A man in his sixties with gait ataxia, dysarthria and mild cognitive impairment. / En mann i 60-årene med gangvansker, dysartri og lett kognitiv svikt.

BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is an immune-mediated, treatable and inflammatory CNS disease first reported by Pittock et al. (2010). CASE PRESENTATION: We describe a 66-year-old man with previous history of diabetes, atrial fibrillation and hypertension, who was admitted to hospital with reduced general condition. He had experienced dizziness and unstable gait for a year, and had been periodically confused, especially in the previous month. MR imaging showed characteristic punctuate and curvilinear gadolinium enhancements in the pons. Our patient was diagnosed with CLIPPERS and was given corticosteroid treatment, initially methylprednisolone intravenously and then prednisone orally. Other differential diagnoses, such as CNS lymphoma, high-grade glioma, CNS vasculitis, neurosarcoidosis, demyelinating disease, Bickerstaff brainstem encephalitis, and acute disseminated encephalomyelitis were ruled out. The patient's condition improved dramatically after corticosteroid treatment. INTERPRETATION: In 2017, the diagnostic criteria for CLIPPERS were published. Based on these criteria we were able to diagnose this patient with possible CLIPPERS, consistent with clinical symptoms, MRI findings, absence of better explanations for the condition, and clinical and radiological improvement after treatment with corticosteroids. An unequivocal diagnosis of CLIPPERS can only be established by characteristic pathological findings.

Volumetric glioma quantification: comparison of manual and semi-automatic tumor segmentation for the quantification of tumor growth.

BACKGROUND: Volumetric magnetic resonance imaging (MRI) is now widely available and routinely used in the evaluation of high-grade gliomas (HGGs). Ideally, volumetric measurements should be included in this evaluation. However, manual tumor segmentation is time-consuming and suffers from inter-observer variability. Thus, tools for semi-automatic tumor segmentation are needed. PURPOSE: To present a semi-automatic method (SAM) for segmentation of HGGs and to compare this method with manual segmentation performed by experts. The inter-observer variability among experts manually segmenting HGGs using volumetric MRIs was also examined. MATERIAL AND METHODS: Twenty patients with HGGs were included. All patients underwent surgical resection prior to inclusion. Each patient underwent several MRI examinations during and after adjuvant chemoradiation therapy. Three experts performed manual segmentation. The results of tumor segmentation by the experts and by the SAM were compared using Dice coefficients and kappa statistics. RESULTS: A relatively close agreement was seen among two of the experts and the SAM, while the third expert disagreed considerably with the other experts and the SAM. An important reason for this disagreement was a different interpretation of contrast enhancement as either surgically-induced or glioma-induced. The time required for manual tumor segmentation was an average of 16 min per scan. Editing of the tumor masks produced by the SAM required an average of less than 2 min per sample. CONCLUSION: Manual segmentation of HGG is very time-consuming and using the SAM could increase the efficiency of this process. However, the accuracy of the SAM ultimately depends on the expert doing the editing. Our study confirmed a considerable inter-observer variability among experts defining tumor volume from volumetric MRIs.

Prediction of survival and progression in glioblastoma patients using temporal perfusion changes during radiochemotherapy.

BACKGROUND: The aim of this study was to investigate changes in structural magnetic resonance imaging (MRI) according to the RANO criteria and perfusion- and permeability related metrics derived from dynamic contrast-enhanced MRI (DCE) and dynamic susceptibility contrast MRI (DSC) during radiochemotherapy for prediction of progression and survival in glioblastoma. METHODS: Twenty-three glioblastoma patients underwent biweekly structural and perfusion MRI before, during, and two weeks after a six weeks course of radiochemotherapy. Temporal trends of tumor volume and the perfusion-derived parameters cerebral blood volume (CBV) and blood flow (CBF) from DSC and DCE, in addition to contrast agent capillary transfer constant (Ktrans) from DCE, were assessed. The patients were separated in two groups by median survival and differences between the two groups explored. Clinical- and MRI metrics were investigated using univariate and multivariate survival analysis and a predictive survival index was generated. RESULTS: Median survival was 19.2 months. A significant decrease in contrast-enhancing tumor size and CBV and CBF in both DCE- and DSC-derived parameters was seen during and two weeks past radiochemotherapy (p < 0.05). A 10%/30% increase in Ktrans/CBF two weeks after finishing radiochemotherapy resulted in significant shorter survival (13.9/16.8 vs. 31.5/33.1 months; p < 0.05). Multivariate analysis revealed an index using change in Ktrans and relative CBV from DSC significantly corresponding with survival time in months (r2 = 0.843; p < 0.001). CONCLUSIONS: Significant temporal changes are evident during radiochemotherapy in tumor size (after two weeks) and perfusion-weighted MRI-derived parameters (after four weeks) in glioblastoma patients. While DCE-based metrics showed most promise for early survival prediction, a multiparametric combination of both DCE- and DSC-derived metrics gave additional information.

Are the current MRI criteria using the DWI-FLAIR mismatch concept for selection of patients with wake-up stroke to thrombolysis excluding too many patients?

BACKGROUND: Up to 25% of stroke patients wake up with a neurological deficit, so called wake-up stroke (WUS). Different imaging approaches that may aid in the selection of patients likely to benefit from reperfusion therapy are currently under investigation. The magnetic resonance imaging (MRI) diffusion weighted imaging - fluid attenuated inversion recovery (DWI-FLAIR) mismatch concept is one proposed method for identifying patients presenting within 4.5 hours of the ischemic event. PURPOSE: To report our experience with the DWI-FLAIR mismatch concept for selection of wake-up stroke patients to be thrombolysed at our centre. MATERIAL AND METHODS: Patients treated with off label intravenous thrombolysis (IVT) for WUS at our centre during a 6.5-month period were included. We performed MRI including DWI and FLAIR in all patients at admission. Each MRI examination was rated as either DWI-FLAIR mismatch or match. National Institutes of Health Stroke Scale (NIHSS) and modified Rankin Scale were used to measure clinical outcome. Cerebral computed tomography (CT) or MRI was performed within 24 hours after thrombolysis to determine the presence of any intracranial haemorrhage (ICH). RESULTS: Ten patients treated with IVT for WUS were included. Four patients had a DWI-FLAIR mismatch and after IVT treatment the mean reduction in NIHSS in the DWI-FLAIR mismatch group was 4.0. In the DWI-FLAIR match group the mean reduction in NIHSS after IVT therapy was 4.8. None of the ten patients had any signs of ICH on follow-up imaging. CONCLUSIONS: In this small series DWI-FLAIR mismatch was not associated with worse outcome or ICH. This suggests that selecting WUS patients using DWI-FLAIR mismatch in clinical trials may exclude a large group of patients who might benefit.