Five-Year Data from the Interdisciplinary Giant-Cell Arteritis Registry at the University Hospital of Würzburg: Value of Temporal Artery Biopsy. / 5-Jahres-Daten des interdisziplinären Riesenzellarteriitis-Registers des Universitätsklinikums Würzburg: Stellenwert der Temporalisbiopsie.

BACKGROUND: Giant-cell arteritis (GCA) requires immediate diagnosis and therapy. The University Hospital of Würzburg established the Centre for Giant-cell Arteritis (ZeRi) to improve interdisciplinary collaboration. AIM OF THE STUDY: Retrospective evaluation of five-year data to assess the clinical relevance of several diagnostic methods, including temporal artery biopsy. PATIENTS AND METHODS: Retrospective evaluation of 101 patients with suspected GCA who had undergone interdisciplinary examination and biopsy between 2017 and 2022. We analysed specificity and sensitivity in clinical symptoms, ESR, CRP, scalp MRI, temporal artery sonography, and temporal artery biopsy. RESULTS: GCA was diagnosed after completing diagnostic testing in 75 of 101 patients with suspected GCA. By definition, biopsy showed a positive predictive value of 100% and a specificity of 84.6%; however, negative predictive value was 51.2%. Sonography of the temporal artery and MRI showed a positive predictive value of more than 93% and sensitivity of 62.5% and 76.1%, respectively. Clinical symptoms showed the highest sensitivity at 92% with a specificity of 57.7%. ESR and CRP were significantly higher in patients with GCA than in patients without GCA, whereby CRP values showed higher predictive power than did ESR. CONCLUSIONS: Most GCA cases can be detected with a precise medical history as well as ESR and CRP assessment. Sonography and MRI on the scalp can usually confirm suspected GCA, only requiring temporal artery biopsy in exceptional cases.

Vessel wall MRI in giant cell arteritis: standardized protocol and scoring approach developed by an international working group.

OBJECTIVES: There are an increasing number of centers performing research on high-resolution vessel wall magnetic resonance imaging (VW-MRI) in giant cell arteritis (GCA). However, harmonized approaches to VW-MRI in GCA are lacking and are essential to performing multicentre studies. Using a data-driven, consensus-based approach, an international expert group developed a standardized MRI protocol and scoring system to advance multi-centered research in cranial GCA. METHODS: A targeted literature review of VW-MRI in cranial GCA was conducted. A working group comprised of radiologists, rheumatologists, and ophthalmologists with expertise in VW-MRI and GCA reviewed the results of the literature search, presented relevant data and images from their respective centers, and then reached consensus on recommendations related to key MRI structures, MRI sequences, scoring system, and other important considerations. RESULTS: A total of 21 relevant articles were identified and reviewed. Based on published literature, structures to be evaluated on MRI were categorized based on anatomic location (extradural cranial, intradural cranial, and orbits) and prioritization (core vs elective). Essential and elective sequences to comprehensively image cranial and orbital structures while minimizing scan time were determined along with scoring systems to grade contrast enhancement. CONCLUSION: This report describes a standardized approach to facilitate research of VW-MRI in cranial GCA that is the result of a multi-disciplinary, international collaboration of experts in VW-MRI and/or GCA.

MRVAS-introducing a standardized magnetic resonance scoring system for assessing the extent of inflammatory burden in giant cell arteritis.

OBJECTIVES: Our aim was to introduce a standardized system for assessing the extent of GCA on MRI, i.e. the Magnetic Resonance Vasculitis Activity Score (MRVAS). To obtain a comprehensive view, we used an extensive MRI protocol including cranial vessels and the aorta with its branches. To test reliability, MRI was assessed by four readers with different levels of experience. METHODS: A total of 80 patients with suspected GCA underwent MRI of the cranial arteries and the aorta and its branches (20 vessel segments). Every vessel was rated dichotomous [inflamed (coded as 1) or not (coded as 0)], providing a summed score of 0-20. Blinded readers [two experienced radiologists (ExR) and two inexperienced radiologists (InR)] applied the MRVAS on an individual vessel and an overall level (defined as the highest score of any of the individual vessel scores). To determine interrater agreement, Cohen's κ was calculated for pairwise comparison of each reader for individual vessel segments. Intraclass correlation coefficients (ICCs) were used for the MRVAS. RESULTS: Concordance rates were excellent for both subcohorts on an individual vessel-based (GCA: ICC 0.95; non-GCA: ICC 0.96) and overall MRVAS level (GCA: ICC 0.96; non-GCA: ICC 1.0). Interrater agreement yielded significant concordance (P < 0.001) for all pairs (κ range 0.78-0.98). No significant differences between ExRs and InRs were observed (P = 0.38). CONCLUSION: The proposed MRVAS allows standardized scoring of inflammation in GCA and achieved high agreement rates in a prospective setting.

High-resolution Magnetic Resonance Imaging visualizes intracranial large artery involvement in Giant Cell Arteritis.

OBJECTIVE: Giant Cell arteritis (GCA) is a large vessel vasculitis, typically involving the aorta and its branches with predilection for the scalp arteries. Intracranial involvement is still part of ongoing research. We assess inflammation of the intracranial arteries on 3D-black-blood magnetic resonance imaging (3D-CS-BB-MRI) in patients with GCA and age-matched controls. METHODS: 105 patients with 3D-CS-BB-MRI of the brain were included in this retrospective dual-center case-control study; 55 with diagnosed GCA and 50 age-matched controls. High-resolution 3D-CS-BB-MRI was performed on a 3 Tesla MR scanner with a post-contrast 3D-compressed-sensing (CS) MR pulse sequence, specifically a T1-weighted sampling perfection, application-optimized contrasts using different flip angle evolution (SPACE) pulse sequence with whole-brain coverage and isotropic resolution of 0.55 mm3. Two neuroradiologists blinded to clinical data independently scored the cerebral arteries qualitatively for inflammation; circumferential vessel wall thickening and contrast enhancement were scored positive for vasculitis. RESULTS: 8 of 55 GCA patients (14.5%) showed inflammation of at least one intracranial artery. The internal carotid artery (ICA) was affected in 6/55 (10.9%), the vertebral artery in 4/55 (7.3%) and the basilar artery and posterior cerebral artery in 1/55 (1.8%). All patients with inflammatory changes reported headaches and none showed any focal neurological deficit. Besides headache and general weakness, there was no significant correlation between inflammation of the intracranial arteries and clinical symptoms. No age-matched control patient showed inflammatory changes of the intracranial arteries. CONCLUSION: High-resolution 3D-CS-BB-MRI revealed inflammatory changes of intracranial arteries in 14.5% of GCA patients with the intradural ICA as the most frequently affected vessel.

PET-Derived Increased Inflammation in Large Vessels is linked to Relapse-Free Survival in Patients with Giant Cell Arteritis.

BACKGROUND: Despite anti-inflammatory treatment, patients with giant cell arteritis (GCA) experience relapse. We aimed to determine respective relapse predictors focusing on [18F]fluorodeoxyglucose ([18F]FDG)-PET-based parameters. MATERIAL AND METHODS: 21 therapy-naïve GCA patients received [18F]FDG-PET/CT. Patients were divided in two groups: those who relapsed during course of disease and those who did not. Median follow up was 15 months. [18F]FDG-PET/CT was analyzed for visual (PET vascular activity score [VAS]) and quantitative parameters, including Target-to-background-Ratio with liver (TBRliver) and jugular vein (TBRjv) serving as reference tissues. In addition, clinical parameters were tested. RESULTS: 8/21 (38.1 %) had relapse. Clinical parameters could not significantly discriminate between relapse vs no-relapse, including age (p = 0.9) or blood-based inflammatory markers (white blood cell counts [WBC] and c-reactive protein [CRP], p = 0.72, each). PETVAS score could also not differentiate between respective subgroups (p = 0.59). In a quantitative assessment, TBRjv demonstrated a trend towards significance (p = 0.28). TBRliver, however, separated between patients with and without relapse (p = 0.03). CONCLUSION: [18F]FDG PET quantification of vessels may be useful to identify GCA patients prone to relapse during follow-up.

Orbital magnetic resonance imaging of giant cell arteritis with ocular manifestations: a systematic review and individual participant data meta-analysis.

OBJECTIVES: We conducted a systematic review and individual participant data meta-analysis of publications reporting the ophthalmologic presentation, clinical exam, and orbital MRI findings in patients with giant cell arteritis and ocular manifestations. METHODS: PubMed and Cochrane databases were searched up to January 16, 2022. Publications reporting patient-level data on patients with ophthalmologic symptoms, imaged with orbital MRI, and diagnosed with biopsy-proven giant cell arteritis were included. Demographics, clinical symptoms, exam, lab, imaging, and outcomes data were extracted. The methodological quality and completeness of reporting of case reports were assessed. RESULTS: Thirty-two studies were included comprising 51 patients (females = 24; median age, 76 years). Vision loss (78%) and headache (45%) were commonly reported visual and cranial symptoms. Ophthalmologic presentation was unilateral (41%) or bilateral (59%). Fundus examination most commonly showed disc edema (64%) and pallor (49%). Average visual acuity was very poor (2.28 logMAR ± 2.18). Diagnoses included anterior (61%) and posterior (16%) ischemic optic neuropathy, central retinal artery occlusion (8%), and orbital infarction syndrome (2%). On MRI, enhancement of the optic nerve sheath (53%), intraconal fat (25%), and optic nerve/chiasm (14%) was most prevalent. Among patients with monocular visual symptoms, 38% showed pathologic enhancement in the asymptomatic orbit. Six of seven cases reported imaging resolution after treatment on follow-up MRIs. CONCLUSIONS: Vision loss, pallid disc edema, and optic nerve sheath enhancement are the most common clinical, fundoscopic, and imaging findings reported in patients diagnosed with giant cell arteritis with ocular manifestations, respectively. MRI may detect subclinical inflammation and ischemia in the asymptomatic eye and may be an adjunct diagnostic tool. CLINICAL RELEVANCE STATEMENT: Brain and orbital MRIs may have diagnostic and prognostic roles in patients with suspected giant cell arteritis who present with ophthalmic symptoms.

Intraorbital findings in giant cell arteritis on black blood MRI.

OBJECTIVE: Blindness is a feared complication of giant cell arteritis (GCA). However, the spectrum of pathologic orbital imaging findings on magnetic resonance imaging (MRI) in GCA is not well understood. In this study, we assess inflammatory changes of intraorbital structures on black blood MRI (BB-MRI) in patients with GCA compared to age-matched controls. METHODS: In this multicenter case-control study, 106 subjects underwent BB-MRI. Fifty-six patients with clinically or histologically diagnosed GCA and 50 age-matched controls without clinical or laboratory evidence of vasculitis were included. All individuals were imaged on a 3-T MR scanner with a post-contrast compressed-sensing (CS) T1-weighted sampling perfection with application-optimized contrasts using different flip angle evolution (SPACE) BB-MRI sequence. Imaging results were correlated with available clinical symptoms. RESULTS: Eighteen of 56 GCA patients (32%) showed inflammatory changes of at least one of the intraorbital structures. The most common finding was enhancement of at least one of the optic nerve sheaths (N = 13, 72%). Vessel wall enhancement of the ophthalmic artery was unilateral in 8 and bilateral in 3 patients. Enhancement of the optic nerve was observed in one patient. There was no significant correlation between imaging features of inflammation and clinically reported orbital symptoms (p = 0.10). None of the age-matched control patients showed any inflammatory changes of intraorbital structures. CONCLUSIONS: BB-MRI revealed inflammatory findings in the orbits in up to 32% of patients with GCA. Optic nerve sheath enhancement was the most common intraorbital inflammatory change on BB-MRI. MRI findings were independent of clinically reported orbital symptoms. KEY POINTS: • Up to 32% of GCA patients shows signs of inflammation of intraorbital structures on BB-MRI. • Enhancement of the optic nerve sheath is the most common intraorbital finding in GCA patients on BB-MRI. • Features of inflammation of intraorbital structures are independent of clinically reported symptoms.

Clinical Utility of C-Reactive Protein and White Blood Cell Count for Scheduling an [18F]FDG PET/CT in Patients with Giant Cell Arteritis.

OBJECTIVES: 2-deoxy-2-[18F]fluoro-D-glucose ([18F]FDG) PET/CT can be utilized in patients with giant cell arteritis (GCA), but pretest probability of established laboratory marker such as C-reactive protein (CRP) and white blood cell count (WBC) has not been defined yet. We aimed to elucidate the clinical utility of CRP and WBC for scheduling an [18F]FDG scan. METHODS: 18 treatment-naïve GCA patients and 14 GCA subjects with anti-inflammatory treatment (glucocorticoids or comparable drugs), who underwent [18F]FDG PET/CT and who had no other inflammatory disease at time of scan, were identified. A semi-quantitative analysis in 11 vessel segments was conducted, with averaged jugular vein, healthy liver and lung tissue (Target-to-background ratio [TBR]VJ/liver/lung) serving as background. Derived TBR were then correlated with CRP and WBC at time of PET using Spearman's correlation. RESULTS: For all treatment-naïve patients, TBRVJ was 2.3±1.1 (95%CI, 2.2-2.5), TBRliver was 1.0±0.5 (95%CI, 0.9-1.0) and average TBRlung was 6.3±3.6 (95%CI, 5.8-6.8). No significant correlation was noted for either CRP (TBRVJ: R=-0.19; TBRliver: R=-0.03; TBRlung: R=-0.17; each P ≥ 0.44) or for WBC (TBRVJ: R=-0.40; TBRliver: R=-0.32; TBRlung: R=-0.37; each P ≥ 0.10). Similar results were recorded for patients under treatment at time of PET. Again, no significant correlation was reached for either CRP (TBRVJ: R=-0.17; TBRliver: R=-0.28; TBRlung: R=-0.09; each P ≥ 0.32) or WBC (TBRVJ: R=-0.06; TBRliver: R=-0.13; TBRlung: R=0.06; each P ≥ 0.65). CONCLUSIONS: In GCA patients with and without anti-inflammatory treatment, CRP and WBC did not substantially correlate with TBR at time of scan. Given the rather limited pretest probability of those parameters, such laboratory values may have less diagnostic utility to order an [18F]FDG PET/CT.

Vasa vasorum of proximal cerebral arteries after dural crossing - potential imaging confounder in diagnosing intracranial vasculitis in elderly subjects on black-blood MRI.

OBJECTIVES: Vessel wall enhancement (VWE) may be commonly seen on MRI images of asymptomatic subjects. This study aimed to characterize the VWE of the proximal internal carotid (ICA) and vertebral arteries (VA) in a non-vasculitic elderly patient cohort. METHODS: Cranial MRI scans at 3 Tesla were performed in 43 patients (aged ≥ 50 years) with known malignancy for exclusion of cerebral metastases. For vessel wall imaging (VWI), a high-resolution compressed-sensing black-blood 3D T1-weighted fast (turbo) spin echo sequence (T1 CS-SPACE prototype) was applied post gadolinium with an isotropic resolution of 0.55 mm. Bilateral proximal intradural ICA and VA segments were evaluated for presence, morphology, and longitudinal extension of VWE. RESULTS: Concentric VWE of the proximal intradural ICA was found in 13 (30%) patients, and of the proximal intradural VA in 39 (91%) patients. Mean longitudinal extension of VWE after dural entry was 13 mm in the VA and 2 mm in the ICA. In 14 of 39 patients (36%) with proximal intradural VWE, morphology of VWE was suggestive of the mere presence of vasa vasorum. In 25 patients (64 %), morphology indicated atherosclerotic lesions in addition to vasa vasorum. CONCLUSIONS: Vasa vasorum may account for concentric VWE within the proximal 2 mm of the ICA and 13 mm of the VA after dural entry in elderly subjects. Concentric VWE in these locations should not be confused with large artery vasculitis. Distal to these segments, VWE may be more likely related to pathologic conditions such as vasculitis. KEY POINTS: • Vasa vasorum may account for concentric VWE within the proximal 2 mm of the ICA and 13 mm of the VA after dural entry in non-vasculitic elderly people. • Concentric enhancement within the proximal 2 mm of the intradural ICA and within the proximal 13 mm of the intradural VA portions should not be misinterpreted as vasculitis. • Distal of this, VWE is likely related to pathologic conditions, in case of concentric VWE suggestive of vasculitis.

Whole-Body [18F]FDG PET/CT Can Alter Diagnosis in Patients with Suspected Rheumatic Disease.

The 2-deoxy-d-[18F]fluoro-D-glucose (FDG) positron emission tomography/computed tomography (PET/CT) is widely utilized to assess the vascular and articular inflammatory burden of patients with a suspected diagnosis of rheumatic disease. We aimed to elucidate the impact of [18F]FDG PET/CT on change in initially suspected diagnosis in patients at the time of the scan. Thirty-four patients, who had undergone [18F]FDG PET/CT, were enrolled and the initially suspected diagnosis prior to [18F]FDG PET/CT was compared to the final diagnosis. In addition, a semi-quantitative analysis including vessel wall-to-liver (VLR) and joint-to-liver (JLR) ratios was also conducted. Prior to [18F]FDG PET/CT, 22/34 (64.7%) of patients did not have an established diagnosis, whereas in 7/34 (20.6%), polymyalgia rheumatica (PMR) was suspected, and in 5/34 (14.7%), giant cell arteritis (GCA) was suspected by the referring rheumatologists. After [18F]FDG PET/CT, the diagnosis was GCA in 19/34 (55.9%), combined GCA and PMR (GCA + PMR) in 9/34 (26.5%) and PMR in the remaining 6/34 (17.6%). As such, [18F]FDG PET/CT altered suspected diagnosis in 28/34 (82.4%), including in all unclear cases. VLR of patients whose final diagnosis was GCA tended to be significantly higher when compared to VLR in PMR (GCA, 1.01 ± 0.08 (95%CI, 0.95-1.1) vs. PMR, 0.92 ± 0.1 (95%CI, 0.85-0.99), p = 0.07), but not when compared to PMR + GCA (1.04 ± 0.14 (95%CI, 0.95-1.13), p = 1). JLR of individuals finally diagnosed with PMR (0.94 ± 0.16, (95%CI, 0.83-1.06)), however, was significantly increased relative to JLR in GCA (0.58 ± 0.04 (95%CI, 0.55-0.61)) and GCA + PMR (0.64 ± 0.09 (95%CI, 0.57-0.71); p < 0.0001, respectively). In individuals with a suspected diagnosis of rheumatic disease, an inflammatory-directed [18F]FDG PET/CT can alter diagnosis in the majority of the cases, particularly in subjects who were referred because of diagnostic uncertainty. Semi-quantitative assessment may be helpful in establishing a final diagnosis of PMR, supporting the notion that a quantitative whole-body read-out may be useful in unclear cases.

Intracranial vessel wall imaging framework - Data acquisition, processing, and visualization.

OBJECTIVE: Assessment of vessel walls is an integral part in diagnosis and disease monitoring of vascular diseases such as vasculitis. Vessel wall imaging (VWI), in particular of intracranial arteries, is the domain of Magnetic Resonance Imaging (MRI) - but still remains a challenge. The tortuous anatomy of intracranial arteries and the need for high resolution within clinically acceptable scan times require special technical conditions regarding the hardware and software environments. MATERIALS AND METHODS: In this work a dedicated framework for intracranial VWI is presented offering an optimized, black-blood 3D T1-weighted post-contrast Compressed Sensing (CS)-accelerated MRI sequence prototype combined with dedicated 3D-GUI supported post-processing tool for the CPR visualization of tortuous arbitrary vessel structures. RESULTS: Using CS accelerated MRI sequence, the scanning time for high-resolution 3D black-blood CS-space data could be reduced to under 10 min. These data are adequate for a further processing to extract straightened visualizations (curved planar reformats - CPR). First patient data sets could be acquired in clinical environment. CONCLUSION: A highly versatile framework for VWI visualization was demonstrated utilizing a post-processing tool to extract CPR reformats from high-resolution 3D black-blood CS-SPACE data, enabling simplified and optimized assessment of intracranial arteries in intracranial vascular disorders, especially in suspected intracranial vasculitis, by stretching their tortuous course. The processing time from about 15-20 min per patient (data acquisition and further processing) allows the integration into clinical routine.

High-resolution Compressed-sensing T1 Black-blood MRI : A New Multipurpose Sequence in Vascular Neuroimaging?

BACKGROUND AND PURPOSE: In vasculopathies of the central nervous system, reliable and timely diagnosis is important against the background of significant morbidity and sequelae in cases of incorrect diagnosis or delayed treatment. Magnetic resonance imaging (MRI) plays a major role in the detection and monitoring of intracranial and extracranial vascular pathologies of different etiologies, in particular for evaluation of the vessel wall in addition to luminal information, thus allowing differentiation between various vasculopathies. Compressed-sensing black-blood MRI combines high image quality with relatively short acquisition time and offers promising potential in the context of neurovascular vessel wall imaging in clinical routine. This case review gives an overview of its application in the diagnosis of various intracranial and extracranial entities. METHODS: An optimized high-resolution compressed-sensing black-blood 3D T1-weighted fast (turbo) spin echo technique (T1 CS-SPACE prototype) precontrast and postcontrast application at 3T was used for the evaluation of various vascular conditions in neuroradiology. RESULTS: In this article seven cases of intracranial and extracranial arterial and venous vasculopathies with representative imaging findings in high-resolution compressed-sensing black-blood MRI are presented. CONCLUSION: High-resolution 3D T1 CS-SPACE black-blood MRI is capable of imaging various vascular entities in high detail with whole head coverage and low susceptibility for motion artifacts and within acceptable scan times. It represents a highly versatile, non-invasive technique for the visualization and differentiation of a wide variety of neurovascular arterial and venous disorders.

Imaging in Vasculitis.

PURPOSE OF REVIEW: Vasculitides are characterized by mostly autoimmunologically induced inflammatory processes of vascular structures. They have various clinical and radiologic appearances. Early diagnosis and reliable monitoring are indispensable for adequate therapy to prevent potentially serious complications. Imaging, in addition to laboratory tests and physical examination, constitutes a key component in assessing disease extent and activity. This review presents current standards and some typical findings in the context of imaging in vasculitis with particular attention to large vessel vasculitides. RECENT FINDINGS: Recently, imaging has gained importance in the management of vasculitis, especially regarding large vessel vasculitides (LVV). Recently, EULAR (European League Against Rheumatism) has launched its recommendations concerning the diagnosis of LVVs. Imaging is recommended as the preferred complement to clinical examination. Color-coded duplex sonography is considered the first choice imaging test in suspected giant cell arteritis, and magnetic resonance imaging is considered the first choice in suspected Takayasu's arteritis. Due to diversity of clinical and radiologic presentations, diagnosis and therapy monitoring of vasculitides may constitute a challenge. As a result of ongoing technological progress, a variety of non-invasive imaging modalities now play an elemental role in the interdisciplinary management of vasculitic diseases.

Imaging in Large Vessel Vasculitides.

BACKGROUND: Large vessel vasculitides comprise primary vasculitides of large and medium-sized arteries with various clinical, laboratory and radiological presentations. Imaging has become increasingly important in the diagnosis and monitoring of large vessel vasculitides. It complements clinical and laboratory examination and displays vasculitic changes of large extra- and intracranial arteries with relatively good diagnostic reliability and a low level of invasiveness. METHOD: This review presents the most important imaging modalities and some typical imaging findings in the context of the two main forms of large vessel vasculitis, giant cell arteritis and Takayasu's arteritis, with special regard to the recently launched EULAR (The European League Against Rheumatism) recommendations on the role of imaging in patients with suspected large vessel vasculitides. RESULTS AND CONCLUSION: Color-coded duplex sonography (CCDS), magnetic resonance imaging (MRI), computed tomography (CT), and 18F-fluorodeoxyglucose positron emission tomography are today's common imaging methods in large vessel vasculitides representing a reasonable and less invasive alternative or at least a good complement to temporal artery biopsy. Today's EULAR guidelines recommend an imaging test as the first complementary method to clinical examination with CCDS as the preferred diagnostic test in suspected giant cell arteritis, MRI as the equivalent alternative in the case of inconclusive results, and MRI as the first choice in suspected Takayasu's arteritis. KEY POINTS: · Imaging is a noninvasive diagnostic test for diagnosing and monitoring large vessel vasculitides and is recommended nowadays as the first complementary method to clinical examination.. · Imaging is a reasonable alternative or at least a good complement to temporal artery biopsy in the case of suspected giant cell arteritis.. · Today's EULAR guidelines recommend CCDS as the preferred diagnostic test in suspected giant cell arteritis, with MRI as an equivalent alternative in the case of inconclusive results, and MRI as the first choice in suspected Takayasu's arteritis.. CITATION FORMAT: · Guggenberger K, Bley T. Imaging in Large Vessel Vasculitides. Fortschr Röntgenstr 2019; 191: 1083 - 1090.

Cerebral granulomatosis as a manifestation of Crohn's disease.

BACKGROUND: Crohn's disease (CD) is associated with a variety of extra-intestinal manifestations. Most commonly these involve the eye, skin, joints, coagulation system and liver. Cerebral manifestations of CD have been reported to a far lesser extent. The extensive detrimental impact of neurological symptoms on a patient's quality of life makes an early diagnosis and treatment particularly important. In previous case-reports, diagnosis of cerebral manifestations in CD often relied upon magnetic resonance imaging (MRI) and computed tomography (CT) alone. To our knowledge, only one case-report has documented a histologically confirmed case of cerebral lesions associated with CD so far. CASE PRESENTATION: A 39-year-old right-handed woman with a history of CD was referred to our hospital with etiologically unexplained Gadolinium (Gd)-enhancing cortical lesions, triggering epileptic seizures. A CT-scan of the thorax and bronchoalveolar lavage found no signs of sarcoidosis. Lumbar punctures and laboratory testing found no underlying infection or coincidental autoimmune disorders and MRI-scans showed progression of lesion load. Consequently, the patient underwent stereotactic biopsy of a cortical lesion. Histological examination revealed a mixed lympho-histiocytic and tuberculoid granulomatous inflammation surrounding small vessels and no signs for infection. After exclusion of other granulomatous diseases and the typical histological findings we diagnosed a cerebral granulomatosis as a manifestation of CD. The patient was initially started on azathioprine, which had to be switched to corticosteroids and methotrexate because of an azathioprine related pancreatitis. The patient has not suffered any further epileptic seizures to date. CONCLUSION: Cerebral manifestation of CD is a possibly underreported entity that may respond well to immunosuppressive treatment. In contrast to earlier reports of cerebral manifestations in CD, our patient showed no coincident gastrointestinal symptoms indicating an activity of CD during the progression of cortical lesion load, suggesting that similar to other extra-intestinal manifestations in CD, the activity of gastrointestinal symptoms does not necessarily reflect the activity of CD associated cerebral vasculitis. Therefore, diagnosis and therapy of cerebral manifestation may be delayed when focusing on gastrointestinal symptoms alone.

MR-spectroscopic imaging of glial tumors in the spotlight of the 2016 WHO classification.

BACKGROUND: The purpose of this study is to map spatial metabolite differences across three molecular subgroups of glial tumors, defined by the IDH1/2 mutation and 1p19q-co-deletion, using magnetic resonance spectroscopy. This work reports a new MR spectroscopy based classification algorithm by applying a radiomics analytics pipeline. MATERIALS: 65 patients received anatomical and chemical shift imaging (5 × 5 × 20 mm voxel size). Tumor regions were segmented and registered to corresponding spectroscopic voxels. Spectroscopic features were computed (n = 860) in a radiomic approach and selected by a classification algorithm. Finally, a random forest machine-learning model was trained to predict the molecular subtypes. RESULTS: A cluster analysis identified three robust spectroscopic clusters based on the mean silhouette widths. Molecular subgroups were significantly associated with the computed spectroscopic clusters (Fisher's Exact test p < 0.01). A machine-learning model was trained and validated by public available MRS data (n = 19). The analysis showed an accuracy rate in the Random Forest model by 93.8%. CONCLUSIONS: MR spectroscopy is a robust tool for predicting the molecular subtype in gliomas and adds important diagnostic information to the preoperative diagnostic work-up of glial tumor patients. MR-spectroscopy could improve radiological diagnostics in the future and potentially influence clinical and surgical decisions to improve individual tumor treatment.