Avital Perry

MR Elastography Demonstrates Increased Brain Stiffness in Normal Pressure Hydrocephalus.

The authors performed MR elastography on 10 patients with normal pressure hydrocephalus and 21 age- and sex-matched volunteers. Shear waves with 60-Hz vibration frequency were transmitted to the brain by a pillow-like passive driver. Significant increase in stiffness was observed in the cerebrum, occipital lobe, parietal lobe, and temporal lobe in the NPH group compared with controls. BACKGROUND AND PURPOSE: Normal pressure hydrocephalus is a reversible neurologic disorder characterized by a triad of cognitive impairment, gait abnormality, and urinary incontinence that is commonly treated with ventriculoperitoneal shunt placement. However, multiple overlapping symptoms often make it difficult to differentiate normal pressure hydrocephalus from other types of dementia, and improved diagnostic techniques would help patient management. MR elastography is a novel diagnostic tool that could potentially identify patients with normal pressure hydrocephalus. The purpose of this study was to assess brain stiffness changes in patients with normal pressure hydrocephalus compared with age- and sex-matched cognitively healthy individuals. MATERIALS AND METHODS: MR elastography was performed on 10 patients with normal pressure hydrocephalus and 21 age- and sex-matched volunteers with no known neurologic disorders. Image acquisition was conducted on a 3T MR imaging scanner. Shear waves with 60-Hz vibration frequency were transmitted into the brain by a pillowlike passive driver. A novel postprocessing technique resistant to noise and edge artifacts was implemented to determine regional brain stiffness. The Wilcoxon rank sum test and linear regression were used for statistical analysis. RESULTS: A significant increase in stiffness was observed in the cerebrum (P = .001), occipital lobe (P < .001), parietal lobe (P = .001), and the temporal lobe (P = .02) in the normal pressure hydrocephalus group compared with healthy controls. However, no significant difference was noted in other regions of the brain, including the frontal lobe (P = .07), deep gray and white matter (P = .43), or cerebellum (P = .20). CONCLUSIONS: This study demonstrates increased brain stiffness in patients with normal pressure hydrocephalus compared with age- and sex-matched healthy controls; these findings should motivate future studies investigating the use of MR elastography for this condition and the efficacy of shunt therapy.

Deadly falls: operative versus nonoperative management of Type II odontoid process fracture in octogenarians

OBJECTIVE Type II odontoid fracture is a common injury among elderly patients, particularly given their predisposition toward low-energy falls. Previous studies have demonstrated a survival advantage following early surgery among patients older than 65 years, yet octogenarians represent a medically distinct and rapidly growing population. The authors compared operative and nonoperative management in patients older than 79 years. METHODS A single-center prospectively maintained trauma database was reviewed using ICD-9 codes to identify octogenarians with C-2 cervical fractures between 1998 and 2014. Cervical CT images were independently reviewed by blinded neurosurgeons to confirm a Type II fracture pattern. Prospectively recorded outcomes included Glasgow Coma Scale (GCS) score, Abbreviated Injury Scale (AIS) score, Injury Severity Score (ISS), additional cervical fracture, and cord injury. Primary end points were mortality at 30 days and at 1 year. Statistical tests included the Student t-test, chi-square test, Fishers exact test, Kaplan-Meier test, and Cox proportional hazard. RESULTS A total of 111 patients met inclusion criteria (94 nonoperative and 17 operative [15 posterior and 2 anterior]). Mortality data were available for 100% of patients. The mean age was 87 years (range 80-104 years). Additional cervical fracture, spinal cord injury, GCS score, AIS score, and ISS were not associated with either management strategy at the time of presentation. The mean time to death or last follow-up was 22 months (range 0-129 months) and was nonsignificant between operative and nonoperative groups (p = 0.3). Overall mortality was 13% in-hospital, 26% at 30 days, and 41% at 1 year. Nonoperative and operative mortality rates were not significant at any time point (12% vs 18%, p = 0.5 [in-hospital]; 27% vs 24%, p = 0.8 [30-day]; and 41% vs 41%, p = 1.0 [1-year]). Kaplan-Meier analysis did not demonstrate a survival advantage for either management strategy. Spinal cord injury, GCS score, AIS score, and ISS were significantly associated with 30-day and 1-year mortality; however, Cox modeling was not significant for any variable. Additional cervical fracture was not associated with increased mortality. The rate of nonhome disposition was not significant between the groups. CONCLUSIONS Type II odontoid fracture is associated with high morbidity among octogenarians, with 41% 1-year mortality independent of intervention-a dramatic decrease from actuarial survival rates for all 80-, 90-, and 100-year-old Americans. Poor outcome is associated with spinal cord injury, GCS score, AIS score, and ISS.

MR Elastography Demonstrates Unique Regional Brain Stiffness Patterns in Dementias

OBJECTIVE The purpose of this study was to investigate age-corrected brain MR elastography (MRE) findings in four dementia cohorts (Alzheimer disease, dementia with Lewy bodies, frontotemporal dementia, and normal pressure hydrocephalus) and determine the potential use as a differentiating biomarker in dementia subtypes. SUBJECTS AND METHODS Institutional review board approval and written informed consent were obtained to perform MRE on 84 subjects: 20 patients with normal pressure hydrocephalus, eight with Alzheimer disease, five with dementia with Lewy bodies, five with frontotemporal dementia, and 46 cognitively normal control subjects. Shear waves of 60-Hz vibration frequency were transmitted into the brain using a pillowlike passive driver, and brain stiffness was determined in eight different regions (cerebrum, frontal, occipital, parietal, temporal, deep gray matter-white matter, sensorimotor cortex, and cerebellum). All stiffness values were age-corrected and compared with control subjects. The Wilcoxon rank sum test and linear regression were used for statistical analysis. RESULTS Regional stiffness patterns unique to each dementing disorder were observed. Patients with Alzheimer disease and frontotemporal dementia showed decreased cerebral stiffness (p = 0.001 and p = 0.002, respectively) with regional softening of the frontal and temporal lobes. Patients with Alzheimer disease additionally showed parietal lobe and sensorimotor region softening (p = 0.039 and p = 0.018, respectively). Patients with normal pressure hydrocephalus showed stiffening of the parietal, occipital, and sensorimotor regions (p = 0.007, p < 0.001, and p < 0.0001, respectively). Patients with dementia with Lewy bodies did not show significant stiffness changes in any of the regions. CONCLUSION Quantitative MRE of changes in brain viscoelastic structure shows unique regional brain stiffness patterns between common dementia subtypes.

Delayed Cerebrospinal Fluid Rhinorrhea After Gamma Knife Radiosurgery with or without Preceding Transsphenoidal Resection for Pituitary Pathology

BACKGROUND Skull base cerebrospinal fluid (CSF) leak after gamma knife radiosurgery (GKRS) is a very rare complication. In patients who were treated with both GKRS and transsphenoidal resection (TSR) for pituitary lesions, early CSF leak occurs at a comparable rate with the general TSR population (4%). Delayed CSF leak occurring more than a year after TSR, GKRS, or dual therapy is exceedingly rare. METHODS Retrospective chart review and review of the literature. RESULTS We present 2 cases of delayed CSF leak after GKRS to treat pituitary adenoma. One patient developed CSF rhinorrhea 16 years after GKRS for growth hormone-producing pituitary adenoma. The patient had previously undergone TSR surgery 7 years prior to GKRS without complication. Additionally, a second patient developed high-flow CSF rhinorrhea 2 years after GKRS for a prolactinoma that failed dopamine agonist therapy. Both patients underwent a complicated clinical course after presentation, requiring multiple revisions for definitive CSF leak repair. CONCLUSIONS Delayed CSF leak is a rare but serious complication after GKRS independent of TSR status; urgent repair is the treatment of choice. Based on our experience, these leaks have the potential to be refractory, and we recommend aggressive reconstruction, preferably with a vascularized flap, and potentially supplemented by placement of a lumbar drain and acetazolamide. Current evidence is scant and provides little insight regarding an underlying mechanism, which may include bony destruction by the tumor, delayed radiation necrosis, or a secondary empty sella syndrome.

Characterizing and predicting the Nelson-Salassa syndrome.

OBJECTIVE Nelson-Salassa syndrome (NSS) is a rare consequence of bilateral adrenalectomy (ADX) for refractory hypercortisolism due to Cushing disease (CD). Although classically defined by rapid growth of a large, invasive, adrenocorticotropin hormone (ACTH)-secreting pituitary tumor after bilateral ADX that causes cutaneous hyperpigmentation, visual disturbance, and high levels of ACTH, clinical experience suggests more variability. METHODS The authors conducted a retrospective chart review of all patients 18 years and older with a history of bilateral ADX for CD, adequate pituitary MRI, and at least 2 years of clinical follow-up. Statistical tests included Students t-test, chi-square test, Fishers exact test, multivariate analysis, and derived receiver operating characteristic curves. RESULTS Between 1956 and 2015, 302 patients underwent bilateral ADX for the treatment of hypercortisolism caused by CD; 88 had requisite imaging and follow-up (mean 16 years). Forty-seven patients (53%) had radiographic progression of pituitary disease and were diagnosed with NSS. Compared with patients who did not experience progression, those who developed NSS were significantly younger at the time of CD diagnosis (33 vs 44 years, p = 0.007) and at the time of bilateral ADX (35 vs 49 years, p = 0.007), had larger tumors at the time of CD diagnosis (6 mm vs 1 mm, p = 0.03), and were more likely to have undergone external-beam radiation therapy (EBRT, 43% vs 12%, p = 0.005). Among NSS patients, the mean tumor growth was 7 mm/yr (SE 6 mm/yr); the median tumor growth was 3 mm/yr. Prevalence of pathognomonic symptoms was low; the classic triad occurred in 9%, while hyperpigmentation without visual field deficit was observed in 23%, and 68% remained asymptomatic despite radiographic disease progression. NSS required treatment in 14 patients (30%). CONCLUSIONS NSS is a prevalent sequela of CD after bilateral ADX and affects more than 50% of patients. However, although radiological evidence of NSS is common, it is most often clinically indolent, with only a small minority of patients developing the more aggressive disease phenotype characterized by clinically meaningful symptoms and indications for treatment. Young age at the time of CD diagnosis or treatment with bilateral ADX, large tumor size at CD diagnosis, and EBRT are associated with progression to NSS and may be markers of aggressiveness.

Meta-analysis of treatment outcomes of posterior circulation non-saccular aneurysms by flow diverters

Introduction Non-saccular aneurysms of the posterior circulation are uncommon but highly dangerous lesions. Flow diverter stents have been demonstrated to be effective treatments of various anterior circulation aneurysms, particularly large and giant proximal internal carotid artery aneurysms. However, evidence regarding the treatment of non-saccular posterior circulation aneurysms with flow diverters is lacking. Methods A systematic literature review of the English language literature since 2007 was conducted using PubMed, MEDLINE, and Embase. Keywords and MeSH terms included flow diversion, flow diverter, pipeline, surpass, intracranial aneurysm, vertebrobasilar, and non-saccular. Case reports were excluded. Angiographic and clinical outcomes were pooled using a random effects meta-analysis. Results 13 retrospective non-comparative studies reporting 129 patients and 131 aneurysm treatments were included. The average number of flow diverters per aneurysm was 4.33. Immediate complete or near complete occlusion of the aneurysm occurred in 25% (95% CI 1% to 60%), and long term occlusion in 52% (29–76%). Periprocedural stroke occurred in 23% of cases. Good long term neurologic outcome (modified Rankin Scale score ≤2) was achieved in 51% (95% CI 31% to 71%). Overall mortality was 21% (95% CI 7% to 38%) and morbidity was 26% (12%–42%). Retreatment was required in 5% (95% CI 0% to 14%). Good neurologic outcome rate was higher in vertebral artery aneurysms (83%) than other locations (18–33%). Conclusions Flow diversion is a feasible and efficacious treatment for non-saccular aneurysms in the posterior circulation. However, the intervention carries a significant risk of periprocedural stroke, and is still associated with high overall mortality. Further studies are needed to identify significant treatment risk factors and optimize patient selection.

Long-term outcomes of tissue-based ACTH-antibody assay–guided transsphenoidal resection of pituitary adenomas in Cushing disease

OBJECTIVE Cushing disease is caused by a pituitary micro- or macroadenoma that hypersecretes adrenocorticotropic hormone (ACTH), resulting in hypercortisolemia. For decades, transsphenoidal resection (TSR) has been an efficacious treatment but with certain limitations, namely precise tumor localization and complete excision. The authors evaluated the novel use of a double-antibody sandwich assay for the real-time quantitation of ACTH in resected pituitary specimens with the goals of augmenting pathological diagnosis and ultimately improving long-term patient outcome. METHODS This study involved a retrospective review of records and an analysis of assay values, pathology slides, and MRI studies of patients with Cushing disease who had undergone TSR in the period from 2009 to 2014 and had at least 1 year of follow-up in coordination with an endocrinologist. In the operating room, biopsy specimens from the patients had been analyzed for tissue ACTH concentration. Additional samples were simultaneously sent for frozen-section pathological analysis. The ACTH assay performance was compared against pathology assessments of surgical tumor samples using receiver operating characteristic (ROC) analysis and against pre- and postoperative MRI studies. RESULTS Fourteen patients underwent TSR with guidance by ACTH-antibody assay and pathological assessment of 127 biopsy samples and were followed up for an average of 3 years. The ACTH threshold for discriminating adenomatous from normal tissue was 290,000 pg/mg of tissue, based on jointly maximized sensitivity (95.0%) and specificity (71.3%). Lateralization discordance between preoperative MRI studies and surgical visualization was noted in 3 patients, confirming the impression that MRI alone may not achieve optimal localization. A majority of the patients (85.7%) attained long-term disease remission based on urinary free cortisol levels, plasma cortisol levels, and long-term corticosteroid therapy. Comparisons of patient-months of remission and treatment failure showed that the remission rate in the study sample statistically exceeds the rate in historical controls (71.9%; p = 0.0007, Fishers exact test). Long-term unexpected hormonal deficiencies were statistically similar between study patients (29%) and those in a meta-analysis (25%; p = 0.7596, Fishers exact test). CONCLUSIONS These preliminary findings reflect the promising potential of tissue-based ACTH-antibody-guided assay for improving the cure rates of Cushing disease patients undergoing TSR. Further studies with larger sample sizes, further refinements of assay interpretation, and longer-term follow-ups are needed.

Gliosarcoma with Primary Skull Base Invasion

Gliosarcoma is an uncommon variant of glioblastoma, which commonly demonstrates dural attachment. However, skull base invasion is rarely seen with this entity. Herein, we report a 44-year-old female patient diagnosed with primary intracranial gliosarcoma extensively invading the skull base and muscles of mastication. She presented to our institution with a three-month history of difficult right jaw opening and retro-orbital pressure and one week of severe right-sided postauricular headache. Head CT demonstrated a 6 cm mass with marked bony erosion. Brain MRI at a one-week interval more clearly characterized tumor extension through the right orbit and muscles of mastication, with overall growth to 7 cm and worsening midline shift. The patient underwent a right frontotemporal craniotomy for gross total resection. Pathology confirmed the diagnosis of gliosarcoma, IDH-wildtype (WHO grade IV). Her postoperative course was uneventful and she was discharged at preoperative neurologic baseline. To our knowledge, this is the third reported case of a primary intracranial gliosarcoma with direct invasion of skull base, brain parenchyma, and extracranial compartment. However, this is the first report case of primary GS invading the surrounding musculature and orbit. This case report highlights the rapid aggressiveness of gliosarcomas and further a prior undescribed radiographic and anatomic finding of skull base invasion with this entity.

Glossopharyngeal Neuralgia Treatment Outcomes After Nerve Section, Microvascular Decompression, or Stereotactic Radiosurgery: A Systematic Review and Meta-Analysis

BACKGROUND Glossopharyngeal neuralgia (GPN) is a rare neuralgic pain syndrome amenable to neurosurgical treatments, including nerve section (NS), microvascular decompression (MVD), and stereotactic radiosurgery (SRS). However, thorough comparisons of the modalities have not been performed to date. The objective of the present study was to compare the pain and complication outcomes after these approaches to GPN. METHODS Searches of 7 electronic databases from inception to June 2018 were conducted following the appropriate guidelines. The incidence rates (IRs) of short-term (≤3 months) and long-term (≥12 months) pain relief and complications were extracted and analyzed using a meta-analysis. Meta-regression was used to assess for heterogeneity. RESULTS A total of 792 GPN cases managed by NS, MVD, or SRS were described by 6, 11, and 6 studies, reporting outcomes for 282 (36%), 446 (56%), and 67 (8%) cases. The short-term pain relief rate was highest after NS postoperatively (IR, 94%; 95% confidence interval [CI], 88%-98%) and lowest after SRS at 3 months postoperatively (IR, 80%; 95% CI, 68%-96%). The postoperative complication rate was greatest after MVD (IR, 26%; 95% CI, 16%-38%) and lowest after SRS (IR, 0%; 95% CI, 0%-4%). The long-term pain relief rate was greatest after NS (IR, 96%; 95% CI, 91%-99%) and lowest after SRS (IR, 82%; 95% CI, 67%-94%). Statistically significant differences between the approaches were found for each outcome. CONCLUSION Neurosurgical treatment of GPN is frequently performed by 1 of 3 modalities with unique outcomes profiles. NS might provide the most favorable treatment response, with respect to short- and long-term pain relief and postoperative outcomes.

Trigeminal Nerve Schwannoma of the Cerebellopontine Angle

Introduction  Large and even moderate sized, extra-axial cerebellopontine angle (CPA) tumors may fill this restricted space and distort the regional anatomy. It may be difficult to determine even with high resolution magnetic resonance imaging (MRI) if the tumor is dural-based, or what the nerve of origin is if a schwannoma. While clinical history and exam are helpful, they are not unequivocal, particularly since many patients present with a myriad of symptoms, or conversely an incidental finding. We present an atypical appearing, asymptomatic CPA tumor, ultimately identified at surgery to be a trigeminal schwannoma. Case History  A 40-year-old man presented with new-onset seizure. MRI identified an incidental heterogeneously contrast-enhancing CPA lesion ( Fig. 1A – D ). The tumor was centered on the internal auditory canal (IAC) with no tumor extension into Meckels cave, IAC or jugular foramen. Audiometry demonstrated 10db of relative left-sided hearing loss with 100% word recognition. Physical examination was negative for focal neurologic deficits. A retrosigmoid craniotomy was performed and an extra-axial, yellow-hued mass was encountered and resected, which was ultimately confirmed to originate from the trigeminal nerve ( Video 1 ). Gross total resection was achieved, and the patient recovered from surgery with partial ipsilateral trigeminal sensory loss and no other new neurologic deficits. Conclusion  Pure CPA trigeminal schwannomas are rare, but should be considered in the differential for enhancing CPA lesions. Although, Meckels cave involvement is frequently observed, it is not universal, and pure CPA schwannomas of all cranial nerves IV–XII have been reported in the literature. The link to the video can be found at: https://youtu.be/AlodYCu70F8 .