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Omar Choudhri and Michael P. Marks

Tentorial dural arteriovenous fistulae are rare intracranial fistulae, in which the fistula pocket is present within the leaves of tentorium cerebelli. These tentorial fistulae can be rarely present near the galenic complex, where they can engorge the deep venous system and cause symptoms of venous hypertension. We present an interesting case of endovascular treatment of a galenic tentorial dural arteriovenous fistula in a patient with headaches and imbalance. The fistula was accessed through the artery of Davidoff and Schecter from the posterior cerebral artery supplying the fistula. The fistula was completely embolized using Onyx and with preservation of vein of Galen.

The video can be found here: http://youtu.be/igX2X5tfvrg.

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Mark P. Piedra, Paul D. Brown, Paul C. Carpenter and Michael J. Link

✓ The authors present the case of a 58-year-old woman who presented with symptoms of diabetes insipidus (DI) 1 year after she was found to have a Stage 3 (of 4) estrogen receptor—positive infiltrating ductal adenocarcinoma of the left breast with pulmonary and bone metastases. Magnetic resonance images demonstrated a solitary site of metastasis in the patient's pituitary stalk, and gamma knife surgery (GKS) was performed to treat the lesion. Three months after GKS the patient was able to reduce the medication she required for the DI. There was no evidence of pituitary failure and no negative effect on her vision.

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Omar Choudhri, Abdullah Feroze, Michael P. Marks and Huy M. Do

Cerebral venous sinus thrombosis (CVST) is characterized by formation of widespread thrombus within the cerebral venous sinus system. CVST can cause venous hypertension, venous infarcts, hemorrhage and seizures. It is managed in most cases with systemic anticoagulation through the use of heparin to resolve the thrombus. Patients that demonstrate clinical deterioration while on heparin are often treated with endovascular strategies to recanalize the sinuses. We present the case of a patient with widespread CVST, involving his superior sagittal sinuses and bilateral transverse sigmoid sinuses, who was treated with a combination of endovascular therapies.

The video can be found here: http://youtu.be/w3wAGlT7h8c.

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Gayle S. Storey, Michael P. Marks, Michael Dake, Alexander M. Norbash and Gary K. Steinberg

✓ The authors report initial results and follow up using stent placement to treat atherosclerotic stenosis in vertebral arteries. Three patients with severe atherosclerotic vascular disease underwent vertebral artery stent placement using a balloon expandable stent. Medical therapy (aspirin and warfarin) and conventional percutaneous angioplasty failed to resolve the disease and the patients developed symptomatic restenosis within 3 months of angioplasty. Two patients had symptoms of anterior circulation ischemia with carotid artery occlusions and reduced supply to the anterior circulation from the stenosed vertebral arteries. One patient had recurrent posterior circulation symptoms. Stents were successfully placed in all three, resulting in immediate reversal of stenosis and resolution of symptoms. Clinical follow-up study (mean 9 months) has shown no recurrent symptoms in the patient with posterior circulation symptoms, but the two patients with anterior circulation ischemia did develop recurrent symptoms. Angiographic follow up in these two patients at 3 months and 1 year, however, demonstrated continued patency of vertebral artery lumina. They underwent extracranial—intracranial bypass surgery to relieve their symptoms. This experience suggests stents can be placed without complication in the proximal vertebral arteries and may have an adjunctive role in the treatment of atherosclerotic cerebrovascular disease following unsuccessful angioplasty.

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Subash Lohani, Diana P. Rodriguez, Hart G. W. Lidov, R. Michael Scott and Mark R. Proctor

Object

Intrasacral meningoceles are rare cystic lesions that can cause focal compression within the bony sacral canal. Their mechanisms are poorly understood, but most intrasacral meningoceles appear to be intrasacral extradural cysts caused by arachnoid herniating through a small dural defect in the caudal end of the thecal sac. As opposed to perineural cysts, they are not associated with an exiting nerve root. When symptomatic, they can cause sacral pain or sacral nerve root dysfunction due to local compression.

Methods

This is a retrospective series from Boston Children's Hospital. All patients in whom symptomatic intrasacral meningocele that required surgical treatment was diagnosed between May 1994 and March 2011 were included in the study. Spine MRI was the diagnostic modality of choice. All patients underwent sacral exploration, with ligation and obliteration of the cyst. Resected cyst wall was subjected to pathological examination.

Results

There were 13 patients (11 boys and 2 girls) who underwent operation for intrasacral meningocele. The median age was 8 years (range 5 months–16 years). The most common presenting symptom was back pain (in 5) often described as deep tail bone pain, followed by urinary incontinence (3) and constipation (2). Three patients had evidence of associated tethered cord on MRI studies. Four patients were asymptomatic and their diagnosis was made following imaging for other reasons; they were surgically treated because of the increasing size of the lesion or association with other congenital lesions. Most patients had symptomatic improvement after surgery.

Conclusions

Intrasacral meningoceles are rare lesions that may result from a congenital dural weakness and a resultant arachnoid diverticulum. They present in childhood either incidentally or with symptoms secondary to nerve root compression. Identification of the point of herniation through the dura mater and ligation of the lesion provides cyst cure and resolution of symptoms in most patients.

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Mark P. Garrett, Richard W. Williamson, Michael A. Bohl, C. Roger Bird and Nicholas Theodore

OBJECTIVE

For a diagnosis of brain death (BD), ancillary testing is performed if patient factors prohibit a complete clinical examination and apnea test. The American Academy of Neurology (AAN) guidelines identify cerebral angiography (CA), cerebral scintigraphy, electroencephalography, and transcranial Doppler ultrasonography as accepted ancillary tests. CA is widely considered the gold standard of these, as it provides the most reliable assessment of intracranial blood flow. CT angiography (CTA) is a noninvasive and widely available study that is also capable of identifying absent or severely diminished intracranial blood flow, but it is not included among the AAN's accepted ancillary tests because of insufficient evidence demonstrating its reliability. The objective of this study was to assess the statistical performance of CTA in diagnosing BD, using clinical criteria alone or clinical criteria plus CA as the gold-standard comparisons.

METHODS

The authors prospectively enrolled 22 adult patients undergoing workup for BD. All patients had cranial imaging and clinical examination results consistent with BD. In patients who met the AAN clinical criteria for BD, the authors performed CA and CTA so that both tests could be compared with the gold-standard clinical criteria. In cases that required ancillary testing, CA was performed as a confirmatory study, and CTA was then performed to compare against clinical criteria plus CA. Radiographic data were evaluated by an independent neuroradiologist. Test characteristics for CTA were calculated.

RESULTS

Four patients could not complete the standard BD workup and were excluded from analysis. Of the remaining 18 patients, 16 met AAN criteria for BD, 9 of whom required ancillary testing with CA. Of the 16 patients, 2 who also required CA ancillary testing were found to have persistent intracranial flow and were not declared brain dead at that time. These patients also underwent CTA; the results were concordant with the CA results. Six patients who were diagnosed with BD on the basis of clinical criteria alone also underwent CA, with 100% sensitivity. For all 18 patients included in the study, CTA had a sensitivity of 75%, a specificity of 100%, a positive predictive value of 100%, and a negative predictive value of 33%.

CONCLUSIONS

Clinical examination with or without CA remains the gold standard in BD testing. Studies assessing the statistical performance of CTA in BD testing should compare CTA to these gold standards. The statistical performance of CTA in BD testing is comparable to several of the nationally accepted ancillary tests. These data add to the growing medical literature supporting the use of CTA as a reliable ancillary test in BD testing.

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Evangelia Katsoulakis, Ilya Laufer, Mark Bilsky, Narasimhan P. Agaram, Michael Lovelock and Yoshiya Yamada

OBJECTIVE

Spine radiosurgery is increasingly being used to treat spinal metastases. As patients are living longer because of the increasing efficacy of systemic agents, appropriate follow-up and posttreatment management for these patients is critical. Tumor progression after spine radiosurgery is rare; however, vertebral compression fractures are recognized as a more common posttreatment effect. The use of radiographic imaging alone posttreatment may makeit difficult to distinguish tumor progression from postradiation changes such as fibrosis. This is the largest series from a prospective database in which the authors examine histopathology of samples obtained from patients who underwent surgical intervention for presumed tumor progression or mechanical pain secondary to compression fracture. The majority of patients had tumor ablation and resulting fibrosis rather than tumor progression. The aim of this study was to evaluate tumor histopathology and characteristics of patients who underwent pathological sampling because of radiographic tumor progression, fibrosis, or collapsed vertebrae after receiving high-dose single-fraction stereotactic radiosurgery.

METHODS

Between January 2005 and January 2014, a total of 582 patients were treated with linear accelerator–based single-fraction (18–24 Gy) stereotactic radiosurgery. The authors retrospectively identified 30 patients (5.1%) who underwent surgical intervention for 32 lesions with vertebral cement augmentation for either mechanical pain or instability secondary to vertebral compression fracture (n = 17) or instrumentation (n = 15) for radiographic tumor progression. Radiation and surgical treatment, histopathology, and long-term outcomes were reviewed. Survival and time to recurrence were calculated using the Kaplan-Meier method.

RESULTS

The mean age at the time of radiosurgery was 59 years (range 36–80 years). The initial pathological diagnoses were obtained for all patients and primarily included radioresistant tumor types, including renal cell carcinoma in 7 (22%), melanoma in 6 (19%), lung carcinoma in 4 (12%), and sarcoma in 3 (9%). The median time to surgical intervention was 24.7 months (range 1.6–50.8 months). The median follow-up and overall survival for all patients were 42.5 months and 41 months (overall survival range 7–86 months), respectively. The majority of assessed lesions showed no evidence of tumor on pathological review (25 of 32, 78%), while a minority of lesions revealed residual tumor (7 of 32, 22%). The median survival for patients after tumor recurrence was 5 months (range 2–70 months).

CONCLUSIONS

High-dose single-fraction radiosurgery is tumor ablative in the majority of instances. In a minority of cases, tumor persists and salvage treatments should be considered.

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Paul M. Foreman, Christoph J. Griessenauer, Michael Falola and Mark R. Harrigan

Object

Traumatic aneurysms occur in 10% of extracranial blunt traumatic cerebrovascular injuries (TCVI). The clinical consequences and optimal management of traumatic aneurysms are poorly understood.

Methods

A prospective study of TCVI at a Level I trauma center identified 7 patients with 19 extracranial traumatic carotid artery or vertebral artery aneurysms. An additional 6 patients with 7 traumatic aneurysms were followed outside of the prospective study, giving a total of 13 patients with 26 traumatic aneurysms. All patients were treated with 325 mg aspirin daily and underwent clinical and imaging follow-up beyond the initial hospitalization. Endovascular treatment was reserved for aneurysms demonstrating significant enlargement on follow-up imaging. Clinical and radiographic features were assessed.

Results

The 7 patients with traumatic aneurysms identified in the prospective cohort comprised 10.3% of all patients with TCVI. Two (15.4%) of the 13 total patients suffered an ischemic stroke in the setting of TCVI with traumatic aneurysm formation. No patient experienced an ischemic stroke or new symptoms after the initiation of antiplatelet therapy. Clinical and radiographic follow-up averaged 15.8 months (range 0.4–41.7 months) and 22.0 months (range 6.6–55.7 months), respectively. Ten (38.5%) of 26 aneurysms were not visualized on last follow-up, 10 (38.5%) were smaller, 1 (3.8%) was unchanged, and 5 (19.2%) were larger. Saccular aneurysms were more likely to enlarge than fusiform aneurysms (33.3% vs 11.8%). Results of a Fisher exact test tend to support the assertion that the 2 different aneurysm morphologies behave differently (p = 0.07). Two saccular aneurysms were treated with stenting.

Conclusions

The majority of traumatic aneurysms can be managed with an antiplatelet regimen of 325 mg aspirin daily and serial imaging. Saccular aneurysms have a greater tendency to enlarge when compared with fusiform aneurysms.

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Annie M. Burrows, Mark P. Mooney, Tim D. Smith, H. Wolfgang Losken and Michael I. Siegel

✓ Human unicoronal synostosis results in plagiocephaly of the cranial vault due to predictable compensatory growth patterns of the contralateral coronal, sagittal, and ipsilateral squamosal sutures. The present study describes the development of plagiocephaly and tests compensatory growth predictions in a naturally occurring rabbit model of uncorrected unicoronal synostosis. Cranial vault and sutural growth data were collected from serial x-ray films in 70 normal rabbits and 19 rabbits with congenital unicoronal synostosis from 1.5 to 18 weeks of age. One-way analysis of variance results revealed that rabbits with unicoronal synostosis had significant (p < 0.05) growth inhibition at both coronal sutures and the contralateral frontonasal suture and a significantly wider (p < 0.05) cranial vault compared to controls. Paired Student's t-tests between affected and unaffected sides of the vault in rabbits with synostosis revealed significant (p < 0.05) asymmetry, with ipsilateral coronal sutures growing less than contralateral ones. Gross qualitative examination of the adult brains revealed severe asymmetry and anteroposterior reduction on the ipsilateral side. These results demonstrate that this congenital rabbit model effectively simulates human cranial vault growth predictions from unicoronal synostosis and produces a plagiocephalic morphology.

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Joseph T. King Jr., Michael B. Horowitz, Amin B. Kassam, Howard Yonas and Mark S. Roberts

Object. Cerebral aneurysms can affect a patient's health status by rupture and stroke, impingement on neural structures, treatment side effects, or psychological stress. The authors assessed the performance, validity, and reliability of the Short Form—12 (SF-12), a self-administered written survey instrument, to assess health status in patients with cerebral aneurysms.

Methods. A cohort of 170 patients with cerebral aneurysms who were seen at a neurosurgery clinic underwent structured interviews including measurement of their health statuses (SF-12 physical component summary [PCS] and mental component summary [MCS]), functional status (Glasgow Outcome Scale score, modified Rankin Scale score, and Barthel Index), and mental health (Hospital Anxiety and Depression Scale score). The SF-12 scores were compared with US population norms by performing t-tests with unequal variances. The validity of the SF-12 was assessed by comparing the PCS and MCS scores with each patient's functional status and mental health scores by using rank-order methods. Inter-item reliability was assessed using the Cronbach alpha statistic.

Patients with cerebral aneurysms had decreased health status PCS and MCS scores when compared with population norms (p < 0.001 for all). A history of subarachnoid hemorrhage (SAH) (p = 0.006) and previous surgical or endovascular treatment (p = 0.047) was associated with worse PCS scores. The validity of the SF-12 was supported by the relationship between the PCS and MCS scores and the patient's functional status and mental health (p < 0.001 for all). The reliability of the SF-12 was documented by the Cronbach alpha statistic (α = 0.76).

Conclusions. Patients with cerebral aneurysms have a diminished physical and mental health status as measured using the SF-12. The presence of SAH and aneurysm treatment are associated with a worse physical health status. The SF-12 is a valid and reliable instrument for measuring health status in patients with cerebral aneurysms.