Timing of Chemoradiation in Newly Diagnosed Glioblastoma: Comparative Analysis Between County and Managed Care Health Care Models.

BACKGROUND: Glioblastoma multiforme (GBM) is a primary brain malignancy with significant morbidity and mortality. The current standard of treatment for GBM is surgery followed by radiotherapy and temozolomide. Despite an established treatment protocol, there exists heterogeneity in outcomes due to patients not receiving all treatments. We analyzed patients in different health care models to investigate this heterogeneity. METHODS: A retrospective analysis was performed at 2 hospitals in San Bernardino County, California, for patients with newly diagnosed GBM from 2004 to 2019. Patients younger than 18 years of age, with history of low-grade glioma, who had undergone prior treatment, and those lost to follow-up were excluded. RESULTS: A total of 57 patients were included in our study. Chemotherapy was started at 41 ± 30 and 77 ± 68 days in the health maintenance organization (HMO) and county model, respectively (P = 0.050); radiation therapy was started at 46 ± 34 and 85 ± 76 days in the HMO and county models, respectively (P = 0.036). In individuals who underwent both chemotherapy and radiation therapy (XRT), the difference in time to XRT was no longer significant (P = 0.060). Recurrence time was 309 ± 263 and 212 ± 180 days in the HMO and county groups, respectively (P = 0.379). The time to death was 412 ± 285 and 343 ± 304 days for HMO and county models, respectively (P = 0.334). CONCLUSIONS: Our study demonstrates a statistically significant difference in time to adjuvant therapies between patients within a county hospital and a managed health care organization. This information has the potential to inform future policies and care coordination for patients within the county model.

Impact of Chronic DMARD Therapy in Patients With Rheumatoid Arthritis Undergoing Surgery of the Craniovertebral Junction: A Multi-center Retrospective Study.

STUDY DESIGN: A multi-centered retrospective review from five institutions. OBJECTIVE: The aim of this study was to determine whether continuing or withholding disease-modifying antirheumatoid drugs (DMARDs) in the perioperative period affect outcomes in rheumatoid arthritis (RA) patients undergoing arthrodesis at the craniovertebral junction SUMMARY OF BACKGROUND DATA.: RA is a chronic systemic inflammatory disease that affects the cervical spine and is treated with DMARDs. Some advocate withholding DMARDs in the perioperative period due to concern for the cytotoxic effects of these medications. However, the impact of DMARDs in the perioperative period is not well understood. METHODS: A multicenter retrospective study from five affiliated institutions was performed. Adult patients with RA on chronic DMARDs undergoing posterior arthrodesis of the craniovertebral junction (occipital-cervical or atlanto-axial arthrodesis) were identified. Patients were stratified based on whether DMARD therapy was continued (C group) or discontinued (DC group) in the perioperative period. The primary outcome was the need for reoperation and reason for reoperation. RESULTS: Thirty-nine patients met inclusion criteria, 19 in C group and 20 in DC group. Average follow-up time was 42 months. Four patients (three in DC group and one in C group) required reoperation. Two patients from the DC group required readmission secondary to RA flare-up. CONCLUSION: Our cohort of RA patients who underwent occipital-cervical and C1/C2 posterior arthrodesis showed no significant differences in surgical complications when DMARD therapy was continued or discontinued in the perioperative period. The decision to continue or discontinue DMARD therapy in the perioperative period is at the discretion of the treating physician, but we encourage physicians to counsel patients regarding this theoretical risk and their tolerance of the medications as well as the risk of RA flare-up. Factors such as overall health, disease burden, nutrition, bone quality, smoking status, and other comorbid conditions are likely to have a larger influence on perioperative complications. LEVEL OF EVIDENCE: 3.

Disseminated intravascular coagulation in spine surgery: illustrative case review and the paradigms of management.

Disseminated intravascular coagulation (DIC) is rarely encountered by spine surgeons outside of deformity or severe trauma cases. The authors report an extraordinarily unique case of refractory DIC after elective resection of multiple en plaque thoracic meningiomas in a patient with neurofibromatosis type 1. A 49-year-old man underwent T1-3 laminoplasty and expansile duraplasty for resection of multiple en plaque meningiomas for thoracic myelopathy. Intraoperatively, the patient was found to be in a state of DIC that did not resolve postoperatively despite massive transfusions of blood products. He required subsequent returns to the operating room due to recurrent epidural hematomas with resulting paraplegia. Ultimately, the wound was left open, and a wound vacuum-assisted closure (VAC) was placed to prevent further returns to the operating room. DIC persisted until the administration of recombinant factor VIIa. In this report, the authors review the mechanisms, subtypes, and approaches to treatment of DIC with a focus on the bleeding subtype. If this subtype is refractory to blood product administration (> 24 hours), recombinant factor VIIa is a safe and effective option. A wound VAC can be safely utilized with exposed dura if deemed necessary by the surgeon; however, the volume and characteristics of the output should be closely monitored. The use of unconventional surgical solutions may provide options to mitigate the morbidity associated with refractory DIC in spine surgery.

Virtual reality for management of pain in hospitalized patients: A randomized comparative effectiveness trial.

OBJECTIVES: Therapeutic virtual reality (VR) has emerged as an effective, drug-free tool for pain management, but there is a lack of randomized, controlled data evaluating its effectiveness in hospitalized patients. We sought to measure the impact of on-demand VR versus "health and wellness" television programming for pain in hospitalized patients. METHODS: We performed a prospective, randomized, comparative effectiveness trial in hospitalized patients with an average pain score of ≥3 out of 10 points. Patients in the experimental group received a library of 21 VR experiences administered using the Samsung Gear Oculus headset; control patients viewed specialized television programming to promote health and wellness. Clinical staff followed usual care; study interventions were not protocolized. The primary outcome was patient-reported pain using a numeric rating scale, as recorded by nursing staff during usual care. Pre- and post-intervention pain scores were compared immediately after initial treatment and after 48- and 72-hours. RESULTS: There were 120 subjects (61 VR; 59 control). The mean within-subject difference in immediate pre- and post-intervention pain scores was larger in the VR group (-1.72 points; SD 3.56) than in the control group (-0.46 points; SD 3.01); this difference was significant in favor of VR (P < .04). When limited to the subgroup of patients with severe baseline pain (≥7 points), the effect of VR was more pronounced vs. control (-3.04, SD 3.75 vs. -0.93, SD 2.16 points; P = .02). In regression analyses adjusting for pre-intervention pain, time, age, gender, and type of pain, VR yielded a .59 (P = .03) and .56 (P = .04) point incremental reduction in pain versus control during the 48- and 72-hour post-intervention periods, respectively. CONCLUSIONS: VR significantly reduces pain versus an active control condition in hospitalized patients. VR is most effective for severe pain. Future trials should evaluate standardized order sets that interpose VR as an early non-drug option for analgesia.

Declining Rates of Referral for Irritable Bowel Syndrome Without Constipation at a Tertiary Care Center.

BACKGROUND: Irritable bowel syndrome (IBS) is a common chronic disorder of the gastrointestinal tract. Several treatments have been developed, including rifaximin for the treatment of IBS without constipation (non-IBS-C), but no studies have evaluated the effect of these therapies on patient referral rates to tertiary care gastroenterology clinics. AIM: To assess referral patterns for IBS patients at a tertiary motility clinic over a 10-year period. METHODS: Data from consecutive patients referred to the clinic during 2006-2016 were analyzed. Trends in the proportion of referrals and prior rifaximin use in IBS-C versus non-IBS-C groups were compared. RESULTS: A total of 814 adult patients were referred to a single physician panel for IBS-related symptoms. Of these, 776 were included in the study [528 females (68%), average age 45.7 ± 15.9 years), comprising 431 IBS-C (55.5%) and 345 non-IBS-C (44.5%) patients. The proportion of non-IBS-C referrals declined significantly from 53.0% in 2006 to 27.3% in 2016 (Chi-square, p < 0.0001, Cochran-Armitage trend test p = 0.0001), and the proportion of IBS-C referrals increased significantly from 46.9% in 2006 to 72.7% in 2016 (Chi-square, p < 0.0001, Cochran-Armitage trend test p = 0.0004). Non-IBS-C referrals with prior rifaximin use significantly increased from 22.7% in 2006 to 66.7% in 2016 (Cochran-Armitage trend test, p = 0.008). CONCLUSIONS: The results indicate a significantly declining tertiary care referral rate for non-IBS-C over the past decade. While not directly linked, there has been an increase in rifaximin use in the same population during the same time interval.

Impact of remote patient monitoring on clinical outcomes: an updated meta-analysis of randomized controlled trials.

Despite growing interest in remote patient monitoring, limited evidence exists to substantiate claims of its ability to improve outcomes. Our aim was to evaluate randomized controlled trials (RCTs) that assess the effects of using wearable biosensors (e.g. activity trackers) for remote patient monitoring on clinical outcomes. We expanded upon prior reviews by assessing effectiveness across indications and presenting quantitative summary data. We searched for articles from January 2000 to October 2016 in PubMed, reviewed 4,348 titles, selected 777 for abstract review, and 64 for full text review. A total of 27 RCTs from 13 different countries focused on a range of clinical outcomes and were retained for final analysis; of these, we identified 16 high-quality studies. We estimated a difference-in-differences random effects meta-analysis on select outcomes. We weighted the studies by sample size and used 95% confidence intervals (CI) around point estimates. Difference-in-difference point estimation revealed no statistically significant impact of remote patient monitoring on any of six reported clinical outcomes, including body mass index (-0.73; 95% CI: -1.84, 0.38), weight (-1.29; -3.06, 0.48), waist circumference (-2.41; -5.16, 0.34), body fat percentage (0.11; -1.56, 1.34), systolic blood pressure (-2.62; -5.31, 0.06), and diastolic blood pressure (-0.99; -2.73, 0.74). Studies were highly heterogeneous in their design, device type, and outcomes. Interventions based on health behavior models and personalized coaching were most successful. We found substantial gaps in the evidence base that should be considered before implementation of remote patient monitoring in the clinical setting.

Erratum: Author Correction: Impact of remote patient monitoring on clinical outcomes: an updated meta-analysis of randomized controlled trials.

[This corrects the article DOI: 10.1038/s41746-017-0002-4.].

Resolution of Tachyarrhythmia Following Posterior Fossa Decompression Surgery for Chiari Malformation Type I.

INTRODUCTION: Chiari malformation (CM) type I commonly presents with symptoms such as tussive headaches, paresthesias, and, in severe cases, corticobulbar dysfunction. However, patients may present with atypical symptoms lending to the complexity in this patient population. We present a case of a CM patient presenting with atypical cardiac symptoms and arrhythmias, all of which resolved after surgical decompression. CASE DESCRIPTION: A 31-year-old female presented with atypical chest pain, palpitations, tachycardia, headaches, and dizziness for 2 years. Multiple antiarrhythmics and ultimately cardiac ablation procedure proved to be ineffective. Magnetic resonance imaging revealed CM, and the patient ultimately underwent surgical decompression with subsequent resolution of her symptoms. CONCLUSION: The surgical management of CM patients presenting with atypical symptoms can be challenging and often lead to delays in intervention. To our knowledge this is the only reported case of a patient presenting with tachyarrhythmia and atypical chest pain with resolution after Chiari decompression. We believe the dramatic improvement documented in the present case should serve to advance Chiari decompression in CM patients presenting with refractory tachyarrhythmia in whom no other discernable cause has been elucidated. Further studies are needed to better correlate the findings and to hopefully establish a criteria for patients that will likely benefit from surgical decompression.

Virtual Reality for Management of Pain in Hospitalized Patients: Results of a Controlled Trial.

BACKGROUND: Improvements in software and design and reduction in cost have made virtual reality (VR) a practical tool for immersive, three-dimensional (3D), multisensory experiences that distract patients from painful stimuli. OBJECTIVE: The objective of the study was to measure the impact of a onetime 3D VR intervention versus a two-dimensional (2D) distraction video for pain in hospitalized patients. METHODS: We conducted a comparative cohort study in a large, urban teaching hospital in medical inpatients with an average pain score of ≥3/10 from any cause. Patients with nausea, vomiting, dementia, motion sickness, stroke, seizure, and epilepsy and those placed in isolation were excluded. Patients in the intervention cohort viewed a 3D VR experience designed to reduce pain using the Samsung Gear Oculus VR headset; control patients viewed a high-definition, 2D nature video on a 14-inch bedside screen. Pre- and postintervention pain scores were recorded. Difference-in-difference scores and the proportion achieving a half standard deviation pain response were compared between groups. RESULTS: There were 50 subjects per cohort (N=100). The mean pain reduction in the VR cohort was greater than in controls (-1.3 vs -0.6 points, respectively; P=.008). A total of 35 (65%) patients in the VR cohort achieved a pain response versus 40% of controls (P=.01; number needed to treat=4). No adverse events were reported from VR. CONCLUSIONS: Use of VR in hospitalized patients significantly reduces pain versus a control distraction condition. These results indicate that VR is an effective and safe adjunctive therapy for pain management in the acute inpatient setting; future randomized trials should confirm benefit with different visualizations and exposure periods. TRIAL REGISTRATION: Clinicaltrials.gov NCT02456987; https://clinicaltrials.gov/ct2/show/NCT02456987 (Archived by WebCite at http://www.webcitation.org/6pJ1P644S).

Chondromyxoid fibroma of the sacrum: A case report and literature review.

BACKGROUND: Chondromyxoid fibroma (CMF) is an extremely rare, benign cartilaginous tumor that makes up <0.5% of all bone tumors, typically presenting in the second or third decade of life. CMF of the sacrum is exceedingly rare, with only seven documented cases reported in the neurosurgical literature. CASE DESCRIPTION: We report a case of a 35-year-old female with a 3 month history of lower back pain after sustaining a fall on her sacrum/coccyx presenting with a progressive complaint of localized lower back pain, occasional urinary retention without incontinence, gluteal hypesthesia, and pressure below the gluteal crease. Imaging demonstrated a large, expansile enhancing soft-tissue lesion involving the sacrum, distal to the S2-3 disc space. The tumor was removed with partial sacrectomy for open en bloc resection with partial nerve sparing. The patient was found at 1.5-year follow-up with the improvement of symptoms, no recurrence, and no residual neurologic dysfunction. CONCLUSION: Sacral CMF is a rare clinical entity that may mirror more aggressive sacral pathology, including chordoma, in both clinical presentation and imaging characteristics. A review of the available literature regarding diagnosis, surgical management options, and prognosis for sacral CMF is provided.

Preventing patient absenteeism: validation of a predictive overbooking model.

OBJECTIVES: To develop a model that identifies patients at high risk for missing scheduled appointments ("no-shows" and cancellations) and to project the impact of predictive overbooking in a gastrointestinal endoscopy clinic-an exemplar resource-intensive environment with a high no-show rate. STUDY DESIGN: We retrospectively developed an algorithm that uses electronic health record (EHR) data to identify patients who do not show up to their appointments. Next, we prospectively validated the algorithm at a Veterans Administration healthcare network clinic. METHODS: We constructed a multivariable logistic regression model that assigned a no-show risk score optimized by receiver operating characteristic curve analysis. Based on these scores, we created a calendar of projected open slots to offer to patients and compared the daily performance of predictive overbooking with fixed overbooking and typical "1 patient, 1 slot" scheduling. RESULTS: Data from 1392 patients identified several predictors of no-show, including previous absenteeism, comorbid disease burden, and current diagnoses of mood and substance use disorders. The model correctly classified most patients during the development (area under the curve [AUC] = 0.80) and validation phases (AUC = 0.75). Prospective testing in 1197 patients found that predictive overbooking averaged 0.51 unused appointments per day versus 6.18 for typical booking (difference = -5.67; 95% CI, -6.48 to -4.87; P < .0001). Predictive overbooking could have increased service utilization from 62% to 97% of capacity, with only rare clinic overflows. CONCLUSIONS: Information from EHRs can accurately predict whether patients will no-show. This method can be used to overbook appointments, thereby maximizing service utilization while staying within clinic capacity.

Validation of an acoustic gastrointestinal surveillance biosensor for postoperative ileus.

BACKGROUND: Postoperative ileus (POI) can worsen outcomes, increase cost, and prolong hospitalization. An objective marker could help identify POI patients who should not be prematurely fed. We developed a disposable, non-invasive acoustic gastro-intestinal surveillance (AGIS) biosensor. We tested whether AGIS can distinguish healthy controls from patients recovering from abdominal surgery. STUDY DESIGN: AGIS is a disposable plastic device embedded with a microphone that adheres to the abdominal wall and connects to a computer that measures acoustic event rates. We compared intestinal rates of healthy subjects using AGIS for 60 min after a standardized meal to recordings of two postoperative groups: (1) patients tolerating standardized feeding and (2) POI patients. We compared intestinal rates among groups using ANOVA and t tests. RESULTS: There were 8 healthy controls, 7 patients tolerating feeding, and 25 with POI; mean intestinal rates were 0.14, 0.03, and 0.016 events per second, respectively (ANOVA p < 0.001). AGIS separated patients from controls with 100 % sensitivity and 97 % specificity. Among patients, rates were higher in fed versus POI subjects (p = 0.017). CONCLUSION: Non-invasive, abdominal acoustic monitoring distinguishes POI from non-POI subjects. Future research will test whether AGIS can identify patients at risk for development of POI and assist with postoperative feeding decisions.

Minimally invasive extracavitary approach for thoracic discectomy and interbody fusion: 1-year clinical and radiographic outcomes in 13 patients compared with a cohort of traditional anterior transthoracic approaches.

OBJECT: Open transthoracic approaches, considered the standard in treating thoracic disc herniation (TDH), are associated with significant comorbidities. The authors describe a minimally invasive lateral extracavitary tubular approach for discectomy and fusion (MIECTDF) to treat TDH. METHODS: In 13 patients (5 men, 8 women; mean age 51.8 years) with myelopathy and 15 noncalcified TDHs, the authors achieved a far-lateral trajectory by dilating percutaneously to a 20-mm working portal docked at the transverse process-facet junction, which then provided a corridor for a near-total discectomy, bilateral laminotomies, and interbody arthrodesis requiring minimal cord retraction. A cohort of 11 demographically comparable patients treated via transthoracic approaches was used as control. RESULTS: Preoperative Frankel grades were B in 1 patient, C in 4, D in 5, and E in 3, whereas at mean of 10 months, 11 had Grade E function and 2 had Grade D function. Mean surgical metrics were operating room time 93.75 minutes, blood loss 33 ml, and hospital stay 3.1 days. Complications included 4 transient paresthesias, 1 CSF leak, 1 abdominal wall weakness, and 3 nonwound infections. One-year follow-up MR imaging revealed full decompression in all cases and no cage migration. Mean visual analog scales scores preoperative, at 6 weeks, 3 months, and 1 year were 5.6, 4.5, 3.2, and 1.2, respectively. No differences existed in preoperative clinical and radiographic profile of the study and control groups. Compared with controls, the MIECTDF group achieved superior scores in all metrics (p < 0.01) except for equivalent 1-year neurological outcomes. CONCLUSIONS: Compared with transthoracic procedures, MIECTDF effectively decompressed the spinal canal, yielding identical 1-year radiographic and clinical outcomes to those seen in controls, while producing superior clinical scores in the interim. Thus, MIECTDF is the authors' treatment of choice for TDH.

The relationship between preoperative cervical alignment and postoperative spinal cord drift after decompressive laminectomy and arthrodesis for cervical spondylotic myelopathy.

BACKGROUND: Cervical spondylotic myelopathy represents a debilitating disorder, often resulting in significant neurological impairment over time. Cervical laminectomy has enjoyed a successful track record in the surgical management of these patients. Little is understood regarding the significance of postdecompressive migration of the spinal cord in relation to patient outcome. METHODS: Preoperative and postoperative cervical spine MRIs of 28 patients who underwent cervical laminectomy and fusion for the treatment of CSM were reviewed. Radiographic parameters including preoperative cervical alignment, LDI, space available at the level cepahlad/caudad to the decompression, percent spinal cord expansion at the radiographically most compressed level, and spinal cord drift to the midpoint of the spinal cord were measured and subsequently analyzed for statistical correlation. The recovery rate based on the mJOA score was calculated for each patient and analyzed for correlation with spinal cord drift. RESULTS: The Cobb angle C2-7, cervical spinal angle, and CCI represented tightly correlated measures of cervical alignment. The preoperative cervical alignment did not statistically correlate with postoperative spinal cord drift. No statistical correlation was revealed between postdecompressive spinal cord drift and recovery rate. CONCLUSIONS: Preoperative cervical alignment does not statistically correlate with postoperative spinal cord drift in patients undergoing multisegmental decompressive laminectomy and fusion for CSM. The observation of significant posterior shifting of the spinal cord in the context of straight or kyphotic preoperative alignment suggests that posterior decompression and arthrodesis represent a viable option in the surgical management of patients with CSM with nonlordotic preoperative alignment.

Hemangiopericytoma of the cerebellopontine angle: a case report and review of the literature.

BACKGROUND: Intracranial hemangiopericytoma represents a rare intracranial tumor that is typically difficult to distinguish from meningioma based on clinical presentation and radiographic findings. These inherently aggressive neoplasms have been observed to occur in numerous intracranial compartments; however, isolated involvement of the CPA is essentially unreported. The authors present a case of a young lady with presumed right acoustic schwannoma, which proved to be HPC on histopathology. The case is described; and a review of the literature pertaining to the diagnosis, optimal management, and follow-up for these lesions is provided. CASE DESCRIPTION: A 37-year-old Asian woman presented with a 7-month history of right ear and mandible numbness, as well as subjective hearing loss involving the right ear. Magnetic resonance imaging demonstrated the presence of a homogeneously enhancing extraaxial lesion in the right CPA, radiographically suggestive of an acoustic schwannoma. The lesion proved to be an intracranial HPC on histologic sections. Review of the neurosurgical literature yielded only one prior detailed account of HPC confined to the CPA. The patient underwent right retrosigmoid craniotomy for gross total resection of the mass, followed by stereotactic radiotherapy several weeks postoperatively. CONCLUSION: Given the fundamentally different treatment approach for HPCs over other more common CPA tumors, it is imperative that the treating surgeon consider this rare diagnosis when evaluating patients with lesions localized to this area. Specifically, gross total resection, followed by adjuvant SRT, provides patients with the highest probability for disease-free survival, based on current evidence in the neurosurgical literature.

JIP1 regulates neuronal apoptosis in response to stress.

We examined if the relative expression of JNK-interacting protein 1 (JIP1) and phosphorylated c-Jun N-terminal kinase (JNK) regulates cell signaling and contributes to selective neuronal vulnerability in response to environmental stress. In clonal neuroblastoma cultures, stresses such as hypoxia, ischemia, Abeta peptides, and UV irradiation rapidly reduced JIP1 expression. JIP1 mRNA expression was also down-regulated by UV stress and was accompanied by increased JNK and c-Jun activation and cell death. JIP1 protein reduction was partially reversed both by inhibitors predominantly of caspase 3 and of the JNK pathway and resulted in significantly increased cell survival. Conversely, overexpression of JIP1 decreased both nuclear translocation of activated-JNK, and c-Jun phosphorylation induced by either UV irradiation, or the JNK upstream activators, MKK7 or MEKK1. Cell death was reduced about 50% compared to GFP-transfected controls. JIP1 overexpression did not facilitate either JNK expression or activation. In the normal, non-stressed human hippocampus and rat hippocampal organotypic cultures, JIP1 and JNK3 were inversely expressed with more JIP1 in CA2 and CA3 and less in CA1 neurons. In the human hippocampus, transient hypoxia/ischemia selectively spares neurons in CA2 and CA3 and induces death of neurons in the hippocampal CA1 subregion. In the cultures, ischemia reduced JIP1 expression and activated JNK, c-Jun, and caspase 3. Inhibitors of the JNK pathway, JNK activation directly and of caspase 3 activation each partially reversed these effects. Thus, under certain stress conditions, down-regulation of JIP1 expression makes neurons more susceptible to apoptosis, suggesting JIP may serve as an anti-apoptosis factor.

Diagnosis of Erdheim-Chester disease by using computerized tomography-guided stereotactic biopsy of a caudate lesion. Case report.

The authors present the case of a 27-year-old woman with Erdheim-Chester disease (ECD) and extensive intracranial involvement, in whom the initial diagnosis of ECD was established based on computerized tomography (CT)-guided stereotactic biopsy of a caudate lesion. Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis of unknown origin that is clinically characterized by bone pain, diabetes insipidus, and exophthalmos. The radiological hallmarks of the disease include symmetrical sclerosis of the long bones with epiphysial sparing and increased tracer uptake in lesions seen on scintigraphic imaging. Erdheim-Chester disease is characterized histologically by the presence of infiltrating lipid-laden histiocytes that commonly involve the retroperitoneum, orbits, skin, pericardium, lungs, and long bones. Although the occurrence of diabetes insipidus often precedes the diagnosis of ECD by more than a decade in most patients, magnetic resonance imaging- and CT-documented central nervous system involvement is exceedingly rare. In the setting of neurological involvement, neurosurgical biopsy has been reported seven times in the literature, with only one of these biopsies being the basis for the initial diagnosis of the disease. The authors' case represents only the second time the disease has been diagnosed by means of neurosurgical biopsy, highlighting the diagnostic difficulties that patients with EDC present. Skeletal radiographs were confirmatory in this case and this modality should be emphasized as the simplest and most direct route to the diagnosis. The degree of neurological involvement further distinguishes the case presented from prior reports in the literature. The multiple bilateral intraaxial lesions were intensely enhancing on contrast CT scans, distributed infra- and supratentorially, involving both white and gray matter, and associated with diffuse cerebral edema. The case presented is also remarkable by virtue of the symmetrical involvement of the caudate nuclei, representing the first such example documented in the literature. The diagnosis, treatment, and outcome in this patient are discussed, and a review of the literature is presented.