Idiopathic chronic temporal lobe herniation with associated epilepsy.

Herniation of parahippocampal gyrus is usually caused by pressure differentials intracranially, and herniation without known risk factors is extremely rare. We describe a patient with a long history of seizures and a remote status epilepticus event. On magnetic resonance imaging, a presumed left temporal lobe tumor was observed. On neurosurgical consultation, the lesion was identified as a chronic mesial temporal lobe herniation. The patient lacked history that would suggest risk of cerebral herniation. Accurately identifying the patient's chronic temporal lobe herniation radiographically likely saved this patient from unnecessary surgery or biopsy and allowed the patient to receive appropriate conservative care.

Repeat radiation with bevacizumab and minocycline in bevacizumab-refractory high grade gliomas: a prospective phase 1 trial.

INTRODUCTION: There are no effective treatments for gliomas after progression on radiation, temozolomide, and bevacizumab. Microglia activation may be involved in radiation resistance and can be inhibited by the brain penetrating antibiotic minocycline. In this phase 1 trial, we examined the safety and effect on survival, symptom burden, and neurocognitive function of reirradiation, minocycline, and bevacizumab. METHODS: The trial used a 3 + 3 design for dose escalation followed by a ten person dose expansion. Patients received reirradiation with dosing based on radiation oncologist judgment, bevacizumab 10 mg/kg IV every two weeks, and oral minocycline twice a day. Symptom burden was measured using MDASI-BT. Neurocognitive function was measured using the COGSTATE battery. RESULTS: The maximum tolerated dose of minocycline was 400 mg twice a day with no unexpected toxicities. The PFS3 was 64.6%, and median overall survival was 6.4 months. Symptom burden and neurocognitive function did not decline in the interval between treatment completion and tumor progression. CONCLUSIONS: Minocycline 400 mg orally twice a day with bevacizumab and reirradiation is well tolerated by physician and patient reported outcomes in people with gliomas that progress on bevacizumab.

Utility of Magnetic Resonance Imaging Features for Improving the Diagnosis of Idiopathic Intracranial Hypertension Without Papilledema.

OBJECTIVE: Revised diagnostic criteria for idiopathic intracranial hypertension (IIH) were proposed in part to reduce misdiagnosis of intracranial hypertension without papilledema (WOP) by using 3 or 4 MRI features of intracranial hypertension when a sixth nerve palsy is absent. This study was undertaken to evaluate the sensitivity and specificity of the MRI criteria and to validate their utility for diagnosing IIH in patients with chronic headaches and elevated opening pressure (CH + EOP), but WOP. METHODS: Brain MRIs from 80 patients with IIH with papilledema (WP), 33 patients with CH + EOP, and 70 control patients with infrequent episodic migraine were assessed in a masked fashion for MRI features of intracranial hypertension. RESULTS: Reduced pituitary gland height was moderately sensitive for IIH WP (80%) but had low specificity (64%). Increased optic nerve sheath diameter was less sensitive (51%) and only moderately specific (83%). Flattening of the posterior globe was highly specific (97%) but had low sensitivity (57%). Transverse venous sinus stenosis was moderately sensitive for IIH WP (78%) but of undetermined specificity. A combination of any 3 of 4 MRI features was nearly 100% specific, while maintaining a sensitivity of 64%. Of patients with CH + EOP, 30% had 3 or more MRI features, suggesting IIH WOP in those patients. CONCLUSION: A combination of any 3 of 4 MRI features is highly specific for intracranial hypertension and suggests IIH WOP when present in patients with chronic headache and no papilledema.

Diffusion-Weighted Imaging and Post-contrast Enhancement in Differentiating Optic Neuritis and Non-arteritic Anterior Optic Neuropathy.

Non-arteritic anterior ischaemic optic neuropathy (NAION) and optic neuritis (ON) may be difficult to distinguish early in their disease courses. Our goal was to determine if specific magnetic resonance imaging characteristics differentiate acute NAION from ON. Neuroradiologists, masked to diagnosis, reviewed the diffusion-weighted imaging (DWI) and post-contrast enhancement (PCE) characteristics of the optic nerve in 140 eyes. PCE and DWI signals of the optic disc alone did not discriminate between NAION and ON. After taking age and sex into consideration, only DWI and PCE of the intraorbital segment of the optic nerve differentiated the two, with ON having the increased likelihood of these findings. Isolated PCE without DWI signal at the optic disc, however, was 100% specific for NAION. This may be the most specific way to radiographically differentiate between NAION and ON in the acute setting.

Spinal Neuroarthropathy: Pathophysiology, Clinical and Imaging Features, and Differential Diagnosis.

Spinal neuroarthropathy (SNA), or Charcot spine, is a progressive destructive arthropathy occurring after loss of neuroprotective sensation and proprioceptive reflexes. Clinical diagnosis is difficult because of the variable length to presentation after initial neurologic damage and the limited symptoms given preexisting neurologic deficits. SNA is also a diagnostic challenge because its imaging features are similar to those of spinal conditions such as discitis-osteomyelitis, osseous tuberculosis, hemodialysis-related spondyloarthropathy, and pseudarthrosis. The most important imaging clues for diagnosis of SNA are involvement of both anterior and posterior elements at the thoracolumbar and lumbosacral junctions. Additional imaging clues include vacuum phenomenon within the disk (indicating excessive motion), malalignment, and paraspinal soft-tissue masses or fluid collections containing bone debris. Despite these imaging signs, findings may overlap in some cases with those of infection, or SNA can be superinfected, and biopsy may be necessary. Development of SNA requires a preexisting neurologic condition, most commonly traumatic spinal cord injury. Areas of greatest mobility and weight bearing within the desensate spine experience repetitive microtrauma and unregulated hyperemia, leading to destruction of the intervertebral articulations. The progressive and destructive nature of SNA causes substantial deformity, loss of function, and often further neurologic deficits. Patients present with deformity, back pain, audible noises during movement, or new neurologic symptoms. The mainstay of treatment is surgical débridement, reduction, and fusion. The radiologist can help initiate early intervention by using key imaging features to distinguish SNA from imaging mimics and prevent further neurologic deterioration. (©)RSNA, 2016.

Molecular features assisting in diagnosis, surgery, and treatment decision making in low-grade gliomas.

The preferred management of suspected low-grade gliomas (LGGs) has been disputed, and the implications of molecular changes for medical and surgical management of LGGs are important to consider. Current strategies that make use of molecular markers and imaging techniques and therapeutic considerations offer additional options for management of LGGs. Mutations in the isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) genes suggest a role for this abnormal metabolic pathway in the pathogenesis and progression of these primary brain tumors. Use of magnetic resonance spectroscopy can provide preoperative detection of IDH-mutated gliomas and affect surgical planning. In addition, IDH1 and IDH2 mutation status may have an effect on surgical resectability of gliomas. The IDH-mutated tumors exhibit better prognosis throughout every grade of glioma, and mutation may be an early genetic event, preceding lineage-specific secondary and tertiary alterations that transform LGGs into secondary glioblastomas. The O6-methylguanine-DNAmethyltransferase (MGMT) promoter methylation and 1p19q codeletion status can predict sensitivity to chemotherapy and radiation in low- and intermediate-grade gliomas. Thus, these recent advances, which have led to a better understanding of how molecular, genetic, and epigenetic alterations influence the pathogenicity of the different histological grades of gliomas, can lead to better prognostication and may lead to specific targeted surgical interventions and medical therapies.

Diagnostic and Therapeutic Challenges of Rare Concurrent Intracranial Meningioma and Oligodendroglioma.

Introduction: Concurrent primary brain tumors are rare clinical entities, with a prevalence ranging from 0.1 to 0.5% of all diagnosed brain tumors. The co-occurrence of meningioma and oligodendroglioma is particularly uncommon, posing unique diagnostic and therapeutic challenges. We describe the case of a patient diagnosed with concurrent meningioma and oligodendroglioma and review the existing literature on this rare phenomenon. Case Presentation: A 55-year-old female patient with a history of seizures presented to the emergency department with worsening headaches, nausea, and vomiting. She had a known right frontoparietal intracranial mass but had previously declined surgery. Magnetic resonance imaging revealed extensive fluid-attenuated inversion recovery /T2 hyperintensity around the lesion, which had slowly increased over 5 years; the growth of the lesion was producing a mass effect with a significant midline shift. The patient underwent urgent hemicraniectomy with subsequent resection. Clinical evaluation, imaging studies, and histopathological examination were conducted to confirm the diagnosis. Genetic and molecular analyses were also performed to explore potential underlying mechanisms. Histopathological findings confirmed a diagnosis of an isocitrate dehydrogenase-mutated World Health Organization Grade II oligodendroglioma with 1p/19q codeletion, along with a Grade I meningioma. Conclusion: The coexistence of meningioma and oligodendroglioma represents a rare clinical event. Surgical management remains the cornerstone of treatment. Further investigation into the genetic and environmental factors that contribute to the co-occurrence of such tumors could pave the way for more targeted therapeutic strategies.

D-TERMINED, a phase 1 trial in newly diagnosed high-grade glioma with temozolomide, radiation, and minocycline followed by adjuvant minocycline/temozolomide.

Background: Standard treatment for newly diagnosed high-grade gliomas remains suboptimal. Preclinical data indicate that mesenchymal transition and radiation resistance in glioblastoma are driven by NF-κB and microglia activation, which can be inhibited by minocycline. We assessed the safety and efficacy of minocycline combined with standard radiation and temozolomide in newly diagnosed high-grade gliomas. Methods: Adults with newly diagnosed high-grade glioma were eligible. Minocycline was given with concurrent and adjuvant temozolomide. Minocycline doses were escalated using a 3 + 3 design and expanded to identify the maximum tolerated dose (MTD) and adverse event profile. Individual progression-free survival (PFS) was compared to predicted PFS based on RTOG RPA class using a binomial test. The relationships between mesenchymal and microglial biomarkers were analyzed with immunohistochemistry. Results: The MTD of minocycline was 150 mg twice per day (N = 20); 1 patient (5%) experienced CTCAE grade 3 + nausea and dizziness, and 2 patients (10%) demonstrated thrombocytopenia requiring temozolomide interruptions. Twelve patients exceeded their predicted PFS (60%), which did not meet the predefined efficacy endpoint of 70%. Symptoms increased during post-radiation treatment but remained mild. No significant correlation was seen between biomarkers and PFS. Expression levels of P-p65, a marker of NF-κB activation, were correlated with the microglia marker IBA-1. Conclusions: Minocycline at 150 mg twice per day is well tolerated with standard chemoradiation in patients with newly diagnosed high-grade gliomas. PFS was not significantly increased with the addition of minocycline when compared to historical controls. NF-κB activation correlates with microglia levels in high-grade glioma.

Intracranial hypotension: improved MRI detection with diagnostic intracranial angles.

OBJECTIVE: Intracranial hypotension is an uncommon cause of headaches that is often misdiagnosed. The classic MRI features of intracranial hypotension can be variable and subjective. The purpose of this study was to provide objective criteria in the MRI evaluation of intracranial hypotension by quantifying normal values for the pontomesencephalic angle, mamillopontine distance, and lateral ventricular angle. MATERIALS AND METHODS: A retrospective review of patients with the clinical diagnosis of intracranial hypotension and a control group was performed with measurements of the pontomesencephalic angle, mamillopontine distance, and lateral ventricular angle. Qualitative evaluation of other MRI findings included dural enhancement, venous engorgement, subdural collections, brainstem slumping, and tonsillar herniation. RESULTS: In 29 patients with intracranial hypotension, the mean pontomesencephalic angle, mamillopontine distance, and lateral ventricular angle were 41.2° (SD, ± 17.4°), 4.4 mm (SD, ± 1.8), and 130.1° (SD, ± 9.8°), respectively. In the control group, the mean pontomesencephalic angle, mamillopontine distance, and lateral ventricular angle were 65° (SD, ± 9.9°), 7.0 mm (SD, ± 1.3), and 132.2° (SD, ± 5.7°), respectively. The differences in the pontomesencephalic angle and mamillopontine distance values for the intracranial hypotension group versus the control group were statistically significant (p < 0.01). The difference in the lateral ventricular angle measurements was not statistically significant (p = 0.37). Cutoff points of a 5.5-mm mamillopontine distance and 50° pontomesencephalic angle were estimated using receiver operating characteristic curves. CONCLUSION: In patients with the clinical suspicion of intracranial hypotension, we found that cutoff values of 5.5 mm or less for the mamillopontine distance and 50° or less for the pontomesencephalic angle were sensitive and specific in strengthening the qualitative MRI findings. Therefore, quantitative assessments may provide a more accurate diagnosis.

Noncontrast computed tomographic Hounsfield unit evaluation of cerebral venous thrombosis: a quantitative evaluation.

INTRODUCTION: Our objective is to determine the utility of noncontrast Hounsfield unit values, Hounsfield unit values corrected for the patient's hematocrit, and venoarterial Hounsfield unit difference measurements in the identification of intracranial venous thrombosis on noncontrast head computed tomography. METHODS: We retrospectively reviewed noncontrast head computed tomography exams performed in both normal patients and those with cerebral venous thrombosis, acquiring Hounsfield unit values in normal and thrombosed cerebral venous structures. Also, we acquired Hounsfield unit values in the internal carotid artery for comparison to thrombosed and nonthrombosed venous structures and compared the venous Hounsfield unit values to the patient's hematocrit. RESULTS: A significant difference is identified between Hounsfield unit values in thrombosed and nonthrombosed venous structures. Applying Hounsfield unit threshold values of greater than 65, a Hounsfield unit to hematocrit ratio of greater than 1.7, and venoarterial difference values greater than 15 alone and in combination, the majority of cases of venous thrombosis are identifiable on noncontrast head computed tomography. CONCLUSION: Absolute Hounsfield unit values, Hounsfield unit to hematocrit ratios, and venoarterial Hounsfield unit value differences are a useful adjunct in noncontrast head computed tomographic evaluation of cerebral venous thrombosis.

Fusiform dilatation of the cavernous carotid artery in acromegalic patients.

BACKGROUND: Surgeons undertaking transsphenoidal surgery in patients with acromegaly confront multiple unique challenges secondary to the anatomic alterations caused by growth hormone-secreting tumors. The senior author has noted a fusiform dilatation of the cavernous carotid artery in many acromegalic patients. The authors aim to quantify this dilatation and correlate it with potential contributing factors. METHODS: Clinical and radiographic data were retrospectively assessed in acromegalic patients undergoing transsphenoidal surgery from 2000 through 2011. Randomly selected patients with nonsecreting pituitary adenomas were used as the control cohort. Demographic information, comorbidities, and preoperative growth hormone and insulin-like growth factor-1 levels were recorded. Magnetic resonance (MR) imaging variables included tumor size, diameters of the petrous, cavernous, and supraclinoid segments of the carotid artery, and extent and location of cavernous sinus invasion. Independent correlations between acromegaly and each variable were assessed with multivariate regression analysis. RESULTS: Forty randomly selected patients with growth hormone-secreting adenomas who underwent surgery and had MR imaging with thin coronal slices of the pituitary region were enlisted in our study cohort. The mean age was 45.7 years. Forty-two males (52.5 %) were included in the study. Mean carotid artery diameter measurements for acromegalic and control patients, respectively, were 4.2 vs. 3.8 mm (petrous carotid), 5.0 vs. 4.0 mm (cavernous carotid), and 3.3 vs. 2.9 mm (supraclinoid carotid). Multivariate analysis showed only age and cavernous carotid diameter were statistically significant independent variables (p = 0.02, p < 0.001, respectively). Age, tumor size, growth-hormone or insulin-like growth factor-1 levels, and cavernous sinus invasion did not correlate with cavernous carotid artery diameter. CONCLUSIONS: In patients with acromegaly, there is a fusiform dilatation of the cavernous carotid artery that must be considered when planning transsphenoidal surgery.

Conventional and Advanced Imaging of Spinal Cord Tumors.

Spinal cord tumors are best identified by conventional MR imaging with contrast. Most intramedullary spinal cord tumors have characteristic MR imaging features that allow an accurate preoperative diagnosis. The spinal cord tumors reviewed in this article include the most common tumors, ependymomas and astrocytomas, as well as the less common tumors such as hemangioblastomas and metastases. Rare tumors such as primary CNS lymphoma and melanocytic tumors are also described. Advanced imaging techqniques of more common intramedullary tumors are also reviewed.

Pearls & Oy-sters: ATX-FGF14 Mimicking Autoimmune Pathology.

ATX-FGF14 (formerly spinocerebellar ataxia 27, OMIM #193003) is an autosomal dominant condition caused by a pathogenic variant in the fibroblast growth factor 14 (FGF14, OMIM #601515) gene located on chromosome 13. The phenotypic expression can vary in patients with the same genotype, often delaying diagnosis, especially in probands without known affected relatives and/or with limited available family history. We describe 2 cases of ATX-FGF14 in 1 family with a focus on the importance of differentiating episodic manifestations of neurogenetic conditions from inflammatory/autoimmune neurologic conditions. A 68-year-old male patient (case 1) presented with episodic dysarthria, dizziness, imbalance, and encephalopathy, creating suspicion for a possible autoimmune etiology. At the first evaluation, the patient reported no significant family history. Four years later, on revisiting the family history, he noted that his 49-year-old niece (case 2) had also developed neurologic symptoms of an unclear etiology. On evaluation, she had tremor and ataxia. Both patients also had coexistent evidence of systemic autoimmunity that likely contributed to the initial suspicion of neurologic autoimmunity, and neither had cerebellar or brainstem volume loss. Ultimately, their genetic testing revealed a pathogenic structural variant in the FGF14 gene, consistent with ATX-FGF14. These 2 cases highlight the importance of a detailed interval family history at each visit, especially in undiagnosed adult patients, as well as the importance of objectively analyzing the impact of immunotherapy diagnostic treatment trials to avoid unnecessary immunomodulatory medications.

Lateralized petrous internal carotid artery: imaging features and distinction from the aberrant internal carotid artery.

INTRODUCTION: This study aimed to describe the lateralized petrous internal carotid artery (ICA), a rare variant of the intratemporal course of the ICA, and distinguish it from aberrant ICA. METHODS: A retrospective multi-institutional review of all patients diagnosed over a 10-year period with lateralized ICA was completed. Medical records were reviewed for demographic data as well as clinical information in all patients. Computerized tomography (CT) studies were reviewed in all patients. Magnetic resonance studies in this patient group were reviewed when available. In order to obtain normative data for the ICA, the intratemporal course of the ICA was evaluated on 50 consecutive high-resolution sinus CT scans. RESULTS: Sixteen cases of lateralized ICA were identified on CT scans in 12 patients. In each of these, the ICA entered the skull base in a position more lateral to the cochlea than normal and protruded into the anterior mesotympanum with dehiscent or thinned overlying bone. Magnetic resonance angiography was available in 5 of 12 patients and catheter angiography in 1 of 12. CONCLUSION: Lateralized petrous ICA can be identified on CT by its more posterolateral entrance to the skull base and protrusion into the anterior mesotympanum. It can be distinguished from the aberrant ICA which enters the posterior hypotympanum through an enlarged inferior tympanic canaliculus, then courses across the inferior cochlear promontory to connect with the normal horizontal petrous ICA. Lateralized ICA is best considered an incidental petrous ICA variant. Awareness of this entity is important in the presurgical evaluation of the temporal bone to avoid vascular injury and confusion with the congenital diagnosis of aberrant ICA.

Adrenal Crisis Presenting as Recurrent Encephalopathy Mimicking Autoimmune, Infectious Encephalitis, and Common Variable Immune Deficiency: A Case Report.

INTRODUCTION: Adrenal crisis can present with life-threatening complications and mimic autoimmune or infectious encephalitis, and common variable immune deficiency (CVID). The literature regarding the neurological complications of adrenal crisis is limited and focuses on patients who present with hypotension and electrolyte abnormalities. CASE REPORT: A 30-year-old man presented 3 times to our hospital with encephalopathy, fever, and left sided weakness with a history of multiple autoimmune diseases and prior hospitalizations for encephalopathy. During his first 2 admissions, he was normotensive and without electrolyte abnormalities. Extensive workup for infectious, paraneoplastic, seizure, metabolic, toxic, and vascular etiologies, and autoimmune encephalitis was negative. His exam returned to baseline with empiric steroid treatment, and he was discharged. He re-presented 2 months later with encephalopathy for a third admission. During this subsequent presentation, he had hyponatremia, low serum osmolality, elevated urine sodium, undetectable morning cortisol, and 21-α hydroxylase autoantibodies. A diagnosis of autoimmune adrenal insufficiency was established, he was treated with physiological doses of hydrocortisone and fludrocortisone, and improved rapidly to near baseline function. He has remained relapse-free at 4-year follow up. During all admissions, he was also found to have low immunoglobulin G levels and met criteria for CVID; however, his immunoglobin levels recovered with steroid replacement. CONCLUSION: The reported patient demonstrated some of the neurological complications of adrenal crisis which can mimic other autoimmune conditions such as CVID. The neurologist should be aware that recurrent encephalopathy from adrenal insufficiency can occur regardless of hemodynamic or electrolyte changes on typical hospital metabolic panels.

GABA-A Receptor Encephalitis After Autologous Hematopoietic Stem Cell Transplant forMultiple Myeloma: Three Cases and Literature Review.

BACKGROUND AND OBJECTIVES: The relationship between autologous hematopoietic stem cell transplant (aHSCT) for multiple myeloma (MM) and anti-GABAA receptor (GABAAR) encephalitis is unknown. We aimed to describe the clinical features, diagnostic process, and outcome of 3 cases of anti-GABAAR encephalitis in patients with a history of prior aHSCT for MM. METHODS: A case series of 3 patients. Anti-GABAAR antibody was tested at the University of Pennsylvania Laboratory. RESULTS: The patients were all male, aged 52 (case 1), 61 (case 2), and 62 (case 3) years at encephalitis symptom onset. The duration between completion of aHSCT and the onset of encephalitis was 43, 18, and 9 months, respectively. All 3 patients presented with new seizures and altered cognitive function. Other symptoms included headache and visual obscurations in cases 1 and 2 and intractable vertigo and mania in case 3. Brain MRI demonstrated nonenhancing multifocal T2-weighted/fluid-attenuated inversion recovery cortical and subcortical hyperintensities in all 3 patients. Cases 2 and 3 underwent brain biopsy before initiating immunomodulatory therapy, which demonstrated nonspecific encephalitis with astrogliosis in the white matter; these 2 patients were started on immunotherapy for the treatment of anti-GABAAR encephalitis after 22 days and 3 months, respectively, from the first presentation. Case 1 was started on empiric immunotherapy within 8 days of presentation without requiring brain biopsy, given characteristic MRI imaging. CSF analysis demonstrated the presence of anti-GABAAR antibodies in all 3 cases. Cases 1 and 3 also tested positive for anti-GABAAR antibodies in the serum (serum test was not performed in case 2). Cases 1 and 2 recovered to work full-time within 1 year. Case 3 reported occasional myoclonic-like movement. DISCUSSION: We highlight the importance of considering anti-GABAAR encephalitis in patients with seizures, multifocal nonenhancing brain lesions, and a history of aHSCT for MM. Awareness in recovered post-aHSCT patients with MM may be crucial because prompt recognition can avoid brain biopsy and delays in treatment. The rapid initiation of immunotherapy while awaiting autoantibody results will likely improve functional outcomes.

Masses and malformations of the third ventricle: normal anatomic relationships and differential diagnoses.

The third ventricle lies in the center of the brain. It is surrounded by critical nuclear structures (the hypothalamus and thalami) and important glandular structures (the pituitary and pineal glands). Although a wide array of pathologic processes may involve the third ventricle, most are extrinsic masses. By understanding the anatomic boundaries of the third ventricle and its relationship to adjacent structures, it is possible to create short lists of differential diagnoses. Third ventricle masses can be classified as arising in or immediately adjacent to one of five locations: anterior, posterior, inferior, foramen of Monro, and intraventricular. Anterior masses involve the optic and infundibular recesses, posterior masses affect or arise in the posterior commissure and pineal gland, and inferior masses involve or affect the ventricle floor. Masses may also arise at or adjacent to the foramen of Monro or entirely within the third ventricle. Of the intraventricular masses, chordoid glioma-a rare low-grade primary neoplasm-is unique to the third ventricle. Congenital malformations of the third ventricle are uncommon and are most often noted during childhood. Most commonly, these anomalies represent malformations of the neurohypophysis, which may manifest as hormonal abnormalities, or stenosis of the aqueduct of Sylvius, which manifests as dilatation of the third and lateral ventricles (hydrocephalus).

Pineal parenchymal tumor of intermediate differentiation: imaging spectrum of an unusual tumor in 11 cases.

INTRODUCTION: Pineal parenchymal tumor of intermediate differentiation (PPTID) was recognized in the 2007 World Health Organization (WHO) classification as a new pineal parenchymal neoplasm, intermediate in malignancy (WHO grade II or III) between pineocytoma (grade I) and pineoblastoma (grade IV). The imaging spectrum of this new tumor has not been previously delineated. We describe the imaging spectrum in 11 pathologically proven PPTIDs and identify findings that may suggest the preoperative diagnosis of this newly recognized entity. METHODS: Electronic medical records over the last 9 years and teaching files between the years 1985 and 1995 were searched for atypical pineal lesions. Additional cases were added from the teaching files of contributing authors. RESULTS: Imaging studies in nine patients (9/11) showed bulky, aggressive pineal region masses with local brain invasion; two patients (2/11) demonstrated circumscribed pineal masses. Two patients had spinal metastases at presentation. On computed tomography (CT), five patients had classic "exploded" calcifications characteristic of pineal parenchymal tumors. All tumors were heterogeneously hypointense on T1WIs and heterogeneously hyperintense on T2WIs. Post-contrast scans showed marked heterogeneous (10/11) or uniform (1/11) enhancement. Cystic foci were identified in eight cases. Intratumoral hemorrhage was present in one case. CONCLUSION: While no singular neuroimaging feature is pathognomonic of PPTID, these tumors are usually larger, demonstrate local invasion, and appear much more heterogeneous than pineocytoma. Because PPTIDs have a higher grade and increased potential for recurrence as compared to pineocytomas, it is important to consider this diagnosis as shorter follow-up, and adjuvant therapy may be indicated in selected cases.

Neurosarcoidosis: Longitudinal experience in a single-center, academic healthcare system.

OBJECTIVE: To characterize patients with neurosarcoidosis within the University of Utah healthcare system, including demographics, clinical characteristics, treatment, and long-term outcomes. METHODS: We describe the clinical features and outcomes of patients with neurosarcoidosis within the University of Utah healthcare system (a large referral center for 10% of the continental United States by land mass). Patients were selected who met the following criteria: (1) at least one International Classification of Diseases Clinical Modification, 9th revision code 135 or International Classification of Diseases Clinical Modification, 10th revision code D86* (sarcoidosis) and (2) at least one outpatient visit with a University of Utah clinician in the Neurology Department within the University of Utah electronic health record. RESULTS: We identified 56 patients meeting the study criteria. Thirty-five patients (63%) were women, and most patients (84%) were white. Twelve patients (22%) met the criteria for definite neurosarcoidosis, 36 patients (64%) were diagnosed with probable neurosarcoidosis, and 8 patients (14%) were diagnosed with possible neurosarcoidosis. A total of 8 medications were used for the treatment of neurosarcoidosis. Prednisone was the first-line treatment in 51 patients (91%). Infliximab was the most effective therapy, with 87% of patients remaining stable or improving on infliximab. Treatment response for methotrexate and azathioprine was mixed, and mycophenolate mofetil and rituximab were the least effective treatments in this cohort. CONCLUSIONS: This is a comprehensive characterization of neurosarcoidosis within a single healthcare system at the University of Utah that reports long-term response to treatment and outcomes of patients with neurosarcoidosis. Our results suggest the use of infliximab as a first-line therapy for neurosarcoidosis.

Gliosarcoma: Neuroimaging and Immunohistochemical Findings.

BACKGROUND AND PURPOSE: Gliosarcoma (GSC) is an intra-axial lesion which often abuts a dural margin and is composed of glial and mesenchymal elements. This lesion is considered a variant of isocitrate dehydrogenase (IDH)-wild type glioblastoma (GBM). The purpose of this study is to evaluate the imaging and molecular features of GSC in a large patient cohort. METHODS: Pathology-proved GSC cases were collected from our quaternary care center spanning the last 16 years and IDH status was documented. Older GSC cases without prior immunohistochemical testing underwent tissue block staining to obtain IDH status. When available, p53, phosphate and tensin (PTEN), MIB-1, EGFR amplification, and MGMT methylation were recorded and imaging findings tabulated. Logistic regression analyses were performed to determine correlation of molecular markers and imaging characteristics. RESULTS: A total of 25 cases were identified (21 de novo, 4 post-treatment). All lesions contacted a dural, pial, or ependymal surface and were negative for an IDH R132H mutation, including postradiation GSC. In total, 16 of 16 cases showed nonamplification of EGFR/CEP7, 2 of 16 demonstrated MGMT methylation, and multiple lesions demonstrated p53 and PTEN mutations. Imaging features included areas of nodular thickening in necrotic lesions which appeared to abut the site of dural contact. There was no significant correlation of molecular markers with imaging characteristics. CONCLUSION: GSC was IDH(-) in all cases, supporting the current understanding of this lesion being a wild-type GBM variant. Additional molecular markers demonstrated no significant correlation with imaging findings in this cohort.