Causalgia: a military pain syndrome.

Causalgia, officially known as complex regional pain syndrome type II, is a pain syndrome characterized by severe burning pain, motor and sensory dysfunction, and changes in skin color and temperature sensation distal to an injured peripheral nerve. The pain syndrome primarily tends to affect combat soldiers after they sustain wartime injuries from blasts and gunshots. Here, the authors provide a historical narrative that showcases the critical contributions of military physicians to our understanding of causalgia and to the field of peripheral nerve neurosurgery as a whole.

Giant chondrosarcoma of the falx in an adolescent: A case report.

BACKGROUND: Intracranial chondrosarcomas are slowly growing malignant cartilaginous tumors that are especially rare in adolescents. CASE DESCRIPTION: A 19-year-old woman with no medical history presented with symptoms of intermittent facial twitching and progressive generalized weakness for 6 months. The patient's physical examination was unremarkable. Imaging revealed a large bifrontal mass arising from the falx cerebri, with significant compression of both cerebral hemispheres and downward displacement of the corpus callosum. The patient underwent a bifrontal craniotomy for gross total resection of tumor. Neuropathologic examination revealed a bland cartilaginous lesion most consistent with low-grade chondrosarcoma. Her postoperative course was uneventful, and she was discharged to home on postoperative day 3. CONCLUSION: This is an unusual case of an extra-axial, non-skull base, low-grade chondrosarcoma presenting as facial spasm in an adolescent patient.

Guidelines in the management of CNS tumors.

INTRODUCTION: Evidence-based, clinical practice guidelines in the management of central nervous system tumors (CNS) continue to be developed and updated through the work of the Joint Section on Tumors of the Congress of Neurological Surgeons (CNS) and the American Association of Neurological Surgeons (AANS). METHODS: The guidelines are created using the most current and clinically relevant evidence using systematic methodologies, which classify available data and provide recommendations for clinical practice. CONCLUSION: This update summarizes the Tumor Section Guidelines developed over the last five years for non-functioning pituitary adenomas, low grade gliomas, vestibular schwannomas, and metastatic brain tumors.

Spinal cord injury in the United States Army Special Forces.

OBJECTIVE: Spinal cord injury (SCI) is an area of key interest in military medicine but has not been studied among the US Army Special Forces (SF), the most elite group of US soldiers. SF soldiers make up a disproportionate 60% of all Special Operations casualties. The objective of this study was to better understand SCI incidence in the SF, its mechanisms of acquisition, and potential areas for intervention by addressing key issues pertaining to protective equipment and body armor use. METHODS: An electronic survey questionnaire was formulated with the close collaboration of US board-certified neurosurgeons from the Mount Sinai Hospital and Cleveland Clinic Departments of Neurosurgery, retired military personnel of the SF, and operational staff of the Green Beret Foundation. The survey was sent to approximately 6000 SF soldiers to understand SCI diagnosis and its associations with various health and military variables. RESULTS: The response rate was 8.2%. Among the 492 respondents, 94 (19.1%) self-reported an SCI diagnosis. An airborne operation was the most commonly attributed cause (54.8%). Moreover, 87.1% of SF soldiers reported wearing headgear at the time of injury, but only 36.6% reported wearing body armor, even though body armor use has significantly increased in post-9/11 SF soldiers compared with that in their pre-9/11 counterparts. SCI was significantly associated with traumatic brain injury, arthritis, low sperm count, low testosterone, erectile dysfunction, tinnitus, hyperacusis, sleep apnea, posttraumatic stress disorder, major depressive disorder, and generalized anxiety disorder. Only 16.5% of SF soldiers diagnosed with SCI had been rescued via medical evacuation (medevac) for treatment. CONCLUSIONS: A high number of SF soldiers self-reported an SCI diagnosis. Airborne operations landings were the leading cause of SCI, which coincided with warfare tactics employed during the Persian Gulf War, Operation Iraqi Freedom, and other conflicts. A majority of SCIs occurred while wearing headgear and no body armor, suggesting the need for improvements in protective equipment use and design. The low rate of medevac rescue for these injuries may suggest that medical rescue was not attainable at the time or that certain SCIs were deemed minor at the time of injury.

Neurosurgical management of brain and spine tumors in the COVID-19 era: an institutional experience from the epicenter of the pandemic.

The challenges of neurosurgical patient management and surgical decision-making during the 2019-2020 COVID-19 worldwide pandemic are immense and never-before-seen in our generation of neurosurgeons. In this case-based formatted report, we present the Mount Sinai Hospital (New York, NY) Department of Neurosurgery institutional experience in the epicenter of the pandemic and the guiding principles for our current management of intracranial, skull base, and spine tumors. The detailed explanations of our surgical reasoning for each tumor case is tailored to assist neurosurgeons across the United States as they face these complex operative decisions put forth by the realities of the pandemic.

Patents and Innovation Among Neurosurgeons from the American Association of Neurological Surgeons.

Objective Neurosurgeons have taken on the role of innovators, continuing to move the field forward over the centuries. More recently, innovation has taken the form of new technological devices and therapeutics, which require patenting. The aim of this study is to identify major areas of innovation in the field of neurosurgery by evaluating patent records. Methods This study quantifies the number of patents the American Association of Neurological Surgeons (AANS) neurosurgeons hold across different subspecialties. The United States Patent and Trademark Office (USPTO) patent database was queried using the names of 7,293 AANS members who filed patents between 1976 and 2019. Results A total of 346 (4.7%) AANS neurosurgeons hold a total of 1,025 patents. The number of patents held by each neurosurgeon ranged from one to 109. The areas that patents were filed under include cellular and genetic science (40), drug delivery (45), image guidance (82), neuromodulation (52), pain (7), peripheral nerve stimulation (24), spine (398), surgical devices (148), trauma (16), tumor (78), vascular (67), and other (68). No patents were filed under pediatrics (0). The fields with the greatest number of filed patents are spine, instruments/devices, and image guidance. Conclusion Given the technical nature of the field of neurosurgery, instruments and devices that improve localization, visualization, targeting, and spinal reconstruction are often in demand. Furthermore, since the rates of spinal procedures and implants continue to increase, higher patenting may be motivated by the opportunity to develop new products that can result in royalty payments to neurosurgeons. The advent of new technologies undoubtedly continues to push the field of neurosurgery forward.

Lung adenocarcinoma presumed to be Pott's disease in a 28-year-old patient: A case report and review of literature.

BACKGROUND: Tuberculous spondylitis (Pott's disease), a common extrapulmonary manifestation of tuberculosis (TB), typically presents with back pain, tenderness, paraparesis/paraplegia, and various constitutional symptoms. Due to radiological similarities between Pott's disease and lung cancer, some lung cancer patients may initially be erroneously diagnosed and treated for TB, allowing for extensive progression of their cancer. CASE DESCRIPTION: A 28-year-old male presented with a chronic dry cough, weight loss, and 2 months of increased back pain accompanied by bilateral lower extremity weakness. Magnetic resonance imaging revealed an epidural collection causing compression of the spinal cord at the T5-T6 level. The initial diagnosis was Mycobacterium tuberculosis/Pott's disease. A thoracic T4-T8 decompression fusion was performed; however, pathologic examination of the tissue revealed adenocarcinoma. Postoperatively, after the patient experienced several episodes of acute respiratory distress and a tension pneumothorax, lung imaging confirmed multiple tumor infiltrates along with lung cancer extending into the thoracic vertebrae. Pelvic studies also confirmed the presence of pelvic metastases. The patient passed away 3 weeks following surgery. CONCLUSION: In this case report, a 28-year-old male was treated for thoracic Pott's disease that proved to be metastatic lung adenocarcinoma. To avoid such misdiagnoses in the future, physicians should better differentiate spinal TB from other malignancies that may affect the spine. This study underscores the importance of obtaining at least a chest X-ray in any patient with suspected Pott's disease, irrespective of age, to help rule out lung cancer or other pathologies.

Initial biopsy and early re-resection practices in the treatment of glioblastoma among AANS/CNS tumor section surgeons.

INTRODUCTION: Surgical management strategies for glioblastoma (GBM) may differ among neurosurgeons with initial biopsy of suspected tumors and the need for early re-resection of tumors within 30 days of initial surgery. This study was initiated by the American Association of Neurological Surgeons (AANS) and Congress of Neurological Surgeons (CNS) Tumor Section's interest in understanding the rates at which pre- and post-resection procedures, specifically biopsies prior to definitive resection and early re-resections, are performed by U.S. neurosurgeons in the management of GBM. METHODS: A ten-question survey was distributed to members of the AANS/CNS Tumor Section. RESULTS: The survey response rate among AANS/CNS Tumor Section surgeons was approximately 16%. Results showed that a majority of respondents performed surgery on 11-25 GBM cases annually. Of those cases, most neurosurgeons claimed that biopsies are rarely performed prior to tumor resection, but in the < 10% of cases for which biopsies are done, common reasons are to confirm radiological findings or improve the treatment plan. Likewise, re-resections are rare, but in the < 5 cases most neurosurgeons performed annually, common reasons included incomplete initial resections, referrals for greater resection, or unspecified reasons. CONCLUSIONS: Further studies are needed to confirm the results of this study, which shows low rates of stereotactic and open biopsy and early re-resection procedures performed among neurosurgeons. These rates may help form guidelines in the treatment of GBM and encourage the use of surgical adjuncts that increase the extent of resection of these tumors, thereby reducing rates of early recurrence.

Snapshot: Socioeconomic Competence in US Neurosurgery Residents.

INTRODUCTION: Socioeconomic topics such as federal mandates/regulations, conflict of interest, and practice management have become increasingly important for all neurosurgeons. Graduating residents immediately need a host of skills to successfully navigate neurosurgical practice. Surgical and medical skills are closely evaluated through the American Board of Neurological Surgery, and a formal socioeconomic curriculum has been developed with defined milestones. Nevertheless, little has been done to evaluate neurosurgery resident competence in socioeconomic and medicolegal principles. The purpose of this study was to assess the competence of Accreditation Council for Graduate Medical Education neurosurgical residents in socioeconomic knowledge. METHODS: Neurosurgery resident members of the American Association of Neurological Surgeons (N = 1385) were sent a Survey Monkey of 10 questions. The survey covered the most basic of socioeconomic principles. Initial survey responses were collected across a 1-month period from April to May 2018. RESULTS: The response rate was 14% (194/1385). Overall, neurosurgery residents would have received a grade of D, with an average score of 67% on the survey. For 7 of the 10 questions, the majority (>50%) of neurosurgery residents answered correctly. Furthermore, for 3 questions, more than 90% of residents selected the correct answer. However, for one-half of all questions, residents averaged a score of less than 65%. Residents tended to answer questions correctly for physician compensation and compensation models, but incorrectly for topics of informed consent, Controlled Substances Act, and conflicts of interest. CONCLUSION: With the increasing complexity of neurosurgery practice, solid knowledge of socioeconomic topics is essential. The study confirms suspected deficiencies in socioeconomic proficiency among neurosurgery residents, despite the availability of a validated curriculum. This knowledge gap will likely affect career success and satisfaction. Nevertheless, this survey had a significantly low response rate, and it may be an incomplete representation of the neurosurgical resident mind. Focused educational initiatives through the neurosurgical Residency Review Committee and individual training programs must facilitate an action plan that ensures the effective implementation of socioeconomic curricula.

Utilization of the Nasoseptal Flap for Repair of Cerebrospinal Fluid Leak after Endoscopic Endonasal Approach for Resection of Pituitary Tumors.

BACKGROUND: One of the most frequent complications after endoscopic endonasal approach (EEA) for resection of pituitary tumors is cerebrospinal fluid (CSF) leaks. With the introduction of the pedicled nasoseptal flap, the reconstruction of the skull base has improved significantly resulting in a decrease in the occurrence of persistent CSF leaks. We present our experience utilizing the pedicled nasoseptal flap technique after EEA for reconstruction of the skull base in cases where CSF leak was detected. METHODS: Data for patients undergoing EEA for pituitary tumors was retrospectively reviewed. These included demographic, clinical, operative, radiographic, and pathological information. Incidence of post-operative complications and CSF leaks were recorded. Descriptive statistical analysis was performed. RESULTS: Between 2008 and 2015, 67 patients and 69 hospital admissions with pituitary tumors underwent a nasoseptal flap to reconstruct a skull base defect at Johns Hopkins Hospital. The mean age at surgery was 54.5±14.2 years. Fifty-two percent of patients were male. Forty-six percent of patients were white, 33% African-American, and 12% belonged to other racial groups. There was an intraoperative CSF leak in 39% of patients. Seventy percent of patients with an intraoperative CSF leak had a nasoseptal flap reconstruction of the skull base. There were zero postoperative CSF leaks. CONCLUSION: With the introduction of the pedicled nasoseptal flap for reconstruction of the skull base after EEA for resection of pituitary adenomas, the incidence of postoperative CSF leaks has decreased significantly. In this retrospective analysis, we demonstrate the effectiveness of the use of nasoseptal flap in repairing CSF leak after EEA.

Preoperative and Histological Predictors of Recurrence and Survival in Atypical Meningioma After Initial Gross Total Resection.

OBJECTIVE: Atypical (World Health Organization grade II) meningiomas (AMs) have been associated with a substantial risk of recurrence even after complete, gross total resection (GTR). The present study evaluated the clinical and AM tumor histopathological features that might predict for the risk of recurrence and survival within this patient population. METHODS: The data from 72 consecutive patients who had undergone primary GTR for AM from 2007 to 2016 and corresponding tumor specimens at a single institution were reviewed. The preoperative patient and tumor characteristics were correlated with the postresection outcomes, including recurrence and 1-year survival. Cox regression models on recurrence-free survival (RFS) and Kaplan-Meier survival estimates were performed. RESULTS: The overall 1-, 3-, and 5-year RFS estimates for the AM cohort were 100.0%, 82.4%, and 78.1% after resection, respectively. A high mitotic index was an independent predictor of RFS on Cox regression analysis (hazard ratio, 1.26; P = 0.008), and the tumor volume showed a trend toward a significant association (hazard ratio, 0.93; P = 0.079). Patient age and the mitotic index were significantly associated with 1-year mortality (odds ratio, 1.11 and 1.36, respectively; P = 0.028 and P = 0.045, respectively). CONCLUSIONS: AM tumors with a high proliferative index showed an increased likelihood of recurrence and short-term survival even after complete GTR. A smaller tumor volume might also have contributed to an increased risk of recurrence for patients with AM. Although other histopathological features were not linked to recurrence or mortality for patients with AM, the biopsy findings can indicate key predictive information, and further molecular analysis might reveal additional prognostic markers.

Diabetes Comorbidity Increases Risk of Postoperative Complications in Traumatic Thoracic Vertebral Fracture Repair: A Propensity Score Matched Analysis.

BACKGROUND: Thoracic vertebral fracture repair after a traumatic injury can be associated with significant risk for postoperative complications. Surgical outcomes are further complicated by patient comorbidity, particularly diabetes mellitus. This study compared outcomes and complication rates for traumatic thoracic vertebral fracture repair in a matched sample of patients with diabetes and nondiabetic control subjects. METHODS: Patients with a surgical repair of a trauma-induced thoracic vertebral fracture treated from 2010 to 2015 were identified from the Trauma Quality Improvement Program database, yielding 5557 cases. Patients with comorbid diabetes were matched by propensity score matching (PSM) with patients without diabetes on age, race, and body type and were compared by postoperative complications and clinical outcomes. RESULTS: Prior to PSM, the diabetes group was older on average and had a greater proportion of patients who were obese (Ps < 0.001). After PSM, each group consisted of 544 patients (N = 1088) and no longer differed by any baseline characteristic. Comorbid diabetes was associated with longer average length of hospital stay and greater frequency of several major and minor postoperative complications (Ps < 0.05), including prolonged intensive care, pneumonia, acute renal failure, stroke, pressure ulcers, and urinary tract infections, but no differences were found in reoperation rates or in-hospital mortality. CONCLUSIONS: Diabetes comorbidity can significantly increase the risk of postoperative complications after traumatic thoracic vertebral fracture repair, which may lead to delayed recovery and greater health care-related costs. This finding is an important consideration for surgical decision-making and patient counseling on treatment options with this comorbid condition.

Malpractice Litigation in Brain Tumor Surgery: A 31-Year Analysis of Causative Factors in the United States from the Westlaw Database.

BACKGROUND: Medical malpractice litigation is an issue of major concern in neurosurgery, with 19.1% of neurosurgeons facing a claim annually. Neurosurgery possesses the greatest risk of malpractice of any specialty, likely owing to the complex clinical environment and disease severity. In the present study, we have characterized such litigation to determine the common factors that compel plaintiffs to file these claims. METHODS: WestLawNext, a prominent legal database, was used to identify all cases from 1985 to 2016 related to brain tumors. A total of 225 cases were identified, and each was analyzed for the cause of litigation (multiple causes were permitted). Because many had >1 ground for litigation, the reported percentages were based on the total counts of litigation rather than the number of cases. Additional information was collected from each case, including location, tumor type, and physician specialty. RESULTS: The cases were distributed across 36 states and U.S. territories: California (n = 42; 20%) and New York (n = 28; 13%) had the greatest number of cases. The top reasons for litigation were failure to diagnose (n = 109; 28%), failure to treat (n = 72; 18%), procedural error (n = 63; 16%), and failure to refer for diagnostic tests (n = 55; 14%). The most common diagnoses included pituitary adenoma (n = 26; 12%), acoustic neuroma (n = 27; 12%), and meningioma (n = 23; 10%). CONCLUSIONS: Malpractice litigation contributes to high overhead and physician burnout and escalates the cost of patient care. We found that benign brain tumors were the most common in litigation and that surgical issues accounted for only a small percentage.

Weekend versus Weekday Admission in Spinal Cord Injury and Its Effect on Timing of Surgical Intervention.

OBJECTIVE: We sought to compare timing of intervention for patients with spinal cord injury (SCI) requiring surgical procedures during weekend versus weekday admissions. METHODS: The National Inpatient Sample database from 2012 to 2014 was queried to identify patients with SCI who underwent surgical treatment (decompression with or without stabilization) as an emergent/urgent procedure. Timing of intervention, inpatient morbidity, and inpatient mortality were compared between patients admitted during the weekend versus a weekday. Multiple logistic regression analyses were also performed. RESULTS: A total of 9390 patients were identified (mean age 55 years, 73.2% male) from the database, with 34.1% admitted during the weekend and 65.9% during a weekday. The average day of intervention for the entire cohort was 2.8 (SD 3.9, interquartile range 1-4); day 2.7 (standard deviation [SD] 4.0) versus day 2.8 ([SD] 3.9) for patients admitted in a weekend versus weekday (P = 0.418). After controlling for patient age, sex, and injury severity score on multiple logistic regression analysis, weekend admission was not significantly associated with early intervention (odds ratio [OR] 0.99; 95% confidence interval [CI], 0.82-1.21; P = 0.993), complication occurrence (OR 1.09; 95% CI, 0.86-1.38; P = 0.476), or inpatient mortality (OR 0.83; 95% CI, 0.44-1.56; P = 0.563). Patients with complete/American Spinal Injury Association A injuries were more likely to undergo early intervention (OR 2.09; 95% CI, 1.31-3.31; P = 0.002). CONCLUSION: In this national study, patients with SCI who were admitted during the weekend received surgical intervention as early as patients admitted during a weekday. Furthermore, no differences in complication or mortality rates between groups were found.

Antidepressant use in patients with meningioma: is there an association with tumor recurrence?

OBJECTIVE With increasing general use of antidepressants (ADs), multiple studies have noted a small protective effect of ADs for patients with glioma, but their impact on meningioma has not been established. This study aims to evaluate the role of ADs in the context of additional clinical factors in relation to long-term risk of meningioma recurrence. METHODS One hundred five patients with an intracranial meningioma presenting from 2011-2014 with at least 3 years of follow-up (median 4.2 years) after resection were reviewed. AD use along with demographics, tumor characteristics, and outcomes were recorded. Multivariate logistic regression was used to analyze the association of AD use with tumor recurrence, including other clinical measures significantly associated with recurrence as covariates. RESULTS Twenty-nine patients (27.4%) were taking ADs (27 selective serotonin reuptake inhibitors, 2 norepinephrine-dopamine reuptake inhibitors) prior to tumor recurrence. Their tumors largely affected the frontal (31.0%) or parietal lobe (17.2%) and were located in convexity, parasagittal, or falcine (CPF) areas more frequently than skull base areas relative to the tumors of non-AD users (p = 0.035). AD use was found to be an independent predictor of recurrence, in addition to subtotal resection and WHO grade II/III classification (p values < 0.05). The median time from AD prescription to tumor recurrence was 36.6 months (interquartile range [IQR] = 20.9-62.9 months) and median length of AD use was 41.4 months (IQR = 24.7-62.8 months). CONCLUSIONS AD use was an independent predictor of meningioma recurrence. This association may be due to mood or affective changes caused by tumor location in CPF regions that may be a sign of early recurrence. The finding calls attention to AD use in the management of patients with meningioma, and warrants further exploration of an underlying relationship.

Impact of frailty on complications in patients with thoracic and thoracolumbar spinal fracture.

OBJECTIVES: It is well-documented that geriatric patients are at risk for serious injuries after fracture due to pre-existing medical conditions, physical changes of aging, and medication effects. Frailty has been demonstrated to be a predictor of morbidity and mortality in inpatient head and neck surgery, and for surgical intervention for adult spinal deformity and degenerative spine disease. However, the impact of frailty on complications following thoracolumbar/thoracic fractures are unknown and has not been previously assessed in the literature, particularly in a nationwide setting. PATIENTS AND METHODS: This was a retrospective study of the prospectively-collected American College of Surgeons National Surgical Quality Improvement database for the years 2007 through 2012. Patients who underwent spinal decompression (+/- fusion) or an alternative intervention, defined as vertebroplasty or kyphoplasty (VP/KP) for thoracic or thoracolumbar fracture were identified. Frailty status was determined using a modified frailty index from the Canadian Study of Health and Aging Frailty Index, with frailty defined as a score = 0.27. 30-day morbidity and mortality were compared between frail and non-frail patients in each treatment group. RESULTS: A total of 303 patients were included in this study. Of these, 38% of patients had VP/KP and 62% underwent surgery. Within the VP/KP cohort, 26% were frail. The proportion of these patients who developed at least one complication was 3.3% versus 3.6% for non-frail patients (p = 1.0). The 30-day mortality for frail versus not frail patients in this cohort was 0% versus 2.4% (p = 1.0). Among the surgical group, 13% were frail. In contrast, the likelihood of complications was 33.3% among frail patients and 4.2% for non-frail patients (p < 0.001). Frail patients also had a 16.7% 30-day mortality rate as compared to 0.6% in the non-frail group (p = 0.001). When comparing the frail versus non-frail patients overall, frail patients had a complication rate of 16.7%, as opposed to 4.0% in non-frail patients. CONCLUSION: Frailty and surgical intervention are correlated with a higher 30-day complication rate in patients with thoracic and thoracolumbar fracture. This finding is an important consideration for surgical decision-making and patient counseling on treatment options.

Is Psychiatric Depression a Presenting Neurologic Sign of Meningioma? A Critical Review of the Literature with Causative Etiology.

BACKGROUND: Benign meningiomas constitute 80%-90% of all meningiomas and represent the most common type of central nervous system tumor in adults. The vast majority of meningiomas are minimally symptomatic or asymptomatic early in their onset and thereby can often result in delayed diagnosis. Early diagnosis of meningioma is critical, as it can maximize treatment options and improve outcomes and survival. Although seizures and focal neurologic deficits are considered to be the most prevalent symptoms, depression also may be an important and significant sign. A subtle neurologic depression may be an even early presenting sign of meningioma and may precede more traditional presenting symptoms. METHODS: We performed a comprehensive literature review that analyzes the results of prospective studies and case reports on this topic. RESULTS: Our findings show evidence to suggest that depression may be correlated with meningioma presentation. Its prevalence is possibly increased with an anterior location of the tumor. CONCLUSIONS: For patients who exhibit nuances of depression without a history of psychiatric illness, an index of suspicion for meningioma may be warranted.

Two-year observational study of deferiprone in superficial siderosis.

INTRODUCTION: Superficial siderosis is a rare, neurodegenerative disease caused by toxic accumulation of hemosiderin on the surface of the brain and the spinal cord, most commonly from chronic subarachnoid hemorrhage. AIMS: The aim of this study was to assess the clinical and radiological outcomes of superficial siderosis patients using deferiprone, a cell permeant iron chelator. Subjects obtained pre- and post-treatment brain MRIs and weekly laboratory tests. Osirix software was used to develop a method of quantifying hemosiderin deposition. Three-dimensional whole brain images of gradient echo images were rendered and compared by dividing the mean T2 hyperintensity to the maximal cerebrospinal fluid signal. RESULTS: A total of 38 subjects completed the study, of which clinical and radiological data were available for 30. The average age was 64 years (range 37-86), 53% were male, 94% were white. Nineteen subjects (63%) reported either no progression of disease or an improvement in at least one neurological domain, with 40% of patients reporting a stabilization in hearing function and 30% reporting stable or improved coordination and walking. By MRI, there was an overall mean increase in T2 hyperintensity of the whole brain of 1%-13% over the 2-year time period in half of patients, indicating a reduction hemosiderosis. There were no cases of agranulocytosis, and declines of white blood cells counts and neutrophils averaged <10%. Fatigue was the most common side effect. CONCLUSION: This is the first long-term prospective study of superficial siderosis on the iron chelator, deferiprone. MRI quantification of hemosiderin appears to demonstrate a measurable reduction in half of patients and this correlated with a stabilized or improving disease course. A future placebo-controlled trial is necessary to determine whether deferiprone is an effective therapy for superficial siderosis.