Comparison of MRI, MRA, and DSA for Detection of Cerebral Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia.

BACKGROUND AND PURPOSE: Patients with hereditary hemorrhagic telangiectasia (HHT) have a high prevalence of brain vascular malformations, putting them at risk for brain hemorrhage and other complications. Our aim was to evaluate the relative utility of MR imaging and MRA compared with DSA in detecting cerebral AVMs in the HHT population. MATERIALS AND METHODS: Of 343 consecutive patients evaluated at the University of California, San Francisco HTT Center of Excellence, 63 met the study inclusion criteria: definite or probable hereditary hemorrhagic telangiectasia defined by meeting at least 2 Curacao criteria or positive genetic testing, as well as having at least 1 brain MR imaging and 1 DSA. MRIs were retrospectively reviewed, and the number of AVMs identified was compared with the number of AVMs identified on DSA. RESULTS: Of 63 patients, 45 (71%) had AVMs on DSA with a total of 92 AVMs identified. Of those, 24 (26%) were seen only on DSA; 68 (74%), on both DSA and MR imaging; and 5 additional lesions were seen only on MR imaging. Of the 92 lesions confirmed on DSA, 49 (53.3%) were seen on the 3D-T1 postgadolinium sequence, 52 (56.5%) were seen on the 2D-T1 postgadolinium sequence, 35 (38.0%) were seen on the SWI sequence, 24 (26.1%) were seen on T2 sequence, and 25 (27.2%) were seen on MRA. The sensitivity and specificity of MR imaging as a whole in detecting AVMs then confirmed on DSA were 80.0% and 94.4%, respectively, and the positive and negative predictive values were 97.3% and 65.4%, respectively. CONCLUSIONS: This study reinforces the use of MR imaging as a primary screening tool for cerebral AVMs in patients with hereditary hemorrhagic telangiectasia and suggests that 3D-T1 postgadolinium and 2D-T1 postgadolinium performed at 3T are the highest yield sequences.

High Prevalence of Spinal Cord Cavernous Malformations in the Familial Cerebral Cavernous Malformations Type 1 Cohort.

BACKGROUND AND PURPOSE: Cavernous malformations occur most often in the brain but can occur in the spinal cord. Small studies of patients with familial cerebral cavernous malformations suggested a prevalence of spinal cord cavernous malformations of 20%-42%. We aimed to review our familial cohort and prospectively estimate the prevalence of spinal cord cavernous malformations. MATERIALS AND METHODS: We initially reviewed our familial cerebral cavernous malformations cohort for spinal cord cavernous malformations and reviewed clinical spine MR imaging examinations for sequence sensitivity. We then prospectively performed research MR imaging of the spinal cord in 29 patients from the familial cohort to estimate the prevalence. RESULTS: Gradient-based sequences identified the most spinal cord cavernous malformations on clinical MR images, forming the basis for developing our screening MR imaging. Screening spinal cord MR imaging demonstrated a prevalence of 72.4%, and a positive correlation with patient age and number of cerebral cavernous malformations. CONCLUSIONS: Spinal cord cavernous malformations occur commonly in the familial cerebral cavernous malformation population. Gradient-based sequences are the most sensitive and should be used when spinal cord cavernous malformations are suspected. This study establishes the prevalence in the familial population at around 70% and supports the idea that this condition is a progressive systemic disease that affects the entire central nervous system.

Differentiation of brain tumor-related edema based on 3D T1rho imaging.

BACKGROUND AND PURPOSE: Cerebral edema associated with brain tumors is an important source of morbidity. Its type depends largely on the capillary ultra-structures of the histopathologic subtype of underlying brain tumor. The purpose of our study was to differentiate vasogenic edema associated with brain metastases and infiltrative edema related to diffuse gliomas using quantitative 3D T1 rho (T1ρ) imaging. MATERIALS AND METHODS: Preoperative MR examination including whole brain 3D T1ρ imaging was performed in 23 patients with newly diagnosed brain tumors (9 with metastasis, 8 with lower grade glioma, LGG, 6 with glioblastoma, GBM). Mean T1ρ values were measured in regions of peritumoral non-enhancing T2 signal hyperintensity, excluding both enhancing and necrotic or cystic component, and normal-appearing white matter. RESULTS: Mean T1ρ values were significantly elevated in the vasogenic edema surrounding intracranial metastases when compared to the infiltrative edema associated with either LGG or GBM (p=0.02 and <0.01, respectively). No significant difference was noted between T1ρ values of infiltrative edema between LGG and GBM (p=0.84 and 0.96, respectively). CONCLUSION: Our study demonstrates the feasibility and potential diagnostic role of T1ρ in the quantitative differentiation between edema related to intracranial metastases and gliomas and as a potentially complementary tool to standard MR techniques in further characterizing pathophysiology of vasogenic and infiltrative edema.

Imaging Characteristics of Pediatric Diffuse Midline Gliomas with Histone H3 K27M Mutation.

BACKGROUND AND PURPOSE: The 2016 World Health Organization Classification of Tumors of the Central Nervous System includes "diffuse midline glioma with histone H3 K27M mutation" as a new diagnostic entity. We describe the MR imaging characteristics of this new tumor entity in pediatric patients. MATERIALS AND METHODS: We retrospectively reviewed imaging features of pediatric patients with midline gliomas with or without the histone H3 K27 mutation. We evaluated the imaging features of these tumors on the basis of location, enhancement pattern, and necrosis. RESULTS: Among 33 patients with diffuse midline gliomas, histone H3 K27M mutation was present in 24 patients (72.7%) and absent in 9 (27.3%). Of the tumors, 27.3% (n = 9) were located in the thalamus; 42.4% (n = 14), in the pons; 15% (n = 5), within the vermis/fourth ventricle; and 6% (n = 2), in the spinal cord. The radiographic features of diffuse midline gliomas with histone H3 K27M mutation were highly variable, ranging from expansile masses without enhancement or necrosis with large areas of surrounding infiltrative growth to peripherally enhancing masses with central necrosis with significant mass effect but little surrounding T2/FLAIR hyperintensity. When we compared diffuse midline gliomas on the basis of the presence or absence of histone H3 K27M mutation, there was no significant correlation between enhancement or border characteristics, infiltrative appearance, or presence of edema. CONCLUSIONS: We describe, for the first time, the MR imaging features of pediatric diffuse midline gliomas with histone H3 K27M mutation. Similar to the heterogeneous histologic features among these tumors, they also have a diverse imaging appearance without distinguishing features from histone H3 wildtype diffuse gliomas.

Multivariate Analysis of MRI Biomarkers for Predicting Neurologic Impairment in Cervical Spinal Cord Injury.

BACKGROUND AND PURPOSE: Acute markers of spinal cord injury are essential for both diagnostic and prognostic purposes. The goal of this study was to assess the relationship between early MR imaging biomarkers after acute cervical spinal cord injury and to evaluate their predictive validity of neurologic impairment. MATERIALS AND METHODS: We performed a retrospective cohort study of 95 patients with acute spinal cord injury and preoperative MR imaging within 24 hours of injury. The American Spinal Injury Association Impairment Scale was used as our primary outcome measure to define neurologic impairment. We assessed several MR imaging features of injury, including axial grade (Brain and Spinal Injury Center score), sagittal grade, length of injury, maximum canal compromise, and maximum spinal cord compression. Data-driven nonlinear principal component analysis was followed by correlation and optimal-scaled multiple variable regression to predict neurologic impairment. RESULTS: Nonlinear principal component analysis identified 2 clusters of MR imaging variables related to 1) measures of intrinsic cord signal abnormality and 2) measures of extrinsic cord compression. Neurologic impairment was best accounted for by MR imaging measures of intrinsic cord signal abnormality, with axial grade representing the most accurate predictor of short-term impairment, even when correcting for surgical decompression and degree of cord compression. CONCLUSIONS: This study demonstrates the utility of applying nonlinear principal component analysis for defining the relationship between MR imaging biomarkers in a complex clinical syndrome of cervical spinal cord injury. Of the assessed imaging biomarkers, the intrinsic measures of cord signal abnormality were most predictive of neurologic impairment in acute spinal cord injury, highlighting the value of axial T2 MR imaging.

The Combined Performance of ADC, CSF CXC Chemokine Ligand 13, and CSF Interleukin 10 in the Diagnosis of Central Nervous System Lymphoma.

BACKGROUND AND PURPOSE: CXC chemokine ligand 13 and interleukin 10 have emerged as CSF biomarkers for the diagnosis of CNS lymphoma. Our hypothesis is that the combined use of ADC, CXC chemokine ligand 13, and interleukin 10 will result in increased diagnostic performance compared with the use of ADC values alone. MATERIALS AND METHODS: Eighty-seven patients were included in this study, including 43 with CNS lymphoma and 44 without CNS lymphoma (21 metastases, 14 high-grade gliomas, 9 tumefactive demyelinating lesions) who had undergone CSF proteomic analysis and had a new enhancing mass on brain MR imaging. Average ADC was derived by contouring the contrast-enhancing tumor volume. Group means were compared via t tests for average ADC, CXC chemokine ligand 13, and interleukin 10. Receiver operating characteristic analysis was performed for each individual variable. Multiple-variable logistic regression with receiver operating characteristic analysis was performed, and the multiple-variable receiver operating characteristic was compared with single-variable receiver operating characteristics. RESULTS: The average ADC was lower and CSF CXC chemokine ligand 13 and interleukin 10 values were higher in CNS lymphoma (P < .001). Areas under the curve ranged from 0.739 to 0.832 for single-variable ROC. Multiple-variable logistic regression yielded statistically significant individual effects for all 3 variables in a combined model. Multiple-variable receiver operating characteristics (area under the curve, 0.928) demonstrated statistically significantly superior diagnostic performance compared with the use of single variables alone. CONCLUSIONS: The combined use of ADC, CSF CXC chemokine ligand 13, and interleukin 10 results in increased diagnostic performance for the diagnosis of CNS lymphoma. This finding highlights the importance of CSF analysis when the diagnosis of CNS lymphoma is considered on the basis of MR imaging.

Direct Cranial Nerve Involvement by Gliomas: Case Series and Review of the Literature.

Malignant gliomas are characterized by infiltrative growth of tumor cells, including along white matter tracts. This may result in clinical cranial neuropathy due to direct involvement of a cranial nerve rather than by leptomeningeal spread along cranial nerves. Gliomas directly involving cranial nerves III-XII are rare, with only 11 cases reported in the literature before 2014, including 8 with imaging. We present 8 additional cases demonstrating direct infiltration of a cranial nerve by a glioma. Asymmetric cisternal nerve expansion compared with the contralateral nerve was noted with a mean length of involvement of 9.4 mm. Based on our case series, the key imaging feature for recognizing direct cranial nerve involvement by a glioma is the detection of an intra-axial mass in the pons or midbrain that is directly associated with expansion, signal abnormality, and/or enhancement of the adjacent cranial nerves.

Ex-PRESS glaucoma filter: an MRI compatible metallic orbital foreign body imaged at 1.5 and 3T.

AIM: To report on the MRI compatibility of the Ex-PRESS glaucoma filtration device, a tiny metallic implant placed into the anterior chamber of the eye that is much smaller than traditional glaucoma shunts, and to educate the radiology community regarding its appearance. MATERIALS AND METHODS: Seven patients with Ex-PRESS glaucoma filtration devices were identified that had undergone MRI at San Francisco General Hospital/University of California San Francisco Medical Center by searching and cross-referencing the radiology reporting system and the electronic medical record. MRI images were reviewed for artefact interfering with interpretation. Ophthalmology examinations were reviewed for evidence of complications. RESULTS: Eighteen individual MRI examinations were performed during 12 unique MRI events on these 7 patients. 13/18 individual MRI examinations and 7/12 MRI events were performed at 3 T with the others performed at 1.5 T. Mean time from Ex-PRESS implantation to MRI was 17.5 months. Mean time from MRI to first ophthalmology examination was 1.1 months and from MRI to latest ophthalmology examination was 6.6 months. Susceptibility artefact did not interfere with image interpretation and no complications related to MRI were encountered. CONCLUSION: The Ex-PRESS glaucoma filtration device appears to be safe for MRI at 1.5 and 3 T and does not produce significant susceptibility artefact to affect diagnostic interpretation adversely.