Cerebral perfusion using ASL in patients with COVID-19 and neurological manifestations: A retrospective multicenter observational study.

BACKGROUND AND PURPOSE: Cerebral hypoperfusion has been reported in patients with COVID-19 and neurological manifestations in small cohorts. We aimed to systematically assess changes in cerebral perfusion in a cohort of 59 of these patients, with or without abnormalities on morphological MRI sequences. METHODS: Patients with biologically-confirmed COVID-19 and neurological manifestations undergoing a brain MRI with technically adequate arterial spin labeling (ASL) perfusion were included in this retrospective multicenter study. ASL maps were jointly reviewed by two readers blinded to clinical data. They assessed abnormal perfusion in four regions of interest in each brain hemisphere: frontal lobe, parietal lobe, posterior temporal lobe, and temporal pole extended to the amygdalo-hippocampal complex. RESULTS: Fifty-nine patients (44 men (75%), mean age 61.2 years) were included. Most patients had a severe COVID-19, 57 (97%) needed oxygen therapy and 43 (73%) were hospitalized in intensive care unit at the time of MRI. Morphological brain MRI was abnormal in 44 (75%) patients. ASL perfusion was abnormal in 53 (90%) patients, and particularly in all patients with normal morphological MRI. Hypoperfusion occurred in 48 (81%) patients, mostly in temporal poles (52 (44%)) and frontal lobes (40 (34%)). Hyperperfusion occurred in 9 (15%) patients and was closely associated with post-contrast FLAIR leptomeningeal enhancement (100% [66.4%-100%] of hyperperfusion with enhancement versus 28.6% [16.6%-43.2%] without, p = 0.002). Studied clinical parameters (especially sedation) and other morphological MRI anomalies had no significant impact on perfusion anomalies. CONCLUSION: Brain ASL perfusion showed hypoperfusion in more than 80% of patients with severe COVID-19, with or without visible lesion on conventional MRI abnormalities.

Delayed [ 18 F]-FDG PET Imaging Increases Diagnostic Performance and Reproducibility to Differentiate Recurrence of Brain Metastases From Radionecrosis.

PURPOSE: Differentiating brain metastasis recurrence from radiation necrosis can be challenging during MRI follow-up after stereotactic radiotherapy. [ 18 F]-FDG is the most available PET tracer, but standard images performed 30 to 60 minutes postinjection provide insufficient accuracy. We compared the diagnostic performance and interobserver agreement of [ 18 F]-FDG PET with delayed images (4-5 hours postinjection) with the ones provided by standard and dual-time-point imaging. METHODS: Consecutive patients referred for brain [ 18 F]-FDG PET after inconclusive MRI were retrospectively included between 2015 and 2020 in 3 centers. Two independent nuclear medicine physicians interpreted standard (visually), delayed (visually), and dual-time-point (semiquantitatively) images, respectively. Adjudication was applied in case of discrepancy. The final diagnosis was confirmed histologically or after 6 months of MRI follow-up. Areas under the receiver operating characteristic curves were pairwise compared. RESULTS: Forty-eight lesions from 46 patients were analyzed. Primary tumors were mostly located in the lungs (57%) and breast (23%). The median delay between radiotherapy and PET was 15.7 months. The final diagnosis was tumor recurrence in 24 of 48 lesions (50%), with histological confirmation in 19 of 48 lesions (40%). Delayed images provided a larger area under the receiver operating characteristic curve (0.88; 95% confidence interval [CI], 0.75-0.95) than both standard (0.69; 95% CI, 0.54-0.81; P = 0.0014) and dual-time-point imaging (0.77; 95% CI, 0.63-0.88; P = 0.045), respectively. Interobserver agreement was almost perfect with delayed images ( κ = 0.83), whereas it was moderate with both standard ( κ = 0.48) and dual-time-point images ( κ = 0.61). CONCLUSIONS: [ 18 F]-FDG PET with delayed images is an accurate and reliable alternative to differentiate metastasis recurrence from radiation necrosis in case of inconclusive MRI after brain stereotactic radiotherapy.

Intracavernous Schwannoma Characterized With 18F-FDG, 68Ga-DOTATOC, and 18F-Choline PET.

ABSTRACT: We report the case of a 75-year-old man with history of prostate cancer whose left intracavernous lesion was successfully characterized by 3 PETs performed successively with different tracers. This poorly characterized tumor was initially discovered on an MRI conducted to investigate an acute diplopia and slowly growing during follow-up. On 18F-FDG PET, the lesion showed no significant uptake, and no extracranial lesion was found nor did it have increased 68Ga-DOTATOC uptake. Finally, this tumor displayed a high 18F-choline uptake, and no extracranial lesion was revealed with this tracer. The diagnosis of schwannoma without malignancy criterion was proven by biopsy.

Combining 18F-DOPA PET and MRI with perfusion-weighted imaging improves delineation of high-grade subregions in enhancing and non-enhancing gliomas prior treatment: a biopsy-controlled study.

PURPOSE: We aimed to compare spatial extent of high-grade subregions detected with combined [18F]-dihydroxyphenylalanine (18F-DOPA) PET and MRI to the one provided by advanced multimodal MRI alone including Contrast-enhanced (CE) and Perfusion weighted imaging (PWI). Then, we compared the accuracy between imaging modalities, in a per biopsy analysis. METHODS: Participants with suspected diffuse glioma were prospectively included between June 2018 and September 2019. Volumes of high-grade subregions were delineated respectively on 18F-DOPA PET and MRI (CE and PWI). Up to three per-surgical neuronavigation-guided biopsies were performed per patient. RESULTS: Thirty-eight biopsy samples from sixteen participants were analyzed. Six participants (38%) had grade IV IDH wild-type glioblastoma, six (38%) had grade III IDH-mutated astrocytoma and four (24%) had grade II IDH-mutated gliomas. Three patients had intratumoral heterogeneity with coexisting high- and low-grade tumor subregions. High-grade volumes determined with combined 18F-DOPA PET/MRI (median of 1.7 [interquartile range (IQR) 0.0, 19.1] mL) were larger than with multimodal MRI alone (median 1.3 [IQR 0.0, 12.8] mL) with low overlap (median Dice's coefficient 0.24 [IQR 0.08, 0.59]). Delineation volumes were substantially increased in five (31%) patients. In a per biopsy analysis, combined 18F-DOPA PET/MRI detected high-grade subregions with an accuracy of 58% compared to 42% (p = 0.03) with CE MRI alone and 50% (p = 0.25) using multimodal MRI (CE + PWI). CONCLUSIONS: The addition of 18F-DOPA PET to multimodal MRI (CE and PWI) enlarged the delineation volumes and enhanced overall accuracy for detection of high-grade subregions. Thus, combining 18F-DOPA with advanced MRI may improve treatment planning in newly diagnosed gliomas.

Neurologic and neuroimaging findings in patients with COVID-19: A retrospective multicenter study.

OBJECTIVE: To describe neuroimaging findings and to report the epidemiologic and clinical characteristics of patients with coronavirus disease 2019 (COVID-19) with neurologic manifestations. METHODS: In this retrospective multicenter study (11 hospitals), we included 64 patients with confirmed COVID-19 with neurologic manifestations who underwent a brain MRI. RESULTS: The cohort included 43 men (67%) and 21 women (33%); their median age was 66 (range 20-92) years. Thirty-six (56%) brain MRIs were considered abnormal, possibly related to severe acute respiratory syndrome coronavirus. Ischemic strokes (27%), leptomeningeal enhancement (17%), and encephalitis (13%) were the most frequent neuroimaging findings. Confusion (53%) was the most common neurologic manifestation, followed by impaired consciousness (39%), presence of clinical signs of corticospinal tract involvement (31%), agitation (31%), and headache (16%). The profile of patients experiencing ischemic stroke was different from that of other patients with abnormal brain imaging: the former less frequently had acute respiratory distress syndrome (p = 0.006) and more frequently had corticospinal tract signs (p = 0.02). Patients with encephalitis were younger (p = 0.007), whereas agitation was more frequent for patients with leptomeningeal enhancement (p = 0.009). CONCLUSIONS: Patients with COVID-19 may develop a wide range of neurologic symptoms, which can be associated with severe and fatal complications such as ischemic stroke or encephalitis. In terms of meningoencephalitis involvement, even if a direct effect of the virus cannot be excluded, the pathophysiology seems to involve an immune or inflammatory process given the presence of signs of inflammation in both CSF and neuroimaging but the lack of virus in CSF. CLINICALTRIALSGOV IDENTIFIER: NCT04368390.

Brain MRI Findings in Severe COVID-19: A Retrospective Observational Study.

Background Brain MRI parenchymal signal abnormalities have been associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Purpose To describe the neuroimaging findings (excluding ischemic infarcts) in patients with severe coronavirus disease 2019 (COVID-19) infection. Materials and Methods This was a retrospective study of patients evaluated from March 23, 2020, to April 27, 2020, at 16 hospitals. Inclusion criteria were (a) positive nasopharyngeal or lower respiratory tract reverse transcriptase polymerase chain reaction assays, (b) severe COVID-19 infection defined as a requirement for hospitalization and oxygen therapy, (c) neurologic manifestations, and (d) abnormal brain MRI findings. Exclusion criteria were patients with missing or noncontributory data regarding brain MRI or brain MRI showing ischemic infarcts, cerebral venous thrombosis, or chronic lesions unrelated to the current event. Categorical data were compared using the Fisher exact test. Quantitative data were compared using the Student t test or Wilcoxon test. P < .05 represented a significant difference. Results Thirty men (81%) and seven women (19%) met the inclusion criteria, with a mean age of 61 years ± 12 (standard deviation) (age range, 8-78 years). The most common neurologic manifestations were alteration of consciousness (27 of 37, 73%), abnormal wakefulness when sedation was stopped (15 of 37, 41%), confusion (12 of 37, 32%), and agitation (seven of 37, 19%). The most frequent MRI findings were signal abnormalities located in the medial temporal lobe in 16 of 37 patients (43%; 95% confidence interval [CI]: 27%, 59%), nonconfluent multifocal white matter hyperintense lesions seen with fluid-attenuated inversion recovery and diffusion-weighted sequences with variable enhancement, with associated hemorrhagic lesions in 11 of 37 patients (30%; 95% CI: 15%, 45%), and extensive and isolated white matter microhemorrhages in nine of 37 patients (24%; 95% CI: 10%, 38%). A majority of patients (20 of 37, 54%) had intracerebral hemorrhagic lesions with a more severe clinical presentation and a higher admission rate in intensive care units (20 of 20 patients [100%] vs 12 of 17 patients without hemorrhage [71%], P = .01) and development of the acute respiratory distress syndrome (20 of 20 patients [100%] vs 11 of 17 patients [65%], P = .005). Only one patient had SARS-CoV-2 RNA in the cerebrospinal fluid. Conclusion Patients with severe coronavirus disease 2019 and without ischemic infarcts had a wide range of neurologic manifestations that were associated with abnormal brain MRI scans. Eight distinctive neuroradiologic patterns were described. © RSNA, 2020.

Pathologic and MRI analysis in acute atypical inflammatory demyelinating lesions.

BACKGROUND: The diagnosis of atypical inflammatory demyelinating lesions can be difficult. Brain biopsy is often required to exclude neoplasms. Moreover, the relationship between these lesions and multiple sclerosis and NMOSD is not clear. OBJECTIVES: Our objectives were to describe radiological and pathological characteristics of patients with acute inflammatory demyelinating lesions. METHODS: We retrospectively identified patients with brain biopsy performed for diagnostic uncertainty revealing a demyelinating lesion. A complete clinical, biological, radiological and pathological analysis was performed. RESULTS: Twenty patients (15 with a single lesion) were included. MRI disclosed a wide range of lesions including infiltrative lesions (40%), ring-like lesion (15%) Baló-like lesion (15%) and acute haemorrhagic leukoencephalitis (20%). In spite of a marked heterogeneity, some findings were common: a peripheral B1000 hyperintense rim (70%), a slight oedema with mild mass effect (75%) and an open-rim peripheral enhancement (75%). Histopathology revealed that all cases featured macrophages distributed throughout, extensive demyelination, axonal preservation and absence of haemorrhagic changes. In the majority of cases, macrophages were the predominant inflammatory infiltrate and astrocytes were reactive and dystrophic. Aquaporin-4 staining was systematically preserved. After a mean follow-up of 5 years (1-12), 16/20 patients had a diagnosis of monophasic acute atypical inflammatory demyelinating lesion. One patient was diagnosed with MS and 3 with AQP4 negative NMOSD. DISCUSSION: Although imaging findings in patients with atypical inflammatory demyelinating lesions are heterogeneous, some common features such as peripheral DWI hyperintense rim with open-rim enhancement and absence of oedema argue in favour of a demyelinating lesion and should preclude a brain biopsy. In this context, AQP4 staining is systematically preserved and argues against an AQP4-positive NMOSD. Moreover, long-term follow-up is characterized by low recurrence rate.

CT angiography for one-year follow-up of intracranial aneurysms treated with the WEB device: Utility in evaluating aneurysm occlusion and WEB compression at one year.

BACKGROUND AND PURPOSE: The WEB is an innovative flow disruption device for cerebral aneurysm embolization with rapidly expanding indications. Our purpose was to evaluate the diagnostic performance of computed tomography angiography (CTA) at 1-year follow-up of aneurysms treated with the WEB. MATERIALS AND METHODS: Between April 2014 and May 2016, the study prospectively included patients treated with the WEB at our institution, and followed up within 24hours by CTA and at 1year by CTA, time-of-flight magnetic resonance angiography (TOF MRA) and digital subtraction angiography (DSA). The diagnostic quality of imaging data was assessed based on the confidence index, artifacts, and WEB shape depiction. The imaging diagnostic performance was assessed using 3 criteria at 1year: aneurysm occlusion status and worsening, and WEB shape compression. Interobserver and intermodality agreement was determined by calculating κ values. RESULTS: The study ultimately included 16 patients (9 women, mean age 53±7.6years). CTA quality confidence was scored as 2/2, artifacts 0.4/2 and WEB shape depiction 1.9/2, superior to TOF MRA for the latter two criteria. Aneurysm occlusion was adequate in 93.7% of patients, with CTA showing excellent interobserver reproducibility and agreement with DSA on a 4-grade scale (κ=1.00), while TOF MRA yielded good reproducibility (κ=0.76) and agreement with DSA (κ=0.69). CTA also identified aneurysm occlusion worsening (43.7%) and WEB compression (81.2%) in excellent agreement with DSA (κ=0.85 and 1.00). CONCLUSIONS: CTA is a reproducible and reliable technique for the follow-up of aneurysms treated with the WEB device.

Acute Neurologic Disorder from an Inhibitor of Fatty Acid Amide Hydrolase.

BACKGROUND: A decrease in fatty acid amide hydrolase (FAAH) activity increases the levels of endogenous analogues of cannabinoids, or endocannabinoids. FAAH inhibitors have shown analgesic and antiinflammatory activity in animal models, and some have been tested in phase 1 and 2 studies. In a phase 1 study, BIA 10-2474, an orally administered reversible FAAH inhibitor, was given to healthy volunteers to assess safety. METHODS: Single doses (0.25 to 100 mg) and repeated oral doses (2.5 to 20 mg for 10 days) of BIA 10-2474 had been administered to 84 healthy volunteers in sequential cohorts; no severe adverse events had been reported. Another cohort of participants was then assigned to placebo (2 participants) or 50 mg of BIA 10-2474 per day (6 participants). This report focuses on neurologic adverse events in participants in this final cohort. A total of 4 of the 6 participants who received active treatment consented to have their clinical and radiologic data included in this report. RESULTS: An acute and rapidly progressive neurologic syndrome developed in three of the four participants starting on the fifth day of drug administration. The main clinical features were headache, a cerebellar syndrome, memory impairment, and altered consciousness. Magnetic resonance imaging showed bilateral and symmetric cerebral lesions, including microhemorrhages and hyperintensities on fluid-attenuated inversion recovery and diffusion-weighted imaging sequences predominantly involving the pons and hippocampi. One patient became brain dead; the condition of two patients subsequently improved, but one patient had residual memory impairment, and the other patient had a residual cerebellar syndrome. One patient remained asymptomatic. CONCLUSIONS: An unanticipated severe neurologic disorder occurred after ingestion of BIA 10-2474 at the highest dose level used in a phase 1 trial. The underlying mechanism of this toxic cerebral syndrome remains unknown.

Diagnostic Value of Fractal Analysis for the Differentiation of Brain Tumors Using 3-Tesla Magnetic Resonance Susceptibility-Weighted Imaging.

BACKGROUND: Susceptibility-weighted imaging (SWI) of brain tumors provides information about neoplastic vasculature and intratumoral micro- and macrobleedings. Low- and high-grade gliomas can be distinguished by SWI due to their different vascular characteristics. Fractal analysis allows for quantification of these radiological differences by a computer-based morphological assessment of SWI patterns. OBJECTIVE: To show the feasibility of SWI analysis on 3-T magnetic resonance imaging to distinguish different kinds of brain tumors. METHODS: Seventy-eight patients affected by brain tumors of different histopathology (low- and high-grade gliomas, metastases, meningiomas, lymphomas) were included. All patients underwent preoperative 3-T magnetic resonance imaging including SWI, on which the lesions were contoured. The images underwent automated computation, extracting 2 quantitative parameters: the volume fraction of SWI signals within the tumors (signal ratio) and the morphological self-similar features (fractal dimension [FD]). The results were then correlated with each histopathological type of tumor. RESULTS: Signal ratio and FD were able to differentiate low-grade gliomas from grade III and IV gliomas, metastases, and meningiomas (P < .05). FD was statistically different between lymphomas and high-grade gliomas (P < .05). A receiver-operating characteristic analysis showed that the optimal cutoff value for differentiating low- from high-grade gliomas was 1.75 for FD (sensitivity, 81%; specificity, 89%) and 0.03 for signal ratio (sensitivity, 80%; specificity, 86%). CONCLUSION: FD of SWI on 3-T magnetic resonance imaging is a novel image biomarker for glioma grading and brain tumor characterization. Computational models offer promising results that may improve diagnosis and open perspectives in the radiological assessment of brain tumors. ABBREVIATIONS: FD, fractal dimensionSR, signal ratioSWI, susceptibility-weighted imaging.

Supra-aortic arteries: three-dimensional time-resolved k-t BLAST contrast-enhanced MRA using a nondedicated body coil at 3 tesla in acute ischemic stroke.

PURPOSE: To assess the image quality and diagnostic performance achieved by using supra-aortic 3D-TR-CE-k-t BLAST MRA and a nondedicated body coil as compared with conventional CE-MRA in patients with acute ischemic stroke. MATERIALS AND METHODS: In this prospective study, 36 consecutive patients with a suspected acute ischemic stroke underwent both k-t BLAST MRA and conventional CE-MRA. Image quality was assessed using visual and quantitative criteria and the techniques were compared. Both techniques were compared for degree of visual and quantitative measurement of carotid stenosis. RESULTS: Delineation of vessel lumen and overall diagnostic confidence were significantly better with CE-MRA, respectively 3.4 ± 0.5 and 3.3 ± 0.6 (mean score ± SD), than with k-t BLAST MRA, respectively 2.8 ± 0.4 and 2.9 ± 0.5 (P < 0.02). SNR and CNR were significantly higher for k-t BLAST MRA, respectively 33.5 ± 19.3 and 27.9 ± 19.3, than for CE-MRA, respectively 25.7 ± 10 and 20.4 ± 8.4 (P < 0.03). Intertechnique agreement was good for carotid stenosis characterization (κ = .763). For the 14 relevant stenosis, stenosis measurements were highly correlated between techniques (0.96; P < 0.0001). The Bland-Altman plot showed a low bias in assessment of the degree of stenosis (mean bias 2.1% ± 7.7). CONCLUSION: k-t BLAST MRA using a nondedicated coil offering and dynamic information was a effective diagnostic tool for detection and characterization of carotid stenosis.

Improving detection of ischemic lesions at 3 Tesla with optimized diffusion-weighted magnetic resonance imaging.

BACKGROUND AND PURPOSE: This study compared three different combinations of DWI parameters: three and six diffusion imaging directions (3dir and 6dir, respectively) using b=1000 or 2000 s/mm(2) (b1000 or b2000, respectively) to improve detection of recent ischemic lesions at 3 Tesla (3 T). MATERIALS AND METHODS: A total of 47 consecutive patients underwent three DWI scans: 3dir b1000; 6dir b1000; and 6dir b2000. Qualitative visual analysis was performed by three readers based on evaluation of the number of lesions, presence of artifacts and diagnostic confidence. Interobserver agreement, sensitivity, specificity, and positive and negative predictive values were calculated. RESULTS: Forty-five lesions were detected by 3dir b1000, 52 by 6dir b1000 and 56 by 6dir b2000 in 30 patients. The additional lesions identified by 6dir b2000 were either small or located in the posterior fossa. Sensitivity with 6dir b2000 was significantly higher than with 3dir b1000 (98.1% vs 77.4%; P<0.05). CONCLUSION: At 3 T, 6dir b2000 DWI detected more acute ischemic lesions than 3dir b1000, particularly small lesions and those located in the brain stem.

Sporadic intracranial haemangioblastomas: surgical outcome in a single institution series.

BACKGROUND: Haemangioblastomas are benign vascular tumours that may appear sporadically or in von Hippel-Lindau disease. Despite their higher incidence, sporadic haemangioblastomas have been less studied than syndromic ones. In this article, we evaluate the specific features, outcome and quality of life of patients with intracranial sporadic haemangioblastomas (ISHs) operated on in our institution. METHODS: Between 1998 and 2010, 38 patients harbouring 38 ISHs were operated on in our department. Their clinical, biological, radiological and surgical features were retrospectively reviewed. All patients were contacted for a quality-of-life (QOL) survey assessed by the Short Form 36 questionnaire (SF36). The mean duration of follow-up was 40 months (13-108 months). RESULTS: ISH represented 0.9 % of primary intracranial neoplasms treated in our centre during this period. Patients comprised 23 men and 15 women with a mean age of 47 years. None had polycythaemia. Cerebellar locations accounted for 79 % of ISHs, and brainstem ISH with involvement of the floor of the fourth ventricle represented 11 % of ISHs. At last follow-up, two patients harbouring solid medulla oblongata haemangioblastoma had died following severe bulbar syndrome and five patients had died of unrelated causes. One patient had multiple surgeries for three recurrences. Tumoral control was achieved in all cases at last follow-up. Results of the SF-36 questionnaire were as follows: median physical functioning score 100 (range 0-100), median physical problems score 100 (range 0-100), median bodily pain score 100 (range 45-100), median social functioning score 100 (range 25-100), median general mental health score 84 (range 40-92), median emotional problems score 100 (range 0-100), median vitality score 70 (range 35-80) and median general health perceptions score 70 (range 35-100). Mean QOL scores were similar to the general healthy population. CONCLUSION: Surgery of ISH provides good QOL and tumoral control except for those located in the medulla oblongata. We recommend considering a careful multimodal therapeutic approach, including radiosurgery for these specific locations.

Anatomical variations of the anterior cerebral arterial circle visualized by multidetector computed tomography angiography: comparison with 3D rotational angiography.

OBJECTIVES: Multidetector computed tomography angiography (MD-CTA) has become the first-line screening technique for patients with subarachnoid hemorrhage not only for detecting aneurysms, but also for providing decisive angioarchitectural information. The anterior cerebral arterial circle (ACAC) is the most common location for anatomical variations and aneurysms. The aim of this study was to assess the diagnostic performance of 64-section MD-CTA in the detection and characterization of anatomical variations of the ACAC compared with three-dimensional rotational angiography (3DRA). MATERIAL AND METHODS: In 104 patients, MD-CTA and 3DRA images of the internal carotid arteries were independently reviewed by two radiologists for variations, focusing on four arterial segments of the ACAC: the anterior communicating artery (ACoA); the A1 segments; the A2-A4 complexes; and the M1 segments. The percentages of variations detected by MD-CTA and 3DRA were compared using the chi-square test. Characterizations of the variations by MD-CTA compared with 3DRA were evaluated using the kappa statistic. RESULTS: A total of 114 variations in 624 segments (18.3%) were detected by MD-CTA compared with 90 variations in 453 segments (19.9%) by 3DRA. The difference was not significant (P=0.56). In 453 selected segments analyzed with both techniques, 15 discordances in characterization were noted, mostly in the ACoA (10/15). However, the overall intertechnical κ was excellent. Sensitivity, specificity, positive predictive values and negative predictive values were all greater than 90%. CONCLUSION: The overall diagnostic performance of MD-CTA in detecting anatomical variations of the ACAC was excellent compared with 3DRA. However, its lower spatial resolution led to misclassifications, especially in the ACoA.

Primary gliomatosis cerebri involving gray matter in pediatrics: a distinct entity? A multicenter study of 14 cases.

BACKGROUND AND PURPOSE: Gliomatosis cerebri (GC) is a rare neoplasm including a variety of tumors, with extremely variable evolution and heterogeneity of prognosis. It may appear either de novo or after a focal glioma, involve predominantly the white or the gray matter, and concern either pediatric or adult patients. We focused on primary GC involving exclusively gray matter in a pediatric population in order better to define the presentation and outcome of this disease. PATIENTS AND METHODS: We reviewed the databases of seven Departments of Pediatric Oncology to identify pediatric cases of GC between 1990 and 2007. Patients were included if they demonstrated a diffuse infiltrative process involving gray matter in magnetic resonance imaging (MRI) and histological tissue analyses, confirming a proliferative glial disorder. RESULTS: Fourteen patients with a median age of 8 years were identified. Epilepsy was the main presenting symptom. Brain MRI showed a lesion of the temporal and insular cerebral cortex associated with tumoral infiltration of the thalami and the basal ganglia. Histological examination confirmed the diagnosis of high-grade glioma. Prognosis was always very gloomy in the short term, with a median survival of less than a year. CONCLUSION: This rare entity, whose prognosis is appalling whatever the treatment proposed, should be clearly identified within the heterogeneous group of GC in the same way as diffuse intrinsic pontine gliomas have been identified among brain stem tumors. Systematic biopsies appear essential to permit the molecular studies which will assist in guiding the choice of future targeted treatments.

Time-of-flight MR angiography at 3T versus digital subtraction angiography in the imaging follow-up of 51 intracranial aneurysms treated with coils.

OBJECTIVE: To compare 3D time-of-flight MR angiography (TOF-MRA) at 3 Tesla (3T) with digital subtraction angiography (DSA) for the evaluation of intracranial aneurysm occlusion after endovascular coiling. METHODS: In a prospective study, 51 consecutive patients (25 females, 26 males; median age, 51 years) with 51 saccular aneurysms treated with endovascular coiling underwent simultaneous DSA and 3T TOF-MRA at follow-up. DSA and TOF-MRA images were analyzed independently by two senior neuroradiologists. Findings were assigned to 1 of 3 categories in the Raymond classification: complete obliteration, residual neck or residual aneurysm. Agreement between observers and techniques was evaluated using kappa statistics. RESULTS: DSA images were not interpretable for one patient. Interobserver agreement was determined as excellent for DSA (kappa=0.86) and TOF-MRA (kappa=0.80). After reaching a consensus, DSA follow-up showed 26 (51%) complete obliterations, 20 (39%) residual necks and 4 (8%) residual aneurysms. TOF-MRA showed 23 (45%) complete obliterations, 22 (43%) residual necks and 6 (12%) residual aneurysms. Comparison between TOF-MRA and DSA showed excellent agreement between the techniques (kappa=0.86). In the four cases that were misclassified, TOF-MRA findings were assigned to a higher class than for DSA. CONCLUSION: TOF-MRA at 3T is at least as efficient as DSA for the evaluation of intracranial aneurysm occlusion after endovascular treatment with detachable coils. We suggest that TOF-MRA at 3T might be used as the primary method for imaging follow-up of coiled intracranial aneurysms.

Endoscopic third ventriculostomy for the treatment of obstructive hydrocephalus during pregnancy.

BACKGROUND: Endoscopic third ventriculostomy is an effective procedure for treating obstructive (noncommunicating) hydrocephalus as an alternative to ventricular shunt placement. CASE: Five pregnant women undergoing endoscopic third ventriculostomy as treatment for newly diagnosed acute obstructive hydrocephalus (n=2) or in cases of malfunction of a preexisting ventriculoperitoneal shunt (n=3) are presented. CONCLUSION: Endoscopic third ventriculostomy may be preferred to ventricular shunt placement as the initial mode of treatment for pregnant patients with newly diagnosed obstructive hydrocephalus as well as in cases of malfunction of a preexisting shunt.

Proposal of a scoring scale as a survival predictor in intracranial oligodendrogliomas.

INTRODUCTION: Histological, clinical and radiological features, and molecular genetic analysis are among the factors that have been considered in defining the prognosis of oligodendrogliomas (OD), but they have yielded conflicting results. The purpose of this study was to test out a scoring scale based on clinical, radiological, pathological and molecular features. MATERIAL AND METHOD: To identify factors with prognostic significance, we analyzed 87 treated patients with a histological diagnosis of OD. Of the parameters analyzed, age, onset, clinical status, radiological enhancement, histological necrosis, mitosis and chromosomal anomalies emerged as significant prognosis factors using univariate analysis. Multivariate analysis revealed age and chromosomal anomalies as independent factors of survival. RESULTS: The factors with a significant prognostic value were combined to determine which grouping factors best predict outcome. The proposed score is a pure number resulting from a combination of: 2 major factors: age and chromosomal anomalies (scored 3-0); 5 minor factors: onset, clinical examination, necrosis, mitoses (scored 1-0), and radiological enhancement (scored 2-0). According to our scale, 10 survival curves were produced for overall survival. Recursive partitioning of patients with the nearest score and outcome produced four groups with a significant difference in survival (p=10(-5)). The power of both the scale and the partitioned groups for predicting outcome was more accurate than the WHO and St Anne grading systems, and the molecular sub-classification. CONCLUSIONS: Our scale is a plausible way of classifying patients harboring intracranial OD according to expected survival.

Dural plasmacytoma revealing multiple myeloma. Case report.

The authors describe the case of a 72-year-old woman with dural plasmacytoma revealing an immunoglobulin (Ig) G-kappa multiple myeloma (MM). She presented with headaches and left hemiparesis. Magnetic resonance imaging demonstrated a right frontal extraaxial lesion arising from the dura mater, and biological studies revealed hypercalcemia, hyperproteinemia, and a serum gamma globulin peak. A diagnosis of IgG-kappa MM was based on microscopic examination and immunohistochemical analysis of the dural plasmacytoma as well as on signs of systemic myeloma after surgery. The patient died 3 years after the first symptoms of MM despite systemic chemotherapy and no recurrence of the dural plasmacytoma. Myelomatous involvement of the dura mater is a rare occurrence given that only three cases have been reported to date. Nevertheless, this pathological entity should be differentiated from solitary dural plasmacytoma (SDP) because the prognosis is radically different. Progression seems to be correlated with systemic disease in contrast to the long-term survival associated with SDP. Careful systemic evaluation should be made in such a presentation to rule out MM, which would require different management and has a different prognosis.