Eberhard Siebert

The clinical and radiological spectrum of posterior reversible encephalopathy syndrome: the retrospective Berlin PRES study

The aim of the study was to characterize the clinical and radiological spectrum of posterior reversible encephalopathy syndrome (PRES) in a large cohort. The radiological report data bases of the authors′ university hospitals were searched for patients with PRES. Various imaging features at onset of symptoms and on follow-up as well as clinical and paraclinical data were tabulated in those patients fulfilling the criteria for PRES. Exploratory univariate analyses were performed. A total of 96 patients with PRES were included into the study. Wide differences in lesion location, diffusivity, distribution pattern, edema severity, hemorrhage, underlying diseases, symptoms, mean arterial pressure (MAP) and coagulation status were encountered. Hemorrhage occurred significantly more frequently in patients with altered coagulation state and was significantly associated with higher edema grades and with the presence of cytotoxic edema. There was a significant difference in MAP between toxic associations with higher MAP in infection, eclampsy and autoimmune disorders, while lower MAP was found in chemotherapy and immunsupression. In 82% of patients complete or near complete resolution of edema was noted during follow-up. Higher MAP levels were associated with incomplete edema resolution. In 43% of patients residual lesions were seen with a relatively even distribution between focal gliosis, infarction, posthemorrhagic residua, atrophy and laminar necrosis. PRES in this large hospital-based retrospective study comprises a wide radiological and clinical spectrum. Residual lesions were encountered more frequently than commonly expected. Our results point towards a differential contribution of high blood pressure to the course of PRES in different underlying etiologies.

Enriched environment induces cellular plasticity in the adult substantia nigra and improves motor behavior function in the 6-OHDA rat model of Parkinson's disease

The adult substantia nigra bears the capacity to generate new neural cells throughout adulthood. The mechanisms of cellular plasticity in this brain region remain unknown. In the adult dentate gyrus, dopamine was suggested to be one of the key players in neurogenesis. We therefore investigated nigral cellular plasticity in the 6-OHDA rat model of Parkinsons disease. The absolute numbers of newborn cells in the SN were not affected by dopamine depletion. Interestingly, we found a specific downregulation of generation of newborn nigral astrocytic cells. As enriched environment with physical activity are robust inducers of neuro- and gliogenesis in the adult DG, we investigated the role of these physiological stimuli in nigral cellular plasticity and in motor behavior of 6-OHDA lesioned rats. We describe a significant increase in numbers of newborn NG2-positive and GFAP-positive cells in the SN. Moreover, 6-OHDA lesioned animals living in enriched environment with physical activity for 7 weeks showed improved motor behavior compared to controls under standard conditions. Thus, physiological neurogenic and gliogenic stimuli induce significant microenvironmental changes in the adult SN and improve motor behavior in the 6-OHDA lesion model of PD.

4-D Imaging in Cerebrovascular Disorders by Using 320-Slice CT: Feasibility and Preliminary Clinical Experience

RATIONALE AND OBJECTIVES The authors report study protocols and initial clinical experience in assessing patients with acute and chronic cerebrovascular disorders using the recently introduced technique of volume computed tomography (VCT). MATERIALS AND METHODS Thirteen patients with presumptive cerebrovascular insufficiency underwent VCT using a 320-slice scanner (detector width, 160 mm), including time-resolved whole-brain perfusion and cerebral angiography (four-dimensional computed tomographic angiography [CTA] and computed tomographic perfusion [CTP]). Unenhanced cranial CT (cCT) and helical cervicocranial CT (three-dimensional CTA) were added according to clinical requirements. Study protocols are presented, and image quality, data management, and radiation exposure issues are discussed. RESULTS In 12 of 13 patients, the procedure was performed successfully on admission; in the other patient, the study was aborted for clinical reasons and repeated. Total scan time amounted to about 5 minutes, and data reconstruction times were up to 10 minutes. About 9000 primary images were generated, partially in the enhanced Digital Imaging and Communications in Medicine format, thus requiring new data postprocessing and management strategies. Image artifacts restricted the use of single-rotation cCT and incremental VCT (three-dimensional CTA). Overall exposure figures (computed tomographic dose index and dose-length product) were increased by 65% on average when three-dimensional CTA was added to volume cCT and four-dimensional CTA and CTP (5.0 mSv and 2178 mGy . cm, respectively). CONCLUSION Preliminary clinical experience indicates that whole-brain four-dimensional CTA and CTP is a robust technique that provides relevant clinical information with respect to whole-brain perfusion as well as cerebral hemodynamics. The exposure benefit of deriving time-resolved perfusion and vessel images from one source data set is compromised when adding three-dimensional CTA to the protocol. Other acquisition techniques specific to VCT, such as single-rotation cCT and incremental three-dimensional CTA, suffer from restrictions in terms of image quality at present.

Lumbar spinal stenosis: syndrome, diagnostics and treatment

Lumbar spinal stenosis (LSS) comprises narrowing of the spinal canal with subsequent neural compression, and is frequently associated with symptoms of neurogenic claudication. To establish a diagnosis of LSS, clinical history, physical examination results and radiological changes all need to be considered. Patients who exhibit mild to moderate symptoms of LSS should undergo multimodal conservative treatment, such as patient education, pain medication, delordosing physiotherapy and epidural injections. In patients with severe symptoms, surgery is indicated if conservative treatment proves ineffective after 3–6 months. Clinically relevant motor deficits or symptoms of cauda equina syndrome remain absolute indications for surgery. The first randomized, prospective studies have provided class I–II evidence that supports a more rapid and profound decline of LSS symptoms after decompressive surgery than with conservative therapy. In the absence of a valid paraclinical diagnostic marker, however, more evidence-based data are needed to identify those patients for whom the benefit of surgery would outweigh the risk of developing complications. In this Review, we briefly survey the underlying pathophysiology and clinical appearance of LSS, and explore the available diagnostic and therapeutic options, with particular emphasis on neuroradiological findings and outcome predictors.

Clinical and radiological differences in posterior reversible encephalopathy syndrome between patients with preeclampsia‐eclampsia and other predisposing diseases

Background:  Posterior reversible encephalopathy syndrome (PRES) is a serious maternal complication in pregnancy, but data on the clinicoradiological differences to other etiologies of PRES are scarce. In this study, we aimed to investigate the clinical and imaging characteristics of PRES in preeclampsia‐eclampsia patients compared with other predisposing diseases in a large cohort.

Scatter-based magnetic resonance elastography

Elasticity is a sensitive measure of the microstructural constitution of soft biological tissues and increasingly used in diagnostic imaging. Magnetic resonance elastography (MRE) uniquely allows in vivo measurement of the shear elasticity of brain tissue. However, the spatial resolution of MRE is inherently limited as the transformation of shear wave patterns into elasticity maps requires the solution of inverse problems. Therefore, an MRE method is introduced that avoids inversion and instead exploits shear wave scattering at elastic interfaces between anatomical regions of different shear compliance. This compliance-weighted imaging (CWI) method can be used to evaluate the mechanical consistency of cerebral lesions or to measure relative stiffness differences between anatomical subregions of the brain. It is demonstrated that CWI-MRE is sensitive enough to reveal significant elasticity variations within inner brain parenchyma: the caudate nucleus (head) was stiffer than the lentiform nucleus and the thalamus by factors of 1.3 +/- 0.1 and 1.7 +/- 0.2, respectively (P < 0.001). CWI-MRE provides a unique method for characterizing brain tissue by identifying local stiffness variations.

Characterization of orbital masses by multiparametric MRI.

OBJECTIVES DWI and dynamic contrast enhanced (DCE) MR imaging are techniques that allow insight to tumor vascularity and cellularity. We evaluated the diagnostic performance of multiparametric MRI (mp-MRI) in distinguishing benign from malignant orbital masses using standard anatomic imaging (sAI), DWI and DCE. MATERIALS AND METHODS This prospective IRB approved study with written informed consent included 65 patients. mp-MRI at 3 Tesla including DWI and DCE was performed in all patients. Parametric maps were generated for obtaining the perfusion parameters including K(trans), kep, ve and iAUC and time-signal intensity curves were recorded to determine the curve pattern. Two radiologists rated the likelihood of malignancy on a five-point scale in three separate, randomized reading sessions (initially only sAI, afterwards sAI+either DWI or DCE and finally sAI+DWI+DCE). Data was statistically analyzed. RESULTS 33 Patients had malignant orbital masses and 32 patients had benign orbital masses (reference standard histopathology in 35 cases and clinical follow-up in 30 patients). The mean ADC of malignant masses differed significantly from the mean (SD) ADC of benign masses (0.825 [0.437]×10(-3)mm(2)/s and 1.257 [0.576]×10(-3)mm(2)/s, respectively) (p=0.001). K(trans), kep and iAUC were significantly higher in malignant masses (p<0.01). The reading of sAI only resulted in a moderate specificity but poor sensitivity in differentiating benign from malignant lesions. Adding DWI and DCE images improved specificity and sensitivity considerably, being the highest for the combined reading of all sequences. CONCLUSION mp-MRI is a helpful tool in differentiating malignant orbital lesions from benign masses and should therefore be included in the routine diagnostic protocol for orbital imaging.

Anti-Ca/anti-ARHGAP26 antibodies associated with cerebellar atrophy and cognitive decline

Recently, we identified a novel Purkinje cell-specific autoantibody (termed anti-Ca) targeting rhoGTPase-activating-protein-26 (ARHGAP26) in a patient with cerebellar ataxia. Here we describe a new case of anti-Ca/ARHGAP26 antibody-positive cerebellar ataxia. Cerebellar ataxia was associated with signs of possible limbic encephalitis in this case. The 24-year-old man presented with subacute pancerebellar ataxia, flattened affect, and cognitive decline. Neuropsychological testing revealed working memory deficits, compromised verbal learning and recall, attention deficits, slowed information processing, interference difficulty, and reduced spatial recognition. MRI showed severe pancerebellar atrophy. Serological examinations revealed high-titre anti-Ca/anti-ARHGAP26 antibodies. The antibodies belonged to the IgG1 subclass and were produced intrathecally. This case further corroborates the association of anti-Ca antibodies with cerebellar ataxia, expands the clinical spectrum, and highlights the necessity of antigen-specific diagnostic testing in immune-mediated cerebellar ataxia.

Clinical and Radiological Spectrum of Posterior Reversible Encephalopathy Syndrome: Does Age Make a Difference? - A Retrospective Comparison between Adult and Pediatric Patients

Background Posterior reversible encephalopathy syndrome (PRES) is a serious and increasingly recognized disorder, but data from observational studies on clinicoradiological differences between etiologies and age groups are limited. In this study, we aimed to investigate the clinical and imaging characteristics of PRES in children compared to adults in a large cohort. Methods We retrospectively reviewed the radiological report data bases between January 1999 and August 2012 for patients with PRES (total of 110 patients). Patients fulfilling the criteria for PRES after detailed investigation of clinical charts and imaging studies were separated into children (<18years) and adults (≥18years). Various imaging features at onset of symptoms and on follow-up as well as clinical and paraclinical data were analyzed. Results A total of 19 pediatric and 91 adult patients with PRES were included into the study. In pediatric PRES patients, seizures were significantly more frequent as initial PRES-related symptom (p = 0.01). In addition, in children the superior frontal sulcus topographic lesion pattern occurred as frequent as the parieto-occipital one and was significantly more prevalent than in adults (p = 0.02). In contrast, in adults visual disturbances tended to occur more frequently than in children (p = 0.05). Also, severity of edema tended to be greater in adults than in children (p = 0.07). Conclusion In our PRES cohort, we found relevant clinicoradiological differences between pediatric and adult PRES patients. However, prospective studies are warranted to establish factors that are specifically associated with pediatric PRES.

Discharge status and in‐hospital mortality in posterior reversible encephalopathy syndrome

Posterior reversible encephalopathy syndrome is a serious and increasingly recognized disorder, but data from observational studies on outcome and mortality in posterior reversible encephalopathy syndrome (PRES) are scarce. We aimed to determine the frequency and associations of in‐hospital death and discharge status in a large cohort of patients with PRES.