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Introduction The delivery of flow-diverting stents (FDS) necessitates a degree of catheter support beyond that required for endovascular coiling. The TracStar Large Distal Platform (LDP) is a novel 0.088″ platform intended for navigation into the intracranial internal carotid artery (ICA). We present an early institutional experience using the TracStar LDP in 44 cases of endovascular aneurysm embolization using FDS. Methods Inclusion criteria for this single-center retrospective review encompassed all patients >18 years of age who were treated for intracranial aneurysms. Procedural success was defined as successful stent deployment using the TracStar LDP. Other outcomes included periprocedural complications, use of an intermediate catheter, length of stay, and discharge disposition. Results The TracStar LDP was utilized in 44 consecutive FDS cases in 42 patients. Cavernous segment aneurysms constituted the majority of cases (12/42; 28.6%), followed by posterior communicating artery (8/42; 19.0%) and supraclinoid aneurysms (8/42; 19.0%). Successful FDS deployment was achieved in 43/44 cases. The LDP achieved stable positioning within the ascending cavernous ICA in 63.6% of cases. A biaxial system was utilized in 54.5% of cases. There was one complication potentially related to use of the TracStar LDP, which was an asymptomatic ICA vessel dissection managed conservatively. Conclusions The TracStar LDP is safe and effective during use in the endovascular treatment of intracranial aneurysms with a FDS. Access to the ascending portion of the cavernous ICA was regularly achieved, and the platform allowed for both biaxial and triaxial configurations.

BackgroundMost conventional 0.088 inch guide catheters cannot safely navigate intracranial vasculature. The objective of this study is to evaluate the safety of stroke thrombectomy using a novel 0.088 inch guide catheter designed for intracranial navigation.MethodsThis is a multicenter retrospective study, which included patients over 18 years old who underwent thrombectomy for anterior circulation large vessel occlusions. Technical outcomes for patients treated using the TracStar Large Distal Platform (TracStar LDP) or earlier generation TRX LDP were compared with a matched cohort of patients treated with other commonly used guide catheters. The primary outcome measure was device-related complications. Secondary outcome measures included guide catheter failure and time between groin puncture and clot engagement.ResultsEach study arm included 45 patients. The TracStar group was non-inferior to the control group with regard to device-related complications (6.8% vs 8.9%), and the average time to clot engagement was 8.89 min shorter (14.29 vs 23.18 min; p=0.0017). There were no statistically significant differences with regard to other technical outcomes, including time to recanalization (modified Thrombolysis In Cerebral Infarction (mTICI) ≥2B). The TracStar was successfully advanced into the intracranial internal carotid artery in 33 cases (73.33%); in three cases (6.67%), it was swapped for an alternate catheter. Successful reperfusion (mTICI 2B-3) was achieved in 95.56% of cases. Ninety-day follow-up data were available for 86.67% of patients, among whom 46.15% had an modified Rankin Score of 0–2%, and 10.26% were deceased.ConclusionsTracstar LDP is safe for use during stroke thrombectomy and was associated with decreased time to clot engagement. Intracranial access was regularly achieved.

Objective A ranula is a mucus-filled salivary pseudocyst that forms in the floor of the mouth, commonly arising from the sublingual or submandibular salivary glands following obstruction or trauma. Complete excision of the injured gland and removal of the cyst content is the first-choice therapy, but has the potential for complications related to injury to nearby structures. As such, minimally invasive approaches such as percutaneous sclerotherapy have been investigated. We aim to contribute to the literature by assessing the efficacy and safety of our technique through our experience with 18 patients over the last decade. Methods This retrospective study evaluated 18 patients with intraoral and plunging ranulas treated by percutaneous bleomycin ablation. The primary endpoint was the treatment result. Secondary endpoints included bleomycin dosage and complications. Results The study evaluated 12 males and six females with a median age of 23.5 years (range 13–39 years). At a final follow-up of at least 2 months (6.5±5.5 months), four patients demonstrated complete response (22%) and 14 patients demonstrated residual presence, recurrence, or regrowth of the lesion (78%). There were no statistically significant associations between outcomes and history of prior treatment, number of treatments, and size or type of ranula. No complications were noted. Conclusions Our findings indicate that bleomycin, while safe for use in various head and neck malformations, is of limited utility in ranula therapy when the offending gland is not addressed primarily.

Background In December 2020, the JET7 Xtra Flex reperfusion catheter was voluntarily recalled after reports of injury and death associated with device malfunction. The FDA’s MAUDE (Manufacturer and User Facility Device Experience) database aggregates reports of adverse events and product problems involving medical devices. We present the first analytical review of all MAUDE reports involving FDA cleared reperfusion catheters in order to summarize safety events to date and characterize unique modes of device failure. Methods All publicly available MAUDE datasets were downloaded and linked, and all events associated with catheters carrying product code ‘NRY’ were extracted. Product code ‘NRY’ encompasses all devices intended to restore blood flow by removing thrombus/clots in patients experiencing ischemic stroke. Note that this review does not include catheters used off-label for aspiration thrombectomy. Event characteristics including device type, malfunction and adverse events were collected directly as reported on MedWatch Form 3500. In order to characterize procedure-specific details, narrative information submitted in MedWatch Form 3500 Sections B and H was coded by 2 independent raters. Results Between 1/1/2003 and 8/31/2020, there were 1,808 reperfusion catheter defects and/or malfunctions reported in the MAUDE database. Of these, 93.4% did not result in any adverse effect on patients. There were 112 malfunctions associated with an adverse event, of which 98 (87.5%) resulted in injury, and 14 (12.5%) resulted in death. A summary of malfunctions is available in table 1. When interpreting these findings, it is important to note that FDA surveillance of these devices does not include data on the number of units sold, thus event rates cannot be determined or compared across devices. Of 14 reported deaths, 13 involved malfunctions of the Jet 7 Xtra Flex catheter. All of these cases reported mechanical failure at the tip of the catheter, 10 of which involved tip rupture or ballooning following contrast injection. Ballooning was unique to the Jet 7 Xtra Flex, and was also responsible for 3 patient injuries and 37 cases of malfunction that did not result in adverse outcomes. One death and 45 injuries involved the 3 MAX Reperfusion catheter. All of these events were caused by catheter fracture leading to fragment embolization or vessel injury. This mechanism of failure was not unique to the 3 MAX, and was present in 61.2% of all malfunctions resulting in injury. Among malfunctions that did not result in adverse events, the majority (59.1%) were attributable to damage that occurred on the back table during device preparation. Detailed descriptions of unique malfunction mechanisms for each catheter are omitted from this brief abstract but are available for presentation. Conclusion Reperfusion catheter failures resulting in injury or death are rare. These findings suggest that current methods of FDA surveillance have effectively identified outliers. Disclosures D. Bageac: None. B. Gershon: None. R. De Leacy: None.

This chapter discusses the evolution of devices and techniques in endovascular stroke therapy, beginning with the development of effective intra-arterial thrombolysis in the late 1990s. We discuss the subsequent rise of early mechanical devices such as the Merci Retriever and Penumbra System, which, despite culminating in a series of failed trials in 2013, set the stage for the modern thrombectomy era. These first-generation devices gave way to the stent retriever, which would change the standard of care for acute large vessel occlusion stroke following a series of landmark trials in 2015. Finally, we discuss the more recent establishment of aspiration thrombectomy as an alternative to stent retrievers, as well as current investigations into novel devices and techniques that might further improve patient outcomes. Throughout, attention is paid to the regulatory landscape and evidentiary standards that shaped the field during its development in order to better understand how lessons from this history can guide future studies.

OBJECTIVE: The clinical diagnosis of primary lateral sclerosis can only be made after upper motor neuron symptoms have progressed for several years without developing lower motor neuron signs. The goal of the study was to identify neuroimaging changes that occur early in primary lateral sclerosis, prior to clinical diagnosis. METHODS: MRI scans were obtained on 13 patients with adult-onset progressive spasticity for five years or less who were followed longitudinally to confirm a clinical diagnosis of primary lateral sclerosis. Resting state functional MRI, diffusion tensor imaging, and anatomical images were obtained. These ‘pre-PLS’ patients were compared to 18 patients with longstanding, established primary lateral sclerosis and 28 controls. RESULTS: Pre-PLS patients had a marked reduction in seed-based resting-state motor network connectivity compared to controls and patients with longstanding disease. White matter regions with reduced fractional anisotropy were similar in the two patient groups compared to controls. Patients with longstanding disease had cortical thinning of the precentral gyrus. A slight thinning of right precentral gyrus was detected in initial pre-PLS patients’ scans. Follow-up scans in 8 pre-PLS patients 1-2 years later showed increasing motor connectivity, thinning of the precentral gyrus, and no change in diffusion measures of the corticospinal tract or callosal motor region. CONCLUSIONS: Loss of motor functional connectivity is an early imaging marker in primary lateral sclerosis. This differs from literature descriptions of amyotrophic lateral sclerosis, warranting further studies to test whether resting-state functional MRI can differentiate between amyotrophic lateral sclerosis and primary lateral sclerosis at early disease stages.

BackgroundOver the past several years there has been increased interest in the use of the Sofia aspiration system (MicroVention, Tustin, California) as a primary aspiration catheter.ObjectiveTo perform a multicenter retrospective study examining the efficacy of the Sofia aspiration catheter as a standalone aspiration treatment for large vessel occlusion.MethodsConsecutive cases in which the Sofia catheter was used for aspiration thrombectomy for large vessel occlusion were included. Exclusion criteria were the following: (1) Sofia not used for first pass, and (2) a stent retriever used as an adjunct on the first pass. The primary outcome of the study was first pass recanalization (Thrombolysis in Cerebral Infarction (TICI) 2c/3). Secondary outcomes included first pass TICI 2b/3, crossover to other thrombectomy devices, number of passes, time from puncture to recanalization, and complications.Results323 patients were included. First pass TICI 2c/3 was achieved in 49.8% of cases (161/323). First pass TICI 2b/3 was achieved in 69.7% (225/323) of cases. 74.8% had TICI 2b/3 with the Sofia alone. Crossover to other thrombectomy devices occurred in 29.1% of cases (94/323). The median number of passes was 1 (IQR=1–3). Median time from puncture to recanalization was 26 min (IQR=17–45). Procedure related complications occurred in 3.1% (10/323) of cases.ConclusionOur study highlights the potential advantage of the Sofia aspiration catheter for primary aspiration thrombectomy in acute ischemic stroke. High rates of first pass recanalization with low crossover rates to other thrombectomy devices were achieved. Median procedure time was low, as were procedural complications.

Endovascular thrombectomy has revolutionized the management of acute ischemic stroke from emergent large vessel occlusion. Continued technological advancement in the field, as evidenced by successive introduction of large bore aspiration catheters with enhanced trackability and large inner diameter, has played a major role in achieving fast and robust recanalization and improved clinical outcome. Here, we present three patients with intraprocedural device malfunction related to the JET 7 XTRA Flex reperfusion catheter.

Two patients, separated by 1 year, underwent mechanical thrombectomy using next generation, highly navigable 0.088-inch large bore catheters, which were navigated to and aspirated within the M1 middle cerebral artery segment. Case 1 demonstrates the first reported clinical application of this technique used in conjunction with stent retriever and direct aspiration through an intermediate catheter, resulting in modified thrombolysis in cerebral infarction (mTICI) score 3 recanalisation, and a 90-day modified Rankin Score of 1. In case 2, direct on-clot aspiration was applied through a 0.088-inch guide catheter in the left M1 segment, resulting in mTICI score 3 recanalisation and a National Institutes of Health Stroke Scale score of 1 at discharge. There was no evidence of untoward events in either case. Advancement of a 0.088-inch catheter into the M1 segment offers potential benefits to thrombectomy by improving device-thrombus interaction, inducing local flow arrest and protecting proximal vessels from embolus to new territories.

Introduction Increased procedure time during endovascular treatment of cerebral aneurysms is associated with increased anesthesia related complications and radiation exposure. Elimination of the need for intermediate catheters during aneurysm treatment may decrease procedure time and increase cost effectiveness. A novel device -the TracStar Large Distal Platform (LDP) -may offer more distal final positioning when compared to commonly used guide catheters, thus decreasing the need for guide catheter use. We investigate the safety and efficacy of the TracStar LDP when used during endovascular aneurysm treatment. Methods We perform a multicenter retrospective review of endovascular cerebral aneurysm embolizations during which the TracStar LDP was utilized. Aneurysm location, procedural information, and complications were recorded as detailed in the operative note. Vascular tortuosity was assessed via pre-procedural CTA. Distal-most position achieved with TracStar was determined by review of intraprocedural imaging. Results A preliminary analysis of 30 cases was performed. Flow diversion was performed in 25 cases (83%), and stent assisted coiling was performed in the remainder. The target aneurysm was located in the ICA in 27 cases (90%), the MCA bifurcation in 2 cases (7%), and the ACA in 1 case (3%). An intermediate catheter was required in 18 cases (60%). TracStar LDP achieved stable access to the Cavernous ICA in 70% of cases. Average procedure time was 67± 33 minutes.There were no procedural complications or new neurologic defects after treatment. TracStar LDP was exchanged for an alternative guide catheter in one case due to catheter kinking, and in two cases due to lack of support. Conclusion Use of Tracstar LDP during flow-diversion and sent-assisted coiling of cerebral aneurysms is safe and effective. Access to the cavernous segment of the ICA was achieved in the majority of cases, and use of an intermediate catheter was not required in 40% of cases. Final analysis of the the full 60 patient multi-center study will be available for presentation at SNIS Annual 2020. Disclosures D. Bageac: None. R. De Leacy: None.

BACKGROUND AND PURPOSE: In recent years, the transradial approach has become more widely adopted for neuroendovascular procedures. The purpose of this study was to evaluate the safety and feasibility of a transradial approach and distal transradial access for neuroendovascular procedures in a single center. MATERIALS AND METHODS: Retrospective analysis was performed for all patients who underwent transradial approach or distal transradial access neuroendovascular procedures from January 2016 to August 2019 at a single center. Exclusion criteria included a Barbeau D waveform, a radial artery of

OBJECTIVES We retrospectively analyzed data of the BECOME trial to investigate whether serial administration of triple-dose (3-dose) gadopentetate dimeglumine would result in the development of T1 signal-to-noise (S/N) changes in the cranial diploic space and whether S/N changes correlated with on-study hypophosphatemia. METHODS Signal intensity analysis was performed on the first year's data of the BECOME trial using 3-dose Gd (14 months, maximum number of doses, 39, mean: 36). Routine blood and urine tests were obtained each month for safety monitoring. Linear mixed regression modeling with random intercept was used to analyze monthly signal-to-noise ratio (S/N = Bone/Air) using an ROI of the diploic space created from T2W images and overlaid on T1FS (T1 fat-saturated) images. Incidence of phosphate abnormalities was analyzed using the general estimation equation; correlation of phosphate and S/N change was achieved with type 3 test of fixed effects. RESULTS Cranial diploic space T1FS S/N increased over 14 months: S/N = 0.039 mean monthly increase (S.E. 0.008; p

BACKGROUND CONTEXT Currently, there is no standard of care regarding the management of osteoporotic, malignant or traumatic vertebral compression fractures (VCFs). It is unclear which clinical parameters constitute sufficient indication for proceeding beyond conservative treatment. The criteria for surgical intervention are also not well defined. Recently, the SpineJack (Stryker, Kalamazoo, MI, USA) implantable device for the reduction of VCFs has gained FDA clearance for marketing in the United States. To date, there have not been any large reports describing early use cases, outcomes, or adverse events with SpineJack in the United States. Of immediate relevance is the use of the device in cases of osteoporotic compression fractures (for which it is FDA approved) and malignant compression fractures (for which it is not). The present study is a retrospective analysis of the largest international multicenter registry for vertebral augmentation using SpineJack. PURPOSE To review the efficacy of vertebral height restoration for painful VCFs using the SpineJack implantable device in a large international sample of real-world cases. STUDY DESIGN/SETTING Retrospective analysis is conducted utilizing a multicenter retrospective registry comprised of 180 patients who underwent vertebral augmentation using SpineJack in the United States and Europe. PATIENT SAMPLE The inclusion criteria of this study encompass any patient who underwent vertebral augmentation in whom the SpineJack device was used for the treatment of painful osteoporotic, malignant, or traumatic VCFs. OUTCOME MEASURES Primary outcomes measures include: (1) vertebral height restoration (mm), (2) change in NRS pain score, (3) rate of adjacent level fractures, and (3) rate of serious adverse events (SAEs). Secondary outcome measures include: (1) change in kyphotic angle, and (2) change in opioid utilization. METHODS Case enrollment began in August 2019. Pre- and postprocedural pain was recorded using the Numeric Rating Scale (NRS). Functional recovery is measured using the Oswestry Disability Index, and change in opioid utilization postprocedurally is measured in morphine equivalent units. Vertebral height is measured at the anterior vertebral body on pre- and postprocedural imaging. Loss of vertebral height due to VCF is calculated using prefracture imaging when available, or the average of vertebral height at the adjacent levels above and below the VCF. In a preliminary analysis of 27 cases, local kyphotic angle is measured as the angle of intersection between planes drawn along the superior and inferior endplates of the affected vertebrae. Final analysis will include segmental kyphotic angle, measured by the method of cobb utilizing the superior endplate of the level above the fracture and the inferior endplate of the level below. Follow-up for all outcome measures in conducted in the immediate postoperative period, 1 month, 3 months and 6 months after the procedure. RESULTS Case enrollment is schedule to close in August 2020. In a preliminary analysis of 27 patients who underwent vertebral augmentation with SpineJack at 34 separate levels, patients experienced an average vertebral height recovery of 74% (loss of 30±19% to 13±25% post procedurally). Local kyphotic angle was reduced by an average of 50% (7.5±7.5° to 4.0±5.3° post procedurally). 33% of patients (n=9) achieved complete restoration of prefracture vertebral height. 49% of patients achieved pain reduction (NRS decrease >50%) within the first 24 hours post-operatively. There were no SAEs or adjacent level fractures in this prelimary cohort, and the rate of opioid utilization remained unchaged in the immediate postoperative period. CONCLUSIONS The present study presents real-world evidence for the safety and efficacy of SpineJack as used in six centers in the United States and Europe. Preliminary results suggest excellent vertebral height restoration and reduction in local kyphotic angle. There were no SAEs or adjacent level fractures. FDA DEVICE/DRUG STATUS SpineJack (Not approved for this indication)

Objectives: The goal of this study was to better understand the changes in tissue microstructure that underlie white matter diffusion changes in ALS patients. Methods: Diffusion tensor imaging was carried out in postmortem brains of 4 ALS patients and two subjects without neurological disease on a 7T MRI scanner using steady-state free precession sequences. Fractional anisotropy (FA) was measured in the genu, body, and splenium of the corpus callosum in formalin-fixed hemispheres. FA of the body and genu was expressed as ratio to FA of the splenium, a region unaffected in ALS. After imaging, tissue sections of the same segments of the callosum were stained for markers of different tissue components. Coded image fields were rated for pathological changes by blinded raters. Results: The FA body/FA splenium ratio was reduced in ALS patients compared to controls. Patchy areas of myelin pallor and cells immunostained for CD68, a microglial-macrophage marker, were only observed in the body of the callosum of ALS patients. Blinded ratings showed increased CD68+ microglial cells in the body of the corpus callosum in ALS patients, especially those with C9orf72 mutations, and increased reactive astrocytes throughout the callosum. Conclusion: Reduced FA of the corpus callosum in ALS results from complex changes in tissue microstructure. Callosal segments with reduced FA had large numbers of microglia-macrophages in addition to loss of myelinated axons and astrogliosis. Microglial inflammation contributed to reduced FA in ALS, and may contribute to a pro-inflammatory state, but further work is needed to determine their role. Keywords: 7T MRI, Amyotrophic lateral sclerosis, Microglia, Motor neuron disease, Pathology, Steady-state free precession

Preclinical models suggest that focal high-dose radiation can make tumors more immunogenic. We performed a pilot study of stereotactic body radiation therapy (SBRT) followed by high-dose interleukin-2 (IL-2) to assess safety and tumor response rate and perform exploratory immune monitoring studies. Patients with metastatic melanoma or renal cell carcinoma (RCC) who had received no previous medical therapy for metastatic disease were eligible. Patients received one, two, or three doses of SBRT (20 Gy per fraction) with the last dose administered 3 days before starting IL-2. IL-2 (600,000 IU per kilogram by means of intravenous bolus infusion) was given every 8 hours for a maximum of 14 doses with a second cycle after a 2-week rest. Patients with regressing disease received up to six IL-2 cycles. Twelve patients were included in the intent-to-treat analysis, and 11 completed treatment per the study design. Response Evaluation Criteria in Solid Tumors criteria were used to assess overall response in nonirradiated target lesions. Eight of 12 patients (66.6%) achieved a complete (CR) or partial response (PR) (1 CR and 7 PR). Six of the patients with PR on computed tomography had a CR by positron emission tomography imaging. Five of seven (71.4%) patients with melanoma had a PR or CR, and three of five (60%) with RCC had a PR. Immune monitoring showed a statistically significantly greater frequency of proliferating CD4(+) T cells with an early activated effector memory phenotype (CD3(+)CD4(+)Ki67(+)CD25(+)FoxP3(-)CCR7(-)CD45RA(-)CD27(+)CD28(+/-)) in the peripheral blood of responding patients. SBRT and IL-2 can be administered safely. Because the response rate in patients with melanoma was significantly higher than expected on the basis of historical data, we believe that the combination and investigation of CD4(+) effector memory T cells as a predictor of response warrant further study.

174 Background: The primary aim of this exploratory trial was to determine the feasibility and safety of chemoradiotherapy (CRT) with combinatorial immunotherapy in LA or BR PC. Methods: 10 patients (pts) with untreated, LA (n=6) or BR (n=4) biopsy-proven PC received gemcitabine (GEM) in combination with telomerase peptide vaccine (GV1001) and sargramostim (GM-CSF) both by intradermal injection. Tadalafil was taken once daily throughout the course of therapy for myeloid derived suppressor cell inhibition. The 2nd month of treatment included external beam radiation therapy (50.4 Gy, 28 fractions) and concurrent twice-weekly GEM 50 mg/m2. Disease response was assessed at 12 weeks. Following recovery from either concurrent CRT or resection, 8 weeks of GV1001 + GM-CSF, tadalafil and GEM completed treatment. Peripheral blood was analyzed by quantitative multiparameter whole blood flow cytometry of immune cell subtypes. Samples were also cryo-preserved for future analysis of peripheral blood mononuclear cell (PBMC) populations and ELISPOT analysis of antigen-specific T cell numbers. Tumor resection specimens were sectioned for immunohistological analysis of macrophage and T cell infiltration. Results: 9 of 10 pts completed therapy to assess response. Grade (Gr) 3-4 hematologic adverse events (AE) were observed during CRT. Immune-related AE were infrequent and mild. 3 of 4 pts with BR disease and 1 with initial LA (unresectable) disease achieved R0 resection. No unanticipated surgical complications were observed. Changes in monocytes and granulocytes occurred throughout treatment and may correlate with clinical benefit. At least 50% reduction in serum CA19-9 was observed in 8 pts. Partial response (n=2) or stable disease (n=7) was achieved in 9 pts. Conclusions: Combinatorial immunotherapy concurrent with GEM-based CRT is feasible and appears safe in LA or BR PC. Initial biochemical responses as well as disease control were observed in the majority of pts. Preliminary immune correlative data suggest a relationship between hematopoietic populations and clinical response.

Expansion mutations in the C9orf72 gene may cause amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), or mixtures of the two clinical phenotypes. Different imaging findings have been described for C9orf72-associated diseases in comparison with sporadic patients with the same phenotypes, but it is uncertain whether different phenotypes have a common genotype-associated imaging signature. To address this question, 27 unrelated C9orf72 expansion mutation carriers (C9 +) with varied phenotypes, 28 age-matched healthy controls and 22 patients with sporadic ALS (sALS) underwent 3T MRI scanning and clinical phenotyping. Measures of brain volumes and cortical thickness were extracted from T1 images. Compared to healthy controls and sALS patients, symptomatic C9 + subjects had greater ventricular volume loss and thalamic atrophy for age, with diffuse, patchy cortical thinning. Asymptomatic carriers did not differ from controls. C9 + ALS and ALS-FTD patients had less thinning of the motor cortex than sALS patients, but more thinning in extramotor regions, particularly in frontal and temporal lobes. C9 + ALS patients differed from sporadic ALS patients in the thickness of the superior frontal gyrus and lateral orbitofrontal cortex. Thickness of the precentral gyrus was weakly correlated with the revised ALS functional rating scale. Thickness of many cortical regions, including several frontal and temporal regions, was moderately correlated with letter fluency scores. Letter fluency scores were weakly correlated with ventricular and thalamic volume. To better understand how imaging findings are related to disease progression, nineteen C9 + subjects and 23 healthy controls were scanned approximately 6 months later. Ventricular volume increased in C9 + patients with FTD and ALS-FTD phenotypes and remained stable in asymptomatic C9 + subjects. We conclude that diffuse atrophy is a common underlying feature of disease associated with C9orf72 mutations across its clinical phenotypes. Ventricular enlargement can be measured over a 6-month time frame, and appears to be faster in patients with cognitive impairment.