou, T.T., Hjaltason, O., Birgisdottir, B., Jonsson, H., Gudnadottir, V.G., Gudmundsdottir, E., Bjornsson, A., Ingvarsson, B., Ingason, A., Sigfusson, S., Hardardottir, H., Harvey, R.P., Lai, D., Zhou, M., Brunner, D., Mutel, V., Gonzalo, A., Lemke, G., Sainz, J., Johannesson, G., Andresson, T., Gudbjartsson, D., Manolescu, A., Frigge, M.L., Gurney, M.E., Kong, A., Gulcher, J.R., Petursson, H., Stefansson, K. 2002. Neuregulin-1 and susceptibility to schizophrenia. Am. J. Hum. Genet. 71, 877-892). Of note, there is now increasing evidence of disturbed myelination in this illness--particularly in subcortical frontal lobe white matter (Konrad, A., Winterer, G. 2008. Disturbed structural connectivity in schizophrenia--primary factor in pathology or epiphenomenon? Schiz. Bull. [Electronic publication ahead of print]). Therefore, we investigated with diffusion tensor imaging (DTI) the impact of a tagging single nucleotide polymorphism (SNP) from the deCODE haplotype, i.e., SNP8NRG221533, on fractional anisotropy (FA), which reflects structural integrity of white matter. SNP8NRG221533 was selected because it gave the single best uncorrected association with schizophrenia in the original report by Stefansson et al. (Stefansson, H., Sigurdsson, E., Steinthorsdottir, V., Bjornsdottir, S., Sigmundsson, T., Ghosh, S., Brynjolfsson, J., Gunnarsdottir, S., Ivarsson, O., Chou, T.T., Hjaltason, O., Birgisdottir, B., Jonsson, H., Gudnadottir, V.G., Gudmundsdottir, E., Bjornsson, A., Ingvarsson, B., Ingason, A., Sigfusson, S., Hardardottir, H., Harvey, R.P., Lai, D., Zhou, M., Brunner, D., Mutel, V., Gonzalo, A., Lemke, G., Sainz, J., Johannesson, G., Andresson, T., Gudbjartsson, D., Manolescu, A., Frigge, M.L., Gurney, M.E., Kong, A., Gulcher, J.R., Petursson, H., Stefansson, K. 2002. Neuregulin-1 and susceptibility to schizophrenia. Am. J. Hum. Genet. 71, 877-892). As predicted, we found medial frontal FA to be significantly associated with this NRG1 gene variation. Using voxel-based morphometry (VBM), we could largely exclude the possibility that this genotype effect is indirectly caused by genotype-dependent effects on brain volume. This is the first demonstration that SNP8NRG221533 of the NRG1 gene affects medial frontal white matter microstructure in humans. As the degree of neuronal myelination contributes to structural integrity, our finding further supports a potential role of NRG1 in neuronal myelination in the human brain. By extension, our findings suggest that SNP8NRG221533 may contribute to the risk for the complex polygenic illness schizophrenia via its impact on myelination in frontal lobe white matter.

Object The authors report on their experience with a 3D virtual reality system for planning minimally invasive neurosurgical procedures. Methods Between October 2002 and April 2006, the authors used the Dextroscope (Volume Interactions, Ltd.) to plan neurosurgical procedures in 106 patients, including 100 with intracranial and 6 with spinal lesions. The planning was performed 1 to 3 days preoperatively, and in 12 cases, 3D prints of the planning procedure were taken into the operating room. A questionnaire was completed by the neurosurgeon after the planning procedure. Results After a short period of acclimatization, the system proved easy to operate and is currently used routinely for preoperative planning of difficult cases at the authors' institution. It was felt that working with a virtual reality multimodal model of the patient significantly improved surgical planning. The pathoanatomy in individual patients could easily be understood in great detail, enabling the authors to determine the surgical trajectory precisely and in the most minimally invasive way. Conclusions The authors found the preoperative 3D model to be in high concordance with intraoperative conditions; the resulting intraoperative “déjà-vu” feeling enhanced surgical confidence. In all procedures planned with the Dextroscope, the chosen surgical strategy proved to be the correct choice. Three-dimensional virtual reality models of a patient allow quick and easy understanding of complex intracranial lesions.

Background: Fabry disease (FD) is a lysosomal storage disorder that is associated with marked cerebrovascular disease. Conventional MRI shows an extensive load of white matter lesions (WMLs) already at an early stage in FD. Objective: Investigator independent and sensitive quantification of brain structural changes in clinically affected men and women with FD. Methods: The authors performed a voxel-based analysis of diffusion tensor images (DTI) in 25 FD patients and 20 age-matched normal controls. Results: DTI revealed significant increases of cerebral white matter diffusivity (MD) in FD patients that were pronounced in the periventricular white matter. Even the subgroup of patients without significant WMLs-load (n = 18) showed increased diffusivity in the cerebral white matter. In gray matter areas MD elevation could only be detected in the posterior part of the thalamus, independently from visible pulvinar alterations on the T1-weighted images. Voxel-based anisotropy (FA) measurements did not differ significantly between patients and controls. Conclusions: The present study demonstrates the clinical feasibility of voxel-based analysis of DTI as a sensitive tool to quantify brain tissue alterations in FD. The pattern of increased brain tissue diffusivity is probably due to microangiopathic alterations mainly affecting the long perforating arteries.

Background Damage of the internal carotid artery (ICA) is very rare but can be a dramatic complication of endonasal sinus surgery. In the literature only a few cases are reported, some of them with fatal results. Methods We present two cases with massive bleeding of the ICA caused by endonasal sinus surgery. Results In both cases bleeding could be stopped sufficiently by implanting a stentgraft. Conclusion The lumen of the artery could be preserved and no neurological deficits were observed.

Mutational analyses in xenopus oocyte and mice models indicate that the positive effect of nicotine on attention may be modulated by genetic variations within exon 5 of the alpha4 subunit of the nicotinergic acetylcholine receptor gene CHRNA4. The potential relevance of exon 5 is further emphasized by two recent family-based association studies of nicotine dependence because subgroups of nicotine-dependent subjects are thought to 'self-medicate' attentional deficits with nicotine. We investigated a synonymous single nucleotide polymorphisms (SNP): rs1044396, which has recently been associated with nicotine-dependence, plus two adjacent synonymous SNPs rs1044394 and rs1044393 in exon 5 of n = 47 unrelated healthy Caucasian subjects (age: 22.7 +/- 1.7 years; sex: n = 23 males; regular smokers: n = 19). Attentional network function was assessed in supplementary motor area/anterior cingulate (SMA/ACC) and parietal cortex with functional magnetic resonance imaging during an attention-requiring visual oddball task. SNP rs1044396 showed genotype effects on attentional network function both in the SMA/ACC and parietal cortex in the absence of overt behavioral effects. In the parietal cortex, a gene-dosage effect was seen. Comparable genotype effects were also found for the other two SNPs. This investigation provides first evidence that attentional network function may be modulated by genetic variations within CHRNA4 exon 5. If confirmed, future studies need to address what 'functional' polymorphisms are causative for the observed effects.

UNLABELLED Central nervous system involvement is a major burden in Fabry disease. Conventional cranial magnetic resonance imaging (MRI) shows micro- and macroangiopathic changes such as severe and progressive white matter lesions (WMLs) at an early age on T2- and fluid-attenuated inversion recovery-weighted images, increased signal intensity in the pulvinar on T1-weighted MRI, as well as tortuosity and dilatation of the larger vessels (dolicho-ectasia). Using diffusion tensor imaging (DTI), a new structural MRI-technique that measures water diffusion characteristics, we showed marked brain tissue alterations in Fabry disease predominantly in the periventricular white matter. Even patients with few WMLs had significantly elevated brain tissue diffusivity. CONCLUSION DTI is more sensitive in detecting brain tissue changes in Fabry disease than conventional MRI. DTI measurements could provide appropriate surrogate parameters with which to monitor the natural history of structural brain involvement and potential effects of therapy (such as enzyme replacement) in Fabry disease.

Hippocampus atrophy is a frequent finding in mild cognitive impairment (MCI), whereas diffusion-tensor-imaging (DTI) has demonstrated its value to detect subtle brain tissue changes in several neuropsychiatric diseases including MCI. To compare the diagnostic accuracy of both methods, high resolution MRI scans for hippocampus volumetry, and co-registered DTI-scans for ROI-based mean diffusivity (MD) and fractional anisotropy (FA) were carried out in 18 patients with amnestic MCI (7 females, age 67.3+/-8.7 years, MMSE 25.2+/-2.2) and 18 controls (age 66.9+/-9.0 years, MMSE 28.7+/-1.0). Diagnostic properties of normalized hippocampus volume (HV) and DTI measures with regard to MCI status were estimated by receiver operating characteristics (ROC) analyses and logistic regression. Parameters of the left hippocampus showed superior predictive power when compared to the right. At a specificity set to 80%, left HV had low sensitivity (50%); left hippocampal MD values revealed superior sensitivity (89%), similar to left hippocampal FA (78%). The results demonstrate higher sensitivity of DTI-derived left hippocampal parameters than volume measures in detecting subtle hippocampal abnormalities related to MCI.

Molecular and functional imaging techniques reveal evidence for lateralization of human cerebral function. Based on animal data, we hypothesized that asymmetry in dopamine neurotransmission declines during normal aging. In order to test this hypothesis, we measured dopamine D2/3 receptor availability with [18F]desmethoxyfallypride-PET (DMFP) in putamen and caudate nucleus (NC) of 21 healthy, right-handed males (24-60 years; 35+/-10). For volumetric analysis, high-resolution T1-weighted MR-images were obtained in 18 of the PET-subjects in order to assess possible age-related decreases in NC and putamen volume. The calculated DMFP binding potentials (BP) showed a right-ward asymmetry in NC of young subjects that decreased with age (r = 0.577, p = 0.006; Pearson correlation; two-tailed). An age-independent analysis showed a right-ward asymmetry in NC of the whole subject group (left: 1.49+/-0.35; right: 1.65+/-0.43 [mean+/-S.D.]; p = 0.020). No such side lateralization or age-effects could be found in the putamen. Volumes tended to be asymmetric in the putamen (right: 4.85+/-0.56 cm3; left: 4.64+/-0.86 cm3 [mean+/-S.D.]; p = 0.063), but not in NC. The decline of putamen volume during aging was significant in the right putamen (r = -0.613; p = 0.007; Pearson correlation; two-tailed). There were no other significant correlations between striatal volumes and age or BP. Because ventral striatal dopamine neurotransmission is involved in cognitive processes, this loss of physiological asymmetry in NC dopamine transmission during aging might be involved in age-related declines of cognitive performance.

Data analysis was performed in a multicenter study to evaluate magnetic resonance (MR) imaging for classification of brain tumors, prognosis, and prediction of tumor histological diagnosis.The clinical, MR imaging, and histological findings obtained in 142 pediatric cases of brainstem disease were assessed in a multicenter study performed as a blinded review. Clinical data were available in 142 cases, histopathological findings in 126, and MR images in 131. The subgroup of cases involving brainstem gliomas (78 cases) was analyzed separately. Images that met criteria for evaluation were reviewed in random order by three experienced observers who were initially blinded to clinical data as well as histopathological diagnoses. Subsequently, the images were randomized again and provided to the observers for review together with the clinical symptoms of the specific patients. The three observers were able to correctly identify lesions as tumors or nontumorous disease on MR images in 99, 96, and 95% of cases, resulting in an overall sensitivity of 0.94, a specificity of 0.43, a positive predictive value of 0.96, and a negative predictive value of 0.45. Awareness of clinical symptoms did not change the results.Based on 14 imaging criteria together with the patient's clinical history and symptoms, laboratory data (results of cerebrospinal fluid analysis as well as infectious and immunological parameters), and imaging follow up, a diagnosis of brainstem tumor, as opposed to demyelination, encephalitis, or granuloma, could generally be made. Given these findings, there is only rarely a need for biopsy, and in those patients in whom it is considered, the potential costs and benefits must be carefully assessed on a case-by-case basis.

Purpose To differentiate fibroblastic meningiomas, usually considered to be of a hard consistency, from other benign subtypes using diffusion tensor imaging (DTI). Materials and Methods From DTI data sets of 30 patients with benign meningiomas, we calculated diffusion tensors and mean diffusivity (MD) and fractional anisotropy (FA) maps as well as barycentric maps representing the geometrical shape of the tensors. The findings were compared to postoperative histology. The study was approved by the local ethics committee, and informed consent was given by the patients. Results According to one-way analysis of variance (ANOVA), FA was the best parameter to differentiate between the subtypes (F = 32.2; p < 0.0001). Regarding tensor shape, endothelial meningiomas were represented by spherical tensors (80%) corresponding to isotropic diffusion, whereas the fibroblastic meningiomas showed a high percentage (43%) of nonspherical tensors, indicating planar or longitudinal diffusion. The difference was highly significant (F = 28.4; p < 0.0001) and may be due to the fascicular arrangement of long spindle-shaped tumor cells and the high content of intra- and interfascicular fibers as shown in the histology. In addition, a capsule-like rim of the in-plane diffusion surrounded most meningiomas irrespective of their histological type. Conclusion If these results correlate to the intraoperative findings of meningioma consistency, DTI-based measurement of FA and analysis of the shape of the diffusion tensor is a promising method to differentiate between fibroblastic and other subtypes of benign meningiomas in order to get information about their “hard” or “soft” consistency prior to removal. J. Magn. Reson. Imaging 2007. © 2007 Wiley-Liss, Inc.

To search for microstructural lesions of normal-appearing cerebral white matter surrounding a tumor or a vascular lesion, after single-fraction stereotactic Gamma Knife (GK) radiosurgery.In 43 patients with different brain lesions, magnetic resonance including diffusion tensor imaging (DTI) was performed before and after GK radiosurgery and change of parameters was measured in areas surrounding the lesion.Outside the lesion, there was an increase in mean diffusivity (MD) and radial diffusivity (RD) between 2.1% and 3.4% in the 15–10 Gy and in the 10–5 Gy perilesional isodose volumes, which reached statistical significance (pairedWe report some minor, but nevertheless significant changes in DTI parameters in normal-appearing perilesional brain tissue after GK radiosurgery progressing with time, which partially may be induced by the radiation itself and partially may be due to indirect effects of lesion reactions to the radiation. Follow-up studies are necessary for further characterization of these changes and assessment of their time course.

Fabry Disease (FD) is a rare X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase A (alpha-GAL) enzyme activity. Neutral glycosphingolipides (esp. Gb3) accumulate in lysosomes of several tissues, particularly in vascular endothelium and smooth muscle cells. Cerebral manifestations that might be mainly due to progressive cerebrovascular dysfunction, are one major and often life-threatening burden of the disease. We reviewed the present literature concerning brain structural alterations in FD and discuss the possibly relevant underlying pathophysiological aspects of these disturbances. Cerebrovascular events (TIA, stroke) occur in FD at a rather early age. In female FD patients who were considered to be less affected "carriers" for a long time, the prevalence of cerebrovascular events seems to be at last as high as in male patients. In structural imaging white matter lesions (WML) can be found frequently even in young FD patients. In a recent study clinically equally affected men and women with FD showed a comparable severity of WML load. Different pathophysiological aspects of cerebral angiopathy and WML development are discussed against the background of current concepts (e. g. accumulation of Gb3 in vascular endothelium with consecutive cell proliferation and luminal stenosis, acceleration of focal intravasal pressure and disturbances of vascular auto-regulation). Pathological increase of pulvinar signal in T1-weighted MRI has also been described in FD. This finding was assumed to be caused by calcification as a consequence of disturbed local circulation. To enhance our knowledge about the relevant neurobiological processes the authors propose a more sensitive and early detection of brain structural changes in FD. New brain structural MRI methods such as diffusion-tensor imaging could provide a pattern of ultrastructural changes even in young patients without visible WML. This strategy could be as well useful for quantification of possible effects of the enzyme replacement therapy on brain structural alterations in FD. Based on recent data a systematic FD-screening by measuring Gb3 in urine of young patients with cryptogenic stroke should be discussed. Basically in such cases FD should be clinically considered.

To determine how opiate receptor distribution is co-localized with the distribution of nociceptive areas in the human brain, eleven male healthy volunteers underwent one PET scan with the subtype-nonselective opioidergic radioligand [(18)F]fluoroethyl-diprenorphine under resting conditions. The binding potential (BP), a parameter for the regional cerebral opioid receptor availability, was computed using the occipital cortex as reference region. The following regions of interest (ROIs) were defined on individual MR images: thalamus, sensory motor strip (SI/MI area), frontal operculum, parietal operculum, anterior insular cortex, posterior insular cortex, anterior cingulate cortex (ACC; peri- and subgenual part of "classical ACC" only), midcingulate cortex (MCC, posterior part of "classical ACC"), putamen, caudate nucleus and the amygdala. BP for [(18)F]fluoroethyl-diprenorphine was lowest in the sensory motor strip (0.30). Highest BP was found in thalamus (1.36), basal ganglia (putamen 1.22, caudate 1.16) and amygdala (1.21). In the cingulate cortex, ACC (1.11) had higher BP than MCC (0.86). In the operculo-insular region, we found high BPs in all ROIs: anterior insula (1.16), posterior insula (1.05), frontal operculum (0.99) and parietal operculum (0.77). Factor analysis of interindividual variability of opiate receptor BP revealed four factors (95% explained variance): (1) operculo-insular areas, ACC, MCC and putamen, (2) amygdala and thalamus, (3) caudate and thalamus, (4) SI/MI and MCC. Nociceptive areas of the lateral pain system (frontoparietal operculum and insula) have opiate receptor BPs significantly higher than SI/MI, comparable to anterior and midcingulate areas of the medial pain system. These findings suggest that the cortical anti-nociceptive effects of opiates are not only mediated by ACC and MCC, but also by the operculo-insular cortex, if it can be assumed that opioid binding mediates anti-nociception in those structures.

Background To look for microstructural white matter alterations in patients with dystonia due to Pantothenate Kinase-Associated Neurodegeneration. Material and methods We examined 21 genetically confirmed patients and an age-matched group of 21 healthy controls by diffusion tensor imaging. Evaluation of data was performed by tract-based spatial statistics analysis and a voxel-wise comparison of calculated maps of fractional anisotropy. Findings were compared between groups and correlated to the dystonia score of the Burke-Fahn-Marsden Scale (p ≤ 0.05). Results Patients showed reductions of fractional anisotropy mainly in the periventricular substance surrounding the third ventricle, in the medial part of both putamina and in the frontal white matter including the anterior limbs of the internal capsules and the corpus callosum. Infratentorially, the cerebellar white matter and dorsal parts of the pons and medulla were affected. Conclusion In addition to cortical grey matter changes, we now have a second structural finding pointing to a more widespread affection of cerebral tissue in PKAN dystonia than just the lesion and iron accumulation in the globus pallidus.

Das Ziel dieser Studie war die Klarung der Frage, ob sich bei Patienten mit einer leichten kognitiven Beeintrachtigung im Sinne eines amnestischen „mild cognitive impairment“ (MCI) sog. ultrastrukturelle Veranderungen mit Hilfe der Diffusions-Tensor-Bildgebung (DTI) nachweisen lassen. MCI stellt einen haufigen Ubergang vom normalen Nachlassen der geistigen Leistungsfahigkeit im Alter zur Alzheimer-Demenz dar. Das posteriore Cingulum ist als Teil des sog. Papez-Kreises ein wichtiges neuronales Substrat der episodischen Gedachtnisleistung, die bei MCI typischerweise gestort ist. Pathologische Verminderungen des Glukosemetabolismus im Bereich des posterioren Cingulum konnten mittels Positronenemissionstomographie (FDG-PET) als Fruhindikator einer Alzheimer-Erkrankung bei Patienten mit MCI nachgewiesen werden. Bei 17 Patienten mit MCI, 25 Patienten mit klinisch manifester Alzheimer-Demenz und 21 altersentsprechenden gesunden Probanden wurden neuropsychologische Untersuchungen und Messungen der beiden mittels DTI ermittelten Parameter mittlere Diffusivitat (MD) und fraktionelle Anisotropie (FA) im posterioren Cingulum durchgefuhrt. Die Platzierung der dazu erforderlichen “regions of interest” erfolgte mit Hilfe von farbkodierten Karten der Marklagerbahnen, die aus den Tensoren berechnet wurden. Die Messung ergab bei den Patienten eine signifikante Steigerung der MD und eine Abnahme der FA im hinteren Anteil des Cingulum. Die pathologischen Veranderungen der Diffusionscharakteristika (MD und FA) korrelierten auserdem mit den kognitiven Leistungen der psychologischen Tests. Diese Befunde stellen moglicherweise einen fruhen MR-Indikator fur die Entwicklung einer Alzheimer-Demenz dar.

Our current understanding of brainstem reflex physiology comes chiefly from the classic anatomical-functional correlation studies that traced the central circuits underlying brainstem reflexes and establishing reflex abnormalities as markers for specific areas of lesion. These studies nevertheless had the disadvantage of deriving from post-mortem findings in only a few patients. We developed a voxel-based model of the human brainstem designed to import and normalize MRIs, select groups of patients with or without a given dysfunction, compare their MRIs statistically, and construct three-plane maps showing the statistical probability of lesion. Using this method, we studied 180 patients with focal brainstem infarction. All subjects underwent a dedicated MRI study of the brainstem and the whole series of brainstem tests currently used in clinical neurophysiology: early (R1) and late (R2) blink reflex, early (SP1) and late (SP2) masseter inhibitory reflex, and the jaw jerk to chin tapping. Significance levels were highest for R1, SP1 and R2 afferent abnormalities. Patients with abnormalities in all three reflexes had lesions involving the primary sensory neurons in the ventral pons, before the afferents directed to the respective reflex circuits diverge. Patients with an isolated abnormality of R1 and SP1 responses had lesions that involved the ipsilateral dorsal pons, near the fourth ventricle floor, and lay close to each other. The area with the highest probabilities of lesion for the R2-afferent abnormality was in the ipsilateral dorsal-lateral medulla at the inferior olive level. SP2 abnormalities reached a low level of significance, in the same region as R2. Only few patients had a crossed-type abnormality of SP1, SP2 or R2; that of SP1 reached significance in the median pontine tegmentum rostral to the main trigeminal nucleus. Although abnormal in 38 patients, the jaw jerk appeared to have no cluster location. Because our voxel-based model quantitatively compares lesions in patients with or without a given reflex abnormality, it minimizes the risk that the significant areas depict vascular territories rather than common spots within the territory housing the reflex circuit. By analysing statistical data for a large cohort of patients, it also identifies the most frequent lesion location for each response. The finding of multireflex abnormalities reflects damage of the primary afferent neurons; hence it provides no evidence of an intra-axial lesion. The jaw jerk, perhaps the brainstem reflex most widely used in clinical neurophysiology, had no apparent topodiagnostic value, probably because it depends strongly on peripheral variables, including dental occlusion.

Durch Integration des Fiber Tracking in die intraoperative Neuronavigation sollten die Lage der kortikospinalen Bahnen bei der Operation von Hirntumoren dargestellt und gleichzeitig die Lokalisation der Trajektorien mit einer unabhangigen Methode auf Ortsgenauigkeit uberpruft werden. Dazu wurden bei 21 Patienten MR-Untersuchungen mit Diffusion Tensor Imaging (DTI) durchgefuhrt, die Tensorenfelder berechnet und die Bahnenverlaufe mittels eines modifizierten „Line-Propagation“-Algorithmus bestimmt. Der Algorithmus kann die Richtung der Haupteigenvektoren auch uber Faserkreuzungen hinweg verfolgen. Das „Tracking“ im peritumoralen Odem ist durch die dort herabgesetzte fraktionale Anisotropie (FA) erschwert, so dass in diesen Fallen eine Modifizierung der Stopp-Kriterien des Algorithmus erforderlich war. Nach Koregistrierung der berechneten Trajektorien mit hochaufgelosten dreidimensionalen (3-D) Datensatzen und Umwandlung in das DICOM-Format wurden die Daten in ein neurochirurgisches Navigationssystem eingespeist. In allen 21 Fallen konnten die kortikospinalen Bahnen dargestellt werden. Dies wurde von den Neurochirurgen als sehr hilfreich empfunden, um eine Schadigung der Bahnen zu vermeiden. Wahrend der Tumorentfernung wurde die Position der Bahnen bei 13 Patienten mittels elektrophysiologischer kortikaler und in 10 Fallen mit subkortikaler Stimulation uberpruft und mit der Position der Trajektorien im Navigationssystem verglichen. In allen 13 Fallen war die kortikale Stimulation erfolgreich und bestatigte die Position des Gyrus praecentralis, aus dem die Trajektorien entsprangen. Eine positive Antwort auf die subkortikale Stimulation in der Wand der Tumorhohle wurde dagegen nur in funf von zehn Fallen erreicht, bei denen der Abstand zwischen Stimulationsort und Position der virtuellen Bahnen ≤ 1 cm war. In allen Fallen mit negativem Ergebnis war der Abstand groser. Wenn sich die Zuverlassigkeit des beschriebenen Fiber Tracking bei weiteren Untersuchungen bestatigt, sollte die Methode als wichtige Komponente in die Neuronavigationssysteme aufgenommen werden.

The aim of this study was to evaluate the applicability, sensitivity, and predictive power of diffusion-weighted MR imaging (DWI) in the diagnosis of vertebrobasilar infarction. From 1997 to 2002, we prospectively recruited 268 patients with acute signs and symptoms suspective of vertebrobasilar ischemia. The patients underwent biplanar EPI-T2 and EPI DWI within 24 h after onset of symptoms and high-resolution MRI as a control within 7 days. One hundred twenty-one patients had additional CT scanning. The DWI revealed acute vertebrobasilar infarction in 71.0%. The mean time exposure of DWI was 8 min and thus no more than that of CT imaging. It showed significantly more acute lesions than CT imaging (28.0%), but additional high-resolution MRI was not able to reveal more lesions than DWI alone. Even in 42 patients with reversible brainstem or cerebellar symptoms classified as TIA or PRIND, DWI demonstrated acute ischemia in 42.8%. Sixty-three patients with optimal final diagnosis of vertebrobasilar ischemia had normal DWI. One week after onset of symptoms, 88.9% of these patients had recovered completely or showed minimal symptoms. Therefore, DWI is a sensitive indicator of acute vertebrobasilar ischemia. It is no more time-consuming than CT imaging, and normal DWI is a predictor of good clinical outcome in patients with brainstem or cerebellar infarction.

Three patients with acute large paramedian ponto-mesencephalic infarctions developed a bilateral retrograde degeneration of the medial cerebellar peduncles within 4 months after the insult. In an initial magnetic resonance imaging (MRI) within the first 2 weeks, the medial cerebellar peduncles showed normal intensities, but a control MRI after 4 months showed bright hyperintensities in the T2-TSE weighted images, and moderately increased signal intensities in echo planar imaging-diffusion weighted imaging were seen, possibly representing bilateral Wallerian degeneration of the cerebellar-pontine fibers.

Purpose To determine if the diffusion tensor imaging (DTI) parameters fractional anisotropy (FA) and mean diffusivity (MD) can differentiate between accompanying edema and tumor cell infiltration of white matter (WM) beyond the tumor edge as defined from conventional MRI in low- and high-grade gliomas. Materials and Methods We examined 12 patients with high-grade gliomas/glioblastomas and eight patients with low-grade gliomas and compared them to 10 patients with meningiomas, in which no tumor infiltration is expected. The tumor was defined as the enhancing area in glioblastomas and meningiomas and as the area of increased T2-signal in low-grade gliomas. FA and MD were measured in the center of the tumor and in the adjacent WM. The contralateral WM and internal capsule were used as an internal standard. Results Comparing the WM areas of increased T2-signal adjacent to meningiomas and glioblastomas, we saw a trend (without significance) towards a reduction of FA, but not of MD, in glioblastomas. We found no changes of FA and MD in the WM adjacent to low-grade gliomas (without T2-signal increase) compared to the WM of the contralateral hemisphere. In meningiomas and high-grade gliomas/glioblastomas, a narrow rim of significantly (P < 0.01) increased FA and decreased MD values around the enhancing tumor area was seen, whereas in low-grade gliomas, such a rim could not be defined. There was no contribution of FA or MD to grading of gliomas. Conclusion In glioblastomas, a reduction of FA in the edematous area surrounding the tumor may indicate tumor cell infiltration, but a reliable differentiation between infiltration and vasogenic edema is not yet possible on the basis of DTI. The additional finding of a narrow rim of increased FA and decreased MD at the edge of glioblastomas (as well as in meningiomas) may be caused by com-pressed WM fibers and/or increased vascularity, but does not contribute to exclude peripheral cellular infiltration. J. Magn. Reson. Imaging 2004;20:905–912. © 2004 Wiley-Liss, Inc.

Mild cognitive impairment (MCI) is considered to be a transitional stage between normal aging and dementia. In Alzheimer’s disease (AD), white matter structural pathology is due to Wallerian degeneration and central angiopathy. However, in MCI patients, the presence and extent of white matter alterations as a possible correlate of impaired memory function and as predictor of subsequent progression to AD is not clarified yet. Diffusion tensor imaging (DTI) reveals the ultrastructural integrity of cerebral white matter tracts. Therefore, it could detect pathological processes that modify tissue integrity in patients with MCI. In our prospective study, conventional and diffusion tensor MR scans were obtained from 14 patients with MCI, 19 patients with AD, and 10 healthy controls. Mean diffusivity (MD) and fractional anisotropy (FA) were measured in temporal, frontal, parietal and occipital white matter regions as well as in the corpus callosum (genu and splenium) and the hippocampus. MCI patients showed higher MD values in the left centrum semiovale (p = 0.013; right: p = 0.026), in the left temporal (p = 0.006), the right temporal (p = 0.014) and the left hippocampal (p = 0.002) region as compared to the control group. FA values of MCI patients and controls did not differ significantly in any region. Compared to controls, AD patients had increased MD values in the left centrum semiovale (p = 0.012), the left parietal (p = 0.001), the right parietal (p = 0.028), the left temporal (p = 0.018), the right temporal (p = 0.011) and the left hippocampal region (p = 0.002). Decreased FA values were measured in the left temporal area (p = 0.017) and in the left hippocampus (p = 0.031) in AD patients compared to controls. FA and MD values did not differ significantly between AD and MCI patients. Elevated MD values indicating brain tissue alterations in MCI patients were found in regions that are typically involved in early changes due to AD, particularly the left hippocampus. The sensitivity of distinguishing MCI patients from controls was 71.4% (with a specificity set at 80%). Therefore, the DTI technique validates the MCI concept, and diffusion tensor MR measurement can be a helpful tool to quantify MCI pathology in vivo.

Seit langer Zeit sind typische Hirnstammsyndrome bekannt. Eines der haufigsten ist das Wallenberg- Syndrom bei dorsolateralem Medulla-oblongata-Infarkt. Ursache ist hier meist ein Verschluss der ipsilateralen PICA. Die Vielzahl der Syndrome auf der einen Seite sowie die Klassifikation der Hirnstamminfarkte in Gruppen andererseits konnen als Hinweis darauf angesehen werden, dass die Hirnstamminfarkte regelmasig bestimmte Territorien und Gebiete bevorzugen. Inwieweit diese Hypothese zutrifft, sollte an einem Patientenklientel mit akuten Hirnstamminfarkten untersucht werden. Ziel unserer Arbeit war die Darstellung typischer Lasionsverteilungen bei Patienten mit akutem Hirnstamminfarkt. Bei 117 Patienten mit akutem, klinisch und im MRT nachgewiesenem Hirnstamminfarkt wurden die MRT-Lasionen (T2-w) normalisiert und schichtweise ubereinander projiziert. Zur besseren Vergleichbarkeit wurden alle Lasionen auf dieselbe Seite projiziert. Die Lasionen wurden sowohl anatomisch mit Hilfe anatomischer Atlanten korreliert als auch zu bekannten Darstellungen der Hirnstammdurchblutung in Verbindung gesetzt. Die 117 Patienten (36 Frauen, 81 Manner) waren im Mittel 63,5 Jahre alt (Median 64,5; SD 12,7 Jahre). 85 Patienten (73%) hatten solitare frische Hirnstamminfarkte, 19 Patienten (16%) wiesen zusatzlich frische Kleinhirninfarkte auf, neun Patienten (8%) zusatzlich frische supratentorielle Infarkte. Vier Patienten (3,4%) hatten multiple frische Infarkte in mindestens drei Gefasterritorien. 65 Patienten (56%) wiesen im MRT keine Hinweise auf vorangegangene Ischamien auf. Die Hirnstamminfarkte zeigten typische Verteilungsmuster in unterschiedlichen Hohen in Bezug auf Ausdehnung, Schwerpunkt und im Verhaltnis zur Mittellinie, den auseren Begrenzungen des Hirnstammes und den bekannten Gefasterritorien. In unserer Studie lag das Maximum nach Uberlagerung aller Infarkte in Hohe der Medulla oblongata im lateralen Gefasterritorium, welches von den Vertebralarterien uber die PICA versorgt wird. In Ubereinstimmung mit der klinischen Erfahrung, dass paramediane Ponsinfarkte die “typischen“ Ponsinfarkte darstellen, fanden wir das Maximum aller pontinen Infarkte weiter ventral in den anteromedialen und anterolateralen Gefasterritorien, hervorgerufen durch Gefasverschlusse pontiner Aste aus der Arteria basilaris. Mesenzephal schlieslich lag das Maximum aller Infarkte erneut im anteromedialen und anterolateralen Gefasterritorium. Diese Untersuchung kann als Basis dienen, uber die Analyse der Lasionsverteilung „typisch“ lokalisierte Hirnstamminfarkte von atypischen Lasionen zu differenzieren. Dies konnte insbesondere bei einzelnen, differentialdiagnostisch fraglichen Lasionen hilfreich sein.

The present fMRI study aimed to investigate effective connectivity within a cortical-subcortical-cerebellar information processing network in drug free schizophrenic patients while performing a 2-back working memory task. The finding of enhanced thalamo-cortical and cortico-cortical intrahemispheric connectivity could be interpreted as a compensatory increase of neuronal connection strength consistent with a model of cortical inefficiency in schizophrenic patients. Additionally, the result could be integrated into a model of deficient thalamo-cortical filter functions. Conversely, lower interhemispheric connectivity of the frontal and parietal association cortex appears to be the functional correlate of reduced cognitive performance in schizophrenic patients. The study demonstrated the feasibility to model information processing within cognitive networks and provided additional evidence for the concept of cognitive dysmetria in schizophrenia.

Im Rahmen dieser Studie sollte untersucht werden, ob sich eine diffusionsgewichtete Sequenz eignet, unter Ausnutzung der anisotropen Diffusionsverhaltnisse entlang der Markfasern die Zentralregion (ZR) bei Probanden und Patienten verlasslich darzustellen. Funf Patienten mit Hirntumoren und 20 gesunde Probanden wurden an einem klinischen 1,5-TMR-Tomographen untersucht. Bei den Probanden dienten die „klassischen“ anatomischen Zeichen zur Lokalisation der Zentralregion an einem 3-D-T1-Datensatz als Goldstandard zur Lokalisation, bei den Patienten zusatzlich noch ein funktionelles Magnet-resonanztomogramm (fMRT) mit einem Finger-Tapping-Paradigma im Blockdesign und der intraoperative Befund. Bei den Probanden konnte die Zentralregion in allen Fallen durch ihre Faserverlaufsrichtung und dadurch selektiv in anterior-posteriorer Richtung behinderte Diffusion lokalisiert werden. Hierzu waren keine weiteren Nachverarbeitungsprozesse notwendig. Bei den Patienten bestand eine gute Korrelation zwischen Diffusionswichtung-(DWI-)Befund, den fMRT-Ergebnissen und der intraoperativen Lokalisation. Mit EPI-DWI kann man bei Probanden und Patienten reproduzierbar, einfach und mit hoher Zuverlassigkeit die ZR darstellen.

The localization of epileptic foci is an important issue in children with extratemporal epilepsies. However, the value of noninvasive methods such as the EEG-assisted functional magnetic resonance imaging (fMRI) has not been sufficiently investigated in children. As a model of extratemporal epilepsies, we studied 7 patients aged 5 to 12 (median 10) years with benign childhood epilepsy and centrotemporal (rolandic) spikes. Interictal spikes were recorded during the fMRI acquisition on a MR-compatible battery-powered digital EEG system with 16 channels. The fMRI sequences were correlated off-line with the EEG spikes and analyzed with the software Statistical Parametrical Mapping SPM99. The fMRI results demonstrated the spike-related activation in the perisylvian central region in three patients; we could not demonstrate fMRI activation despite active spiking in 2 patients, and 2 patients did not produce sufficient spikes for fMRI analysis. We currently consider the spike-related fMRI as a research tool that localizes epileptic activity in selected patients. Further improvements of the technique are necessary to allow a clinical application of this method.

The function and course of the cortico-respiratory projections in man are not yet well established. In 30 normal volunteers respiratory muscles were activated by magnetic stimulation of the motor cortex and the cervical and thoracic spinal roots with bilateral recordings from the respiratory muscles. Following cortical stimulation contralateral responses were obtained in all subjects during voluntary inspiration showing a mean latency and amplitude of 13.4±1.4ms/1.6±1.2 mV (stimulation of the left hemisphere) and 13.2±1.3ms/2.5±2.5 mV (stimulation of the right hemisphere). Ipsilateral responses were obtained in only 18 (right side) and 21 (left side) subjects and had a significantly (p < 0.001) longer mean latency and lower mean amplitude than the contralateral responses. In 31 patients with impairment of the cortico-respiratory projections due to cerebral infarction demonstrated by magnetic resonance imaging studies, the responsible lesion topography was analysed. We found that, 1. the voluntary activation of respiratory muscles is mediated predominantly by the contralateral motor cortex, 2. the descending cortico-respiratory projections are located within the pyramidal tract, and 3. the cortico-respiratory projections to the respiratory muscles are frequently affected in patients with hemiparesis due to acute stroke. These findings might explain the increased incidence of pneumonia at the hemiparetic side in patients with cerebral ischemia as shown in previous studies.

Purpose To investigate if primary cholesteatomas of the petrous bone show high signal in diffusion-weighted imaging (DWI). Materials and methods In this blinded study, we compared 15 patients with clinically certain cases and later surgically proven cholesteatomas vs. 12 patients with clinically acute otitis of the middle ear and 20 volunteers without petrous bone disease. Two blinded readers without knowledge of the clinical data decided in consensus agreement whether there was a pathologic signal increase in the petrous bone in an anisotropic single-shot echo-planar imaging (EPI) DWI sequence, an artifact, or no signal increase. Results Thirteen of 15 patients with cholesteatomas showed bright signal in EPI DWI, whereas 10 of 12 patients with acute otitis media and all volunteers presented the usual low signal of petrous bone. Conclusion EPI DWI is a fast diagnostic method that may be an additional valuable tool in the workup of suspected cholesteatomas. The ability of this technique to differentiate between cholesteatomas and granulomas or chronic otitis is not yet available.

There is a significant number of individual patients with cranial nerve palsies as the sole manifestation of MRI- and, less frequently, CT-documented small brainstem infarctions or hemorrhages. The 3rd and 6th nerves are most commonly involved and, less frequently, the 4th, 5th, 7th, and 8th nerves. An intra-axial basis for such lesions may be underestimated if the diagnosis is based solely on MRI. The electrophysiologic abnormalities indicating brainstem lesions may be independent of MRI-documented morphological lesions. This paper reviews the literature on cerebrovascular brainstem diseases manifesting as isolated cranial nerve palsies. It supports the concept that small pontine and mesencephalic infarctions are the main cause of non-traumatic cranial nerve palsies in the middle-aged and elderly population. Microvascular infarction of the respective extra-axial cranial nerve segments seem to be less important.

The case presented describes the simultaneous occurrence of an unusually large anterior fontanellar bone with a syndrome of vascular malformation and overgrowth in a three-month-old child, which to our knowledge has not yet been reported. This combination may strengthen the arguments for a possible genetic contribution to the occurrence of supernumerary ossicles in the skull. Although of minor clinical importance, the shape and variations of these Wormian bones should be well-known to prevent misleading interpretations of imaging Results.

In tropical spastic paraparesis, spinal cord atrophy is a well-known finding in magnetic resonance imaging. But in contrast to histological reports, focal lesions of the spinal cord have only been described in imaging reports in exceptional acute cases. Here, we looked for such focal lesions and for alterations of diffusion tensor imaging parameters of the long fibre tracts in the usual case of a long-standing and slowly progressive disease. We examined 10 symptomatic patients, 11 seropositive, but asymptomatic human T-lymphotrophic virus type 1 carriers and 18 seronegative volunteers as controls. Sagittal and transversal T2-weighted images were visually assessed for atrophy and focal cord lesions. The spinal cord cross-sectional areas and the segmental cord volumes were measured at all levels. High-resolution diffusion tensor imaging was performed in sagittal planes from the bregma down to the cervical spine. For tractography and calculation of fractional anisotropy and mean diffusivity, we used manufacturer-provided software. Two-thirds of patients showed focal lesions affecting the antero-lateral columns and in two cases also the dorsal columns. Compared to carriers and volunteers, patients presented a significant spinal cord atrophy and a reduction of fractional anisotropy (p

Background Pantothenate Kinase-Associated Neurodegeneration (PKAN) is a rare heritable disease marked by dystonia and loss of movement control. In contrast to the well-known “Eye-of-the-Tiger” sign affecting the globus pallidus, little is known about other deviations of brain morphology, especially about grey matter changes. Methods We investigated 29 patients with PKAN and 29 age-matched healthy controls using Magnet Resonance Imaging and Voxel-Based Morphometry. Results As compared to controls, children with PKAN showed increased grey matter density in the putamen and nucleus caudatus and adults with PKAN showed increased grey matter density in the ventral part of the anterior cingulate cortex. A multiple regression analysis with dystonia score as predictor showed grey matter reduction in the cerebellum, posterior cingulate cortex, superior parietal lobule, pars triangularis and small frontal and temporal areas and an analysis with age as predictor showed grey matter decreases in the putamen, nucleus caudatus, supplementary motor area and anterior cingulate cortex. Conclusions The grey matter increases may be regarded as a secondary phenomenon compensating the increased activity of the motor system due to a reduced inhibitory output of the globus pallidus. With increasing age, the grey matter reduction of cortical midline structures however might contribute to the progression of dystonic symptoms due to loss of this compensatory control.

Background: Although intracranial Rosai–Dorfman disease is a principally benign lymphohistiocytosis, some patients run a relapsing or progressive course. However, reports about long-term follow-up are extremely rare. Case Description: In two patients, initial tumor resection was incomplete or followed by recurrences over 3 years, which finally subsided after application of chemotherapy, and patients remained tumor-free for more than 7 years thereafter. Conclusion: Up to now there is no agreement on how to treat complicated cases of intracranial Rosai–Dorfman disease; our good experience with adjuvant chemotherapy and long-term follow-up will contribute to treatment planning in complicated cases.

We acquired high-resolution MRI and anisotropically diffusion-weighted images (DWI) with direction-selective gradients of the brain stem in 20 healthy volunteers, to identify brain-stem structures such as white-matter tracts and nuclei which show diffusion anisotropy. After averaging and superposition of individual cuts, the images were projected onto appropriate plates of the Schaltenbrand and Wahren anatomical atlas. We identified 20 structures – white-matter tracts and some nuclei – with high contrast. The direction of fibres could be determined as areas of increased (parallel to) or decreased diffusion (perpendicular to the gradient). This study may contribute to understanding of the functional anatomy of the brain stem.

5-(2'-[18F]Fluoroethyl)flumazenil ([18F]FEF) is a fluorine-18 labelled positron emission tomography (PET) tracer for central benzodiazepine receptors. Compared with the established [11C]flumazenil, it has the advantage of the longer half-life of the fluorine-18 label. After optimisation of its synthesis and determination of its in vitro receptor affinities, we performed first PET studies in humans. PET studies in seven healthy human volunteers were performed on a Siemens ECAT EXACT whole-body scanner after injection of 100-280 MBq [L8F]FEF. In two subjects, a second PET scan was conducted after pretreatment with unlabelled flumazenil (1 mg or 2.5 mg i.v., 3 min before tracer injection). A third subject was studied both with [18F]FEF and with [11C]flumazenil. Brain radioactivity was measured for 60-90 min p.i. and analysed with a region of interest-oriented approach and on a voxelwise basis with spectral analysis. Plasma radioactivity was determined from arterial blood samples and metabolites were determined by high-performance liquid chromatography. In human brain, maximum radioactivity accumulation was observed 4 +/- 2 min p.i., with a fast clearance kinetics resulting in 50% and 20% of maximal activities at about 10 and 30 min, respectively. [18F]FEF uptake followed the known central benzodiazepine receptor distribution in the human brain (occipital cortextemporal cortexcerebellumthalamuspons). Pretreatment with unlabelled flumazenil resulted in reduced tracer uptake in all brain areas except for receptor-free reference regions like the pons. Parametric images of distribution volume and binding potential generated on a voxelwise basis revealed two- to three-fold lower in vivo receptor binding of [18F]FEF compared with [11C]flumazenil, while relative uptake of [18F]FEF was higher in the cerebellum, most likely owing to its relatively higher affinity for benzodiazepine receptors containing the alpha6 subunit. Metabolism of [18F]FEF was very rapid. Polar metabolites represented about 50%-60% of total plasma radioactivity at 5 min and 80%-90% at 20 min p.i. Although [11C]flumazenil has some advantages over [18F]FEF (higher affinity, slower metabolism, slower kinetics), our results indicate that [18F]FEF is a suitable PET ligand for quantitative assessment of central benzodiazepine receptors, which can be used independently of an on-site cyclotron.

We investigated the reliability of a new digital post-processing magnetic resonance imaging (MRI) technique in ischemic brain stem lesions to identify relations of the lesion to anatomical brain stem structures. The target was a medial longitudinal fasciculus (MLF) lesion, which was evident from ipsilateral internuclear ophthalmoplegia (INO). Sixteen patients with acute unilateral INO and an isolated acute brain stem lesion in T2- and EPI-diffusion weighted MRI within 2 days after the onset of symptoms were studied. The MRI slice direction was parallel and perpendicular to a slice selection of a stereotactic anatomical atlas. The individual slices were normalized and projected in the digitalized atlas. The eye movement disorder was monitored by electro-oculography. In all patients with clinical or subclinical electro-oculographically documented INO and MRI proven brain stem infarction the lesion covered or at least partially overlapped the ipsilateral MLF at one or more atlas levels. We conclude that digital post-processing MRI with normalizing and projecting brain stem lesions in an anatomical atlas is a reliable method to demonstrate the anatomical structures involved by the lesion. Combined with electrophysiological brain stem testing, this method may be a useful tool to identify incompletely understood pathways mediating brain stem reflexes or the generators of evoked potentials.

Aim: CT and MR in the diagnosis and course of Graves’ disease (endocrine orbitopathy) are presented and discussed. Value of CT and MRT: Both methods are able to show exactly the morphological changes, with thickening of the external eye muscles relative to the tendon and the extension of proptosis. MR is additionally able to define – on the basis of T2 measurements – the acuity of the disease, a chronic course with fatty degeneration and muscle fibrosis. Conclusion: The complexity of the disease is an interdisciplinary challenge to all physicians dealing with these patients. Even in times of cost reduction in healthcare the use of CT and MR is mandatory, as it helps to define and control the therapy.

The purpose of this study was to identify clinical predictors and anatomical structures involved in patients with pain after dorsolateral medullary infarction. Eight out of 12 patients (67%) developed poststroke pain within 12 days to 24 months after infarction. The pain occurred in the ipsilateral face (6 patients) and/or the contralateral limbs and trunk (5 patients, 3 of whom also had facial pain). Ipsilateral facial pain was significantly correlated with lower medullary lesions, including those of the spinal trigeminal tract and/or nucleus, as documented by magnetic resonance imaging. The R2 blink reflex component was abnormal only in patients with facial pain. Likewise, pain and temperature sensation in the ipsilateral face was decreased in all patients with facial pain but not in patients without pain. Ipsilateral touch sensation in the face was also decreased in all patients with facial pain, but the lesions revealed on magnetic resonance imaging did not involve the principal sensory nucleus of the fifth cranial nerve, and the R1 blink reflex latencies were normal. Although facial pain was correlated with lesions of the spinal trigeminal tract and/or nucleus, none of the lesions involved the subnucleus caudalis, which contains most nociceptive neurons. These findings suggest that facial pain after medullary infarction is due to lesions of the lower spinal trigeminal tract (axons of primary afferent neurons), leading to deafferentation of spinal trigeminal nucleus neurons.

In order to investigate the diagnostic properties of MRI of the brain and spine in achondroplastic children with regard to decompressive surgery, 25 patients were examined by conventional morphological and by "functional" imaging of CSF flow and magnetic resonance angiography (MRA) of the veins and sinuses at the cranial base following a special protocol. The results were compared to those from age-matched controls and were correlated with each other and retrospectively with the neurological findings. Measurements of distances and angulations at the cranio-cervical junction (CCJ) from MR scans showed similar values to those from conventional radiographs and CTs and thus can be used without correction for spatial distorsion. Signs of cervical medullary compression, myelomalacia and intramedullary cyst formation were found in six, seven and three children respectively. These alterations correlated significantly with each other (P0.05). Semiquantitative evaluation of CSF flow demonstrated interruption of CSF pathways at the CCJ, which correlated with CCJ narrowing (P0.05). MRA showed a significant narrowing of the jugular foramina with a variable compensatory enlargement of the emissary veins and a significant reduction of the total outflow area (P0.01). There were no significant correlations between these MR changes and neurological deficits.Due to this unexpectedly poor correlation between magnetic resonance and clinical findings in achondroplastic children, the present role of magnetic resonance in the clinical setting is limited to the demonstration of spinal cord compression in individual cases. In three of our patients with prominent neurological abnormalities, the severe changes demonstrated by magnetic resonance imaging strongly supported the indication for surgical decompression.

In einer prospektiven Studie untersuchetn wir 106 konsekutiv eingeschlossene Patienten mit apoplektiform auftretender Dysarthrophonie aufgrund eines im MRT gesicherten singluaren, nicht raumfordernden Infarkts.

Fragestellung: Im Kleinhirnbruckenwinkel (KHBW) vereinigen sich der N. vestibulocochlearis und facialis, bevor sie gemeinsam in den inneren Gehorgang eintreten. Pathologische Prozesse in dieser Region sind in der Regel mit Funktionseinbusen des VII. und/oder VIII. Hirnnerven verbunden. Das Akustikusneurinom gilt aus haufigste Ursache eines KHBW-Syndroms, das aber auch durch entzundliche oder vaskulare Prozesse verursacht werden kann. Vaskulare Malformationen stellen dabei eine Ausnahme dar. Patienten/Methoden: Es werden 5 klinische Falle beschrieben, bei denen eine Schleifenbildung der A. basilaris, A. vertebralis oder A. cerebelli inferior anterior im KHBW-Bereich als Ursache von vestibulokochlearen Storungen in Form von Drehschwindel, Horminderung oder pulssynchronen Ohrgerauschen in Frage kam. Bei 2 Patienten konnte diese Annahme durch eine operative Intervention bestatigt werden. Im Rahmen der Differentialdiagnostik wurden die Befunde der MRT des Cerebrums, der Angiographie, der BERA, der Tonaudiometrie und des ENG erhoben. Ergebnisse/Schlusfolgerungen: Trotz der haufigen tumorosen Ursachen konnen abnorme Gefasschlingen kausal fur ein KHBW-Syndrom sein und mussen differentialdiagnostisch in Erwagung gezogen werden.

Far-field somatosensory evoked potentials (SSEPs) after median nerve stimulation were recorded from scalp- (Fz), epidural- (ED) and intravascular electrodes (basilar artery [Bas]) to study the nature of the controversial N18a component of the widespread N18 potential. In healthy volunteers frequently an N18a potential was recorded at Fz. Simultaneous Fz and ED recordings at the pontomesencephalic junction as well as Bas-recordings at the caudal basilar artery showed N18a components identical in latency and shape. With intravascular recordings the shapes differed between the top of the basilar artery and the caudal artery recordings. These findings support the existence of a separate N18a potential. The generator of the N18a is likely to be localized within the upper brainstem.

Springer-Verlag 2009 Sirs, Rheumatoid meningoencephalitis is a rare but usually lethal extra-articular manifestation of rheumatoid arthritis (RA) in patients with longstanding seropositive disease. Only 25 histopathologically proven cases have been reported. We describe a patient refractory to conventional immunosuppressive treatments, but whose disease temporarily stabilized over 12 months by administering the anti

OBJECTIVES—Pathways of late blink reflexes are detected by high resolution MRI. Electronically matched stroke lesions superimposed to an anatomical atlas show the suspected course. METHODS—Fifteen patients with infarction of the lower brainstem, MRI lesions and electrically elicited blink reflexes were examined. The involved structures in patients with R2 and R2c blink reflex changes were identified by biplane high resolution MRI with individual slices matched to an anatomical atlas at 10 different levels using digital postprocessing methods. RESULTS—The blink reflexes were normal in five of 15 patients (33%) and showed loss or delay of R2 and R2c to stimulation ipsilaterally to lesion (R2-i and R2c-i) in eight (53%). Loss or delay of R2-i/R2c-i was seen in lesions covering the entire trigeminal spinal tract and nucleus (TSTN) at at least one level. These infarctions were located more dorsally within the medulla. Patients with normal blink reflexes showed lesions sparing or involving the TSTN only partially. They more often had incomplete Wallenberg's syndromes and MRI lesions were located more ventrally. CONCLUSIONS—Using digital postprocessing MRI methods it was possible to identify central pathways of late blink reflex in patients with Wallenberg's syndrome. This method is suggested as a new approach to identify incompletely understood functional structures of the brainstem.

We performed MRI, including diffusion-weighted imaging, in 15 patients with recurrent strokes with acute ischaemia and at least one old lesion according to the clinical history and/or CT. Routine MRI showed similar signal intensity changes in both situations. Diffusion-weighted images, however, were positive in all acute or subacute infarcts. The high signal of acutely disturbed diffusion due to intracellular oedema could also be identified in small brain stem lesions. Spatial resolution was increased by applying separate gradients in each axis instead of creating anisotropy-independent trace images.

We show the capability and results of MRI examinations of peracute brainstem and hemispheric stroke patients in clinical emergency situations. The hypothesis that CT scan is faster and more accurate under this conditions is to be disproved.

A new method is introduced to project the sulcal pattern of the brain surface onto a 2D plane. Twin brains are compared against each other using the planar representation. We obtained T1-weighted Flash-3D MRI volumes from 14 male twins (seven monozygotic, seven dizygotic) with 3 mm-thick coronal slices. The projection is based on potential theory: A virtual electrostatic field is calculated between the area of the segmented brain and a surrounding spherical electrode. Field lines starting from each border point of the segmented brain follow the gradient towards the sphere, leading to field line concentrations due to the underlying sulci. The unwrapped sphere surface with the number of field lines per area unit is used as the 2D representation of the sulcal pattern. The resulting brain projections show a distinctive pattern, and a visual assignment of the twin pairs from the unsorted set is possible because of a high similarity of the patterns between twin pairs. Global correlation coefficients for each pair of maps yield significantly higher values for matching monozygotic twin pairs (mean = 20.2, range 12.3-25.6) than for unmatched pairs (mean = 13.0, range 1.1-28.5). As a conclusion, our method allows us to map the location and depth of the sulci on a 2D plane. The resulting maps allow quantitative inter-individual comparisons on the entire brain or parts of the brain surface.

Reduced N-acetyl aspartate (NAA) and increased myo-inositol (MI) levels have been reported in patients with dementia of Alzheimer type (DAT) in comparison with controls. We wished to assess the validity of these findings and to evaluate possible correlations of metabolite proportions with cognitive dysfunction in DAT. Twelve patients with DAT and 10 healthy age-matched controls were included. The severity of dementia was assessed using different scales including the Mini-Mental State Examination. MRS was performed with a conventional 1.5 Tesla scanner in a single voxel in the centrum semi-ovale (TE = 30 ms or TE = 136 ms; TR = 1500 ms). The evaluation of MRS results was limited by low interrater, intermeasurement (different echo times) and test-retest reliabilities, by a high interindividual variance and by the failure to measure absolute metabolite concentrations. These problems in mind, it was remarkable that previously reported reductions of NAA levels in patients with DAT could be reproduced in the present sample. The proportion of NAA was diminished in demented subjects in comparison with controls (37% vs 44.90%, short TE). A non-significant trend towards minor reductions of creatine, choline and MI proportions in these subjects might indicate that proportions of other metabolites necessarily increase when NAA is reduced. Cognitive dysfunction of demented subjects was significantly correlated with reductions of NAA, but not with increases of MI. Due to the present technical and methodological problems and to the non-specificity of findings, proton MRS cannot be applied to support the diagnosis of DAT in a clinical setting.