Predictors of salvage therapy for parasagittal meningiomas treated with primary surgery, radiosurgery, or surgery plus adjuvant radiotherapy.

OBJECTIVE: Parasagittal meningiomas (PM) are treated with primary microsurgery, radiosurgery (SRS), or surgery with adjuvant radiation. We investigated predictors of tumor progression requiring salvage surgery or radiation treatment. We sought to determine whether primary treatment modality, or radiologic, histologic, and clinical variables were associated with tumor progression requiring salvage treatment. METHODS: Retrospective study of 109 consecutive patients with PMs treated with primary surgery, radiation (RT), or surgery plus adjuvant RT (2000-2017) and minimum 5 years follow-up. Patient, radiologic, histologic, and treatment data were analyzed using standard statistical methods. RESULTS: Median follow up was 8.5 years. Primary treatment for PM was surgery in 76 patients, radiation in 16 patients, and surgery plus adjuvant radiation in 17 patients. Forty percent of parasagittal meningiomas in our cohort required some form of salvage treatment. On univariate analysis, brain invasion (OR: 6.93, p < 0.01), WHO grade 2/3 (OR: 4.54, p < 0.01), peritumoral edema (OR: 2.81, p = 0.01), sagittal sinus invasion (OR: 6.36, p < 0.01), sagittal sinus occlusion (OR: 4.86, p < 0.01), and non-spherical shape (OR: 3.89, p < 0.01) were significantly associated with receiving salvage treatment. On multivariate analysis, superior sagittal sinus invasion (OR: 8.22, p = 0.01) and WHO grade 2&3 (OR: 7.58, p < 0.01) were independently associated with receiving salvage treatment. There was no difference in time to salvage therapy (p = 0.11) or time to progression (p = 0.43) between patients receiving primary surgery alone, RT alone, or surgery plus adjuvant RT. Patients who had initial surgery were more likely to have peritumoral edema on preoperative imaging (p = 0.01). Median tumor volume was 19.0 cm3 in patients receiving primary surgery, 5.3 cm3 for RT, and 24.4 cm3 for surgery plus adjuvant RT (p < 0.01). CONCLUSION: Superior sagittal sinus invasion and WHO grade 2/3 are independently associated with PM progression requiring salvage therapy regardless of extent of resection or primary treatment modality. Parasagittal meningiomas have a high rate of recurrence with 80.0% of patients with WHO grade 2/3 tumors with sinus invasion requiring salvage treatment whereas only 13.6% of the WHO grade 1 tumors without sinus invasion required salvage treatment. This information is useful when counseling patients about disease management and setting expectations.

Cranial Nerve Palsies in the Setting of Arachnoid Cysts: A Case Series and Literature Review.

BACKGROUND: Arachnoid cysts (ACs) are benign lesions typically believed to not cause neurologic defects in the adult population and are most often found incidentally on imaging. We describe 2 patients with ACs potentially leading to isolated cranial nerve (CN) dysfunction. METHODS: We describe 2 patients, 1 with a fourth nerve palsy and the other with a sixth nerve palsy found to have ACs on MRI brain imaging in locations that potentially caused a compressive CN palsy. We review previous literature of ACs presenting with CN III, IV, or VI palsy. RESULTS: Patient 1 was a 62-year-old man who presented with a 22-year history of diplopia with strabismus examination consistent with a congenital CN IV palsy. Despite multiple surgeries, his CN IV palsy insidiously worsened. An AC in the posterior fossa with mass effect on the quadrigeminal plate and asymmetric atrophy of the right superior oblique was identified on imaging. Patient 2 was an 80-year-old man who presented with an 18-year history of diplopia and was found to have a left esotropia and abduction deficit consistent with complete CN VI palsy. An AC in the left cavernous sinus was identified on imaging. He underwent strabismus surgery with satisfactory resolution of diplopia. We identified a total of 18 previously published cases: 8 reports of CN III palsy, 4 reports of CN IV palsy, and 6 reports of CN VI palsy. Patient ages ranged from 1 to 67 with a median of 34.5. In 16/18 (89%) cases, the diagnosis of ACs was made within 1 year of symptom onset. Surgical removal of the AC was successful in resolving diplopia in 7/12 (58%) cases. In no case was strabismus surgery performed as primary treatment. CONCLUSIONS: Although ACs are typically congenital asymptomatic lesions, we present a case series of 2 patients with ACs in anatomic locations that potentially caused chronic, progressive, isolated CN palsies leading to strabismus. Our literature review revealed that most published cases detailing this clinical scenario resulted in neurosurgical fenestration of ACs with mixed results. Our cases represent 2 patients with AC-associated CN palsy treated with strabismus surgery.

Case Report: Anterior Spinal Cord Ischemia Following Embolization of Cerebellar Arteriovenous Malformation: An Illustrative Case and Review of Spinal Cord Vascular Anatomy.

Spinal cord ischemia (SCI) is a rare entity with high mortality and morbidity which can arise from causes such as atherosclerosis, aortic dissection or aneurysm, thromboembolic events or systemic hypotension, and is a potential complication of spinal surgery. Published literature contains very few reports of SCI as a complication of intracranial interventions, highlighting the uncommon nature of SCI in these circumstances. We report the occurrence of anterior SCI in a 69-year-old patient following successful embolization of a cerebellar arteriovenous malformation (AVM), marked by upper extremity weakness, lower extremity paraplegia, loss of bladder and bowel control, and hypercapnic respiratory failure requiring mechanical ventilation. Magnetic resonance imaging (MRI) demonstrated upper cervical diffusion restriction and T2/STIR hyperintensity. Unusually, SCI occurred in this case without intraprocedural catheter wedging or obvious flow limitation, prolonged procedure time, hypercoagulable state, or general hypotension. We review previous cases in the literature as well as spinal cord vascular anatomy, and discuss the possible etiologies of this complication. Spinal cord ischemia could be a very rare complication of neuroendovascular procedures even in the absence of warning signs and should be carefully evaluated in patients with suspected neurologic symptoms after such procedures.

Will CT ordering practices change if we educate residents about the potential effects of radiation exposure? Experience at a large academic medical center.

RATIONALE AND OBJECTIVES: The aim of this study was to determine if educating residents about the potential effects of radiation exposure from computed tomographic (CT) imaging alters ordering patterns. This study also explored whether referring physicians are interested in radiation education and was an initial effort to address their CT ordering behavior. MATERIALS AND METHODS: Two to four months after a radiologist's lecture on the potential effects of radiation exposure related to CT scans, urology and orthopedic residents were surveyed regarding the number and types of CT scans they ordered, the use of alternative imaging modalities, and whether they used the lecture information to educate patients. RESULTS: Twenty-one resident lecture attendants completed the survey. The number of CT scans ordered after the lecture stayed constant for 90% (19 of 21) and decreased for 10% (two of 21). The types of CT scans ordered changed after the lecture for 14% (three of 21). Thirty-three percent (seven of 21) reported increases in alternative imaging after the lecture, including 24% (five of 21) reporting increases in magnetic resonance imaging and 19% (four of 21) reporting increases in ultrasound. Patients directed questions about radiation exposure to 57% (12 of 21); 38% (eight of 21) used the lecture information to educate patients. Referring physicians were interested in the topic, and afterward, other physician groups requested radiation education lectures. CONCLUSIONS: Most clinicians did not change their CT scan ordering after receiving education about radiation from a radiologist. Radiation education allowed clinicians to discuss CT benefits and risks with their patients and to choose appropriate CT protocols. Referring physician groups are interested in this topic, and radiologists should be encouraged to give radiation lectures to them.

Frequency and diagnostic utility of intralabyrinthine FLAIR hyperintensity in the evaluation of internal auditory canal and inner ear pathology.

RATIONALE AND OBJECTIVES: Fluid-attenuated inversion recovery (FLAIR) imaging can detect elevated protein levels in fluid that may be difficult or impossible to detect with T1- or T2-weighted imaging. The purpose of this study is to evaluate the frequency and asses the diagnostic utility of abnormal FLAIR signal in various types of inner ear and internal auditory canal (IAC) pathology. MATERIALS AND METHODS: A retrospective review of medical records and magnetic resonance images from 187 consecutive patients who underwent imaging of the temporal bones for possible inner ear or IAC pathology over a 1-year period were reviewed for abnormal increased FLAIR signal, increased intrinsic T1 signal, abnormal enhancement after gadolinium administration, and the presence of a mass lesion within the cerebellopontine angle, IAC, or inner ear. Reviewers were blinded to clinical diagnosis. RESULTS: Twenty-five of 32 (78%) patients with schwannomas restricted to the IAC and cerebellopontine angle demonstrated associated increased FLAIR signal within the ipsilateral inner ear structures. The sensitivity, specificity, positive predictive value, and negative predictive value of inner ear FLAIR hyperintensity for a schwannoma were 80%, 95%, 78%, and 95%, respectively. Inner ear FLAIR hyperintensity was also seen in cases of intracochlear hemorrhage, labyrinthitis, and labyrinthitis ossificans, but these conditions did not occur with sufficient frequency in our study population to determine statistical significance. CONCLUSION: Identification of inner ear FLAIR hyperintensity can alert the radiologist to scrutinize pre- and post-gadolinium T1-weighted images and T2-weighted images for subtle IAC and inner ear abnormalities. Although enhancement after the administration of gadolinium contrast media is the gold standard for detection of schwannoma, inner ear FLAIR hyperintensity may be a helpful diagnostic adjunct for vestibular schwannoma. This may be particularly helpful when gadolinium was not administered and the T2-weighted images are equivocal, especially when dedicated thin-section imaging of the IACs and temporal bones was not performed.

Autologous non-myeloablative haemopoietic stem cell transplantation in relapsing-remitting multiple sclerosis: a phase I/II study.

BACKGROUND: Autologous non-myeloablative haemopoietic stem cell transplantation is a method to deliver intense immune suppression. We evaluated the safety and clinical outcome of autologous non-myeloablative haemopoietic stem cell transplantation in patients with relapsing-remitting multiple sclerosis (MS) who had not responded to treatment with interferon beta. METHODS: Eligible patients had relapsing-remitting MS, attended Northwestern Memorial Hospital, and despite treatment with interferon beta had had two corticosteroid-treated relapses within the previous 12 months, or one relapse and gadolinium-enhancing lesions seen on MRI and separate from the relapse. Peripheral blood haemopoietic stem cells were mobilised with 2 g per m2 cyclophosphamide and 10 microg per kg per day filgrastim. The conditioning regimen for the haemopoietic stem cells was 200 mg per kg cyclophosphamide and either 20 mg alemtuzumab or 6 mg per kg rabbit antithymocyte globulin. Primary outcomes were progression-free survival and reversal of neurological disability at 3 years post-transplantation. We also sought to investigate the safety and tolerability of autologous non-myeloablative haemopoietic stem cell transplantation. FINDINGS: Between January, 2003, and February, 2005, 21 patients were treated. Engraftment of white blood cells and platelets was on median day 9 (range day 8-11) and patients were discharged from hospital on mean day 11 (range day 8-13). One patient had diarrhoea due to Clostridium difficile and two patients had dermatomal zoster. Two of the 17 patients receiving alemtuzumab developed late immune thrombocytopenic purpura that remitted with standard therapy. 17 of 21 patients (81%) improved by at least 1 point on the Kurtzke expanded disability status scale (EDSS), and five patients (24%) relapsed but achieved remission after further immunosuppression. After a mean of 37 months (range 24-48 months), all patients were free from progression (no deterioration in EDSS score), and 16 were free of relapses. Significant improvements were noted in neurological disability, as determined by EDSS score (p<0.0001), neurological rating scale score (p=0.0001), paced auditory serial addition test (p=0.014), 25-foot walk (p<0.0001), and quality of life, as measured with the short form-36 (SF-36) questionnaire (p<0.0001). INTERPRETATION: Non-myeloablative autologous haemopoietic stem cell transplantation in patients with relapsing-remitting MS reverses neurological deficits, but these results need to be confirmed in a randomised trial.

Linkage to chromosome 2q36.1 in autosomal dominant Dandy-Walker malformation with occipital cephalocele and evidence for genetic heterogeneity.

We previously reported a Vietnamese-American family with isolated autosomal dominant occipital cephalocele. Upon further neuroimaging studies, we have recharacterized this condition as autosomal dominant Dandy-Walker with occipital cephalocele (ADDWOC). A similar ADDWOC family from Brazil was also recently described. To determine the genetic etiology of ADDWOC, we performed genome-wide linkage analysis on members of the Vietnamese-American and Brazilian pedigrees. Linkage analysis of the Vietnamese-American family identified the ADDWOC causative locus on chromosome 2q36.1 with a multipoint parametric LOD score of 3.3, while haplotype analysis refined the locus to 1.1 Mb. Sequencing of the five known genes in this locus did not identify any protein-altering mutations. However, a terminal deletion of chromosome 2 in a patient with an isolated case of Dandy-Walker malformation also encompassed the 2q36.1 chromosomal region. The Brazilian pedigree did not show linkage to this 2q36.1 region. Taken together, these results demonstrate a locus for ADDWOC on 2q36.1 and also suggest locus heterogeneity for ADDWOC.

Extradural spinal meningioma as a source of plasmacytoid cells. A case report.

BACKGROUND: Meningiomas, tumors that often affect middle-aged and elderly people, occasionally arise in the spine, typically at the thoracic level. The cytologic findings in meningiomas include whorls and syncytial clusters of bland-looking cells with scattered, psammomatous calcifications and intranudclear cytoplasmic inclusions. However, in many cases, not all these findings are seen, and in rare cases, unusual cytomorphologic features are observed. CASE: A case of spinal meningioma was located in the extradural compartment and composed predominantly of singly scattered cells with a plasmacytoid appearance, demonstrated on fine needle aspiration biopsy smear preparations. The cell block showed more typical features of meningioma, and the diagnosis was supported by the results of immunohistochemical staining. CONCLUSION: The diagnosis of spinal meningioma is readily made by employing magnetic resonance imaging. The diagnosis can be difficult to confirm pathologically when atypical histologic findings are present, as in this case, with prominent plasmacytoid features. Sections from the cell block and immunohistochemical stains as well as clinical and radiologic findings were extremely helpful in arriving at the final diagnosis.

Radiation doses in interventional radiology procedures: the RAD-IR Study. Part III: Dosimetric performance of the interventional fluoroscopy units.

PURPOSE: To present the physics data supporting the validity of the clinical dose data from the RAD-IR study and to document the performance of dosimetry-components of these systems over time. MATERIALS AND METHODS: Sites at seven academic medical centers in the United States prospectively contributed data for each of 12 fluoroscopic units. All units were compatible with International Electrotechnical Commission (IEC) standard 60601-2-43. Comprehensive evaluations and periodic consistency checks were performed to verify the performance of each unit's dosimeter. Comprehensive evaluations compared system performance against calibrated ionization chambers under nine combinations of operating conditions. Consistency checks provided more frequent dosimetry data, with use of each unit's built-in dosimetry equipment and a standard water phantom. RESULTS: During the 3-year study, data were collected for 48 comprehensive evaluations and 581 consistency checks. For the comprehensive evaluations, the mean (95% confidence interval range) ratio of system to external measurements was 1.03 (1.00-1.05) for fluoroscopy and 0.93 (0.90-0.96) for acquisition. The expected ratio was 0.93 for both. For consistency checks, the values were 1.00 (0.98-1.02) for fluoroscopy and 1.00 (0.98-1.02) for acquisition. Each system was compared across time to its own mean value. Overall uncertainty was estimated by adding the standard deviations of the comprehensive and consistency measurements in quadrature. The authors estimate that the overall error in clinical cumulative dose measurements reported in RAD-IR is 24%. CONCLUSION: Dosimetric accuracy was well within the tolerances established by IEC standard 60601-2-43. The clinical dose data reported in the RAD-IR study are valid.

Intracranial chondrosarcoma: a case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Chondrosarcomas rarely occur intracranially away from the skull base. We present a case of a supratentorial dural-based, low-grade classic chondrosarcoma and emphasize salient imaging findings that assist in its diagnosis. CLINICAL PRESENTATION: A 23-year-old female presented with new onset seizures. Multiple radiographic imaging studies identified a large left frontal mass with radiological findings that suggested the correct diagnosis. INTERVENTION: The patient underwent a left frontal craniotomy and gross total resection of the lesion and associated dura. CONCLUSION: Intracranial chondrosarcomas away from the skull based are extremely rare malignancies. As with other extra-axial masses, they often grow to a relatively large size before generating symptoms. Careful analysis of CT, MR, and angiographic imaging characteristics should suggest the correct diagnosis.

Radiation doses in interventional radiology procedures: the RAD-IR study: part II: skin dose.

PURPOSE: To determine peak skin dose (PSD), a measure of the likelihood of radiation-induced skin effects, for a variety of common interventional radiology and interventional neuroradiology procedures, and to identify procedures associated with a PSD greater than 2 Gy. MATERIALS AND METHODS: An observational study was conducted at seven academic medical centers in the United States. Sites prospectively contributed demographic and radiation dose data for subjects undergoing 21 specific procedures in a fluoroscopic suite equipped with built-in dosimetry capability. Comprehensive physics evaluations and periodic consistency checks were performed on each unit to verify the stability and consistency of the dosimeter. Seven of 12 fluoroscopic suites in the study were equipped with skin dose mapping software. RESULTS: Over a 3-year period, skin dose data were recorded for 800 instances of 21 interventional radiology procedures. Wide variation in PSD was observed for different instances of the same procedure. Some instances of each procedure we studied resulted in a PSD greater than 2 Gy, except for nephrostomy, pulmonary angiography, and inferior vena cava filter placement. Some instances of transjugular intrahepatic portosystemic shunt (TIPS) creation, renal/visceral angioplasty, and angiographic diagnosis and therapy of gastrointestinal hemorrhage produced PSDs greater than 3 Gy. Some instances of hepatic chemoembolization, other tumor embolization, and neuroembolization procedures in the head and spine produced PSDs greater than 5 Gy. In a subset of 709 instances of higher-dose procedures, there was good overall correlation between PSD and cumulative dose (r = 0.86; P <.000001) and between PSD and dose-area-product (r = 0.85, P <.000001), but there was wide variation in these relationships for individual instances. CONCLUSIONS: There are substantial variations in PSD among instances of the same procedure and among different procedure types. Most of the procedures observed may produce a PSD sufficient to cause deterministic effects in skin. It is suggested that dose data be recorded routinely for TIPS creation, angioplasty in the abdomen or pelvis, all embolization procedures, and especially for head and spine embolization procedures. Measurement or estimation of PSD is the best method for determining the likelihood of radiation-induced skin effects. Skin dose mapping is preferable to a single-point measurement of PSD.

Isolated dissections and dissecting aneurysms of the posterior inferior cerebellar artery: topic and literature review.

Isolated dissections of the posterior inferior cerebellar artery (PICA) are rare. Thus, no large series of cases have been reported in the literature. Due to limited knowledge regarding the natural history of these lesions and the lack of high-quality evidence supporting various treatment options, management is controversial and practice parameters are ill defined. In order to offer a comprehensive reference for the diagnosis and management of isolated PICA dissections, the authors reviewed the National Library of Medicine from 1966 to October 2001. Twenty-seven patients averaging 43.6 years of age and including 14 males and 13 females were reported. Subarachnoid hemorrhage occurred in 20 patients, and two died. Dissections were located in the proximal PICA in 22 patients and were three times more common on the left side (left:right=3:1). Six patients were managed conservatively, and four with endovascular techniques. Seventeen had open surgery: five underwent resection, two went through trapping, and two had proximal clipping. Wrapping with muscle was performed in two patients, encasement with Sundt clips in two, and four had occipital artery (OA)-PICA bypass surgery. A meticulous analysis of reported cases with regard to clinical and pathological features, management strategies, and outcomes is presented.

Radiation doses in interventional radiology procedures: the RAD-IR study: part I: overall measures of dose.

PURPOSE: To determine patient radiation doses for interventional radiology and neuroradiology procedures, to identify procedures associated with higher radiation doses, and to determine the effects of various parameters on patient doses. MATERIALS AND METHODS: A prospective observational study was performed at seven academic medical centers. Each site contributed demographic and radiation dose data for subjects undergoing specific procedures in fluoroscopic suites equipped with built-in cumulative dose (CD) and dose-area-product (DAP) measurement capability compliant with International Electrotechnical Commission standard 60601-2-43. The accuracy of the dosimetry was confirmed by comprehensive measurements and by frequent consistency checks performed over the course of the study. RESULTS: Data were collected on 2,142 instances of interventional radiology procedures, 48 comprehensive physics evaluations, and 581 periodic consistency checks from the 12 fluoroscopic units in the study. There were wide variations in dose and statistically significant differences in fluoroscopy time, number of images, DAP, and CD for different instances of the same procedure, depending on the nature of the lesion, its anatomic location, and the complexity of the procedure. For the 2,142 instances, observed CD and DAP correlate well overall (r = 0.83, P <.000001), but correlation in individual instances is poor. The same is true for the correlation between fluoroscopy time and CD (r = 0.79, P <.000001). The correlation between fluoroscopy time and DAP (r = 0.60, P <.000001) is not as good. In 6% of instances (128 of 2,142), which were principally embolization procedures, transjugular intrahepatic portosystemic shunt (TIPS) procedures, and renal/visceral artery stent placements, CD was greater than 5 Gy. CONCLUSIONS: Most procedures studied can result in clinically significant radiation dose to the patient, even when performed by trained operators with use of dose-reducing technology and modern fluoroscopic equipment. Embolization procedures, TIPS creation, and renal/visceral artery stent placement are associated with a substantial likelihood of clinically significant patient dose. At minimum, patient dose data should be recorded in the medical record for these three types of procedures. These data should include indicators of the risk of deterministic effects as well as the risk of stochastic effects.

Superior sagittal sinus thrombosis in a patient with postdural puncture headache.

BACKGROUND AND OBJECTIVES: The occurrence of concomitant intracranial pathology in a patient with postdural puncture headache (PDPH) is rare. We present a patient who had a superior sagittal sinus thrombosis in addition to his PDPH. The signs and symptoms of intracranial pathology in patients with dural puncture headache, in addition to their postural headache, are discussed. CASE REPORT: A 32-year-old man with lymphoblastic lymphoma received treatment with daunorubicin, vincristine, and prednisone. He developed postural headache and severe nausea and vomiting after a diagnostic lumbar puncture. Magnetic resonance imaging (MRI) showed superior sagittal sinus (SSS) thrombosis and meningeal enhancement. An epidural blood patch was performed and enoxaparin was prescribed for 6 months. He has remained asymptomatic. CONCLUSIONS: Patients with PDPH have classic postural headache. The occurrence of additional signs and symptoms should alert the clinician to the presence of intracranial pathology. Patients with lymphoblastic lymphoma who had treatment with L-asparaginase and steroid are predisposed to the development of cortical venous thrombosis and may have this syndrome in addition to a dural puncture headache.