CSF Spaces Research Articles

Lumboperitoneal Shunt Combined With Ommaya Reservoir Enables Continued Intraventricular Chemotherapy for Leptomeningeal Metastasis With Increased Intracranial Pressure.

Intra-cerebrospinal fluid (CSF) chemotherapy for leptomeningeal metastasis (LM) can be delivered intraventricularly via an Ommaya reservoir. However, hydrocephalus associated with LM can interfere with chemotherapeutic drug distribution, and ventriculoperitoneal shunts can prevent drug distribution to the extra-ventricular CSF space. This study examined the feasibility of combining a lumboperitoneal (LP) shunt with an Ommaya reservoir to both control intracranial pressure and allow for intraventricular chemotherapy. We identified 16 patients with LM who received both an Ommaya reservoir and an LP shunt, either concurrently or sequentially, and subsequently received intraventricular chemotherapy. The feasibility of this combination for intraventricular chemotherapy was evaluated by assessing 1) the distribution of intraventricularly injected drugs in CSF samples collected 0, 6, and 12 h post-injection and 2) adverse events associated with the procedure and drug administration. Patients received a median of seven rounds (range 1-37) of intraventricular chemotherapy during a median follow-up period of 5.2 months after LP shunt insertion. Pharmacokinetic data were obtained from six patients. Baseline methotrexate (MTX) levels from Ommaya reservoirs varied from 339.9 µM to 1,523.5 µM. CSF sampled from LP shunt reservoirs revealed an elimination half-life (t1/2) of 2.63 h, and the mean ratio of MTX concentration at 12 h to that at baseline was 0.05±0.05, ensuring drug distribution from the ventricle to the spinal canal. Nine patients (56%) underwent revision surgery due to catheter migration, malfunction, or infection. Among these patients, CSF infections attributable to intraventricular chemotherapy (n=3) occurred, but no infections occurred in later cases after we began to employ a complete aseptic technique. LP shunt combined with Ommaya reservoir insertion is a feasible option for achieving both intracranial pressure control and the continuation of intraventricular chemotherapy in patients with LM.

Assessment of patients with a Chiari malformation type I

IntroductionThe prevalence of Chiari malformation type I (CM-I) has been estimated as up to 1% of the general population. The majority of patients are asymptomatic and usually do not need treatment. Symptomatic patients, and some asymptomatic patients with associated conditions, may benefit from further assessment and treatment. Research questionThe aim of this review was to describe the clinical and radiological assessment of patients presenting with a CM-I. Material and methodsA literature search was performed using the PubMed and Embase databases focused on clinical assessment and imaging techniques used to diagnose CM-I. ResultsFollowing a complete clinical evaluation in patients with symptomatic CM-I and/or radiologically significant CM-I (tonsillar impaction, resulting tonsillar asymmetry and loss of CSF spaces), MRI of the brain and whole spine enables an assessment of the CM-I and potential associated or causative conditions. These include hydrocephalus, syringomyelia, spinal dysraphism, and tethered cord. Flow and Cine MRI can provide information on CSF dynamics at the craniocervical junction, and help in surgical decision-making. Hypermobility or instability at the upper cervical and craniocervical junction is less common and can be measured with CT imaging and flexion/extension or upright MRI. Discussion and conclusionThe majority of CM-I detected are incidental findings on MRI imaging of brain or spine, and do not require intervention. Once a radiological diagnosis and concern has been raised, clinical assessment by an appropriate specialist is required. A MRI brain and cervical spine is indicated in all radiologically labelled CM-I. In symptomatic patients or cases of radiologically significant CM-I, MRI of the brain and entire spine is indicated. Further investigations should be tailored to individuals’ needs.

Clinical Reasoning: A 64-Year-Old Man With History of Meningitis Presenting With Proximal Weakness of the Arms

A 64-year-old man presented for evaluation of proximally pronounced weakness of the arms with preserved facial and lower extremity strength. Symptoms slowly developed over the last 2 years, and the patient's history was notable for severe Listeria monocytogenes meningitis 4 years before presentation, which was adequately treated with antibiotics. On examination, symptoms clinically reassembled man-in-the-barrel syndrome and localized to the cervicothoracic central cord. Blood analysis was unremarkable, and CSF analysis showed no recurrent or persistent infection. Spinal MRI revealed pockets of sequestered CSF from C3 to C4 and areas of CSF space effacement from C3 to T12. MRI findings were interpreted as cord tethering suggestive of adhesive arachnoiditis. CT myelogram showed insufficient contrast agent migration above T10 and contour irregularities of the conus medullaris, confirming the postulated pathomechanism of cord tethering. Final diagnosis was therefore cervicothoracic central cord damage due to cord tethering in the setting of postinfectious adhesive arachnoiditis following bacterial meningitis. The patient failed a course of pulsed methylprednisolone therapy, and symptoms progressed. Best supportive care was provided. The clinical presentation of adhesive arachnoiditis is variable, and advanced imaging techniques and invasive studies such as CT myelogram may be required to establish the diagnosis. Timely diagnosis is warranted as early surgical or medical therapy can improve symptoms.

NCMP-03. ANATOMIC AND SURGICAL FACTORS PREDICT DEVELOPMENT OF LEPTOMENINGEAL DISEASE IN PATIENTS WITH METASTATIC MELANOMA

Abstract Leptomeningeal disease (LMD) is a devastating complication of systemic malignancy, portending a poor prognosis with an estimated median survival of 4-6 weeks if left untreated. Several reports have suggested surgical resection as a potential causative factor. Herein, we explore if surgical and anatomical factors are correlated with development of LMD in patients with melanoma brain metastases. METHODS: Patients treated at our institution between 1999-2019 for primary melanoma with brain metastasis were compiled into a database based on ICD9/10 coding. 1,079 patients with melanoma brain metastases and appropriate imaging were identified, and 834 patients with a minimum of 3 months’ follow up were included. Patients were dichotomized by development of LMD or lack thereof, and categorized into an overall cohort, and surgical and non-surgical cohorts. Anatomic factors and ventricular access during surgery were investigated as possible correlative factors for the development of LMD. RESULTS: In the overall cohort, female gender(p=0.033), presence of dural metastasis(p=0.018), presence of periventricular lesions(p< .001), presence of intraventricular lesions(p< .001), and ventricular access during surgery(p< .001) were significantly associated with LMD. Patients undergoing surgery, or those undergoing surgery without ventricular access, were not at higher risk of LMD. On multivariate analysis, female gender(p=.033), presence of periventricular lesions (p< .001), presence of intraventricular lesions(p< .002), and presence of dural metastasis(p=0.032) were significantly associated with development of LMD. In patients who had surgery, iatrogenic ventricular access(p< .001) was significantly correlated with LMD. In the group of patients without surgery, those with periventricular lesions had significantly higher odds of LMD(p< .001). CONCLUSIONS: In a retrospective cohort of patients with melanoma metastatic to the brain, surgical intervention does not increase odds of LMD; however, iatrogenic access to the CSF space during surgery is highly correlated with LMD development. Anatomic contact with the CSF space predicts LMD regardless of surgical status.

Subdural hematoma in glutaric aciduria type 1: High excreters are prone to incidental SDH despite newborn screening.

Subdural hematoma (SDH) was initially reported in 20% to 30% of patients with glutaric aciduria type 1 (GA1). A recent retrospective study found SDH in 4% of patients, but not in patients identified by newborn screening (NBS). 168 MRIs of 69 patients with GA1 (age at MRI 9 days - 73.8 years, median 3.2 years) were systematically reviewed for presence of SDH, additional MR and clinical findings in order to investigate the frequency of SDH and potential risk factors. SDH was observed in eight high-excreting patients imaged between 5.8 and 24.4months, namely space-occupying SDH in two patients after minor accidental trauma and SDH as an incidental finding in six patients without trauma. In patients without trauma imaged at 3 to 30 months (n=36, 25 NBS, 27/9 high/low excreters), incidence of SDH was 16.7% (16% in NBS). SDH was more common after acute (33.3%) than insidious onset of dystonia (14.3%) or in asymptomatic patients (5.9%). It was only seen in patients with wide frontoparietal CSF spaces and frontotemporal hypoplasia. High excreters were over-represented among patients with SDH (6/27 vs 0/9 low excreters), acute onset (10/12), and wide frontoparietal CSF spaces (16/19). Incidental SDH occurs despite NBS and early treatment in approximately one in six patients with GA1 imaged during late infancy and early childhood. Greater risk of high excreters is morphologically associated with more frequent enlargement of external CSF spaces including frontotemporal hypoplasia, and may be furthered aggravated by more pronounced alterations of cerebral blood volume and venous pressure.

Posterior cranial fossa and cervical spine morphometric abnormalities in symptomatic Chiari type 0 and Chiari type 1 malformation patients with and without syringomyelia

To better understand how anatomical features of Chiari malformation type 0 (CM0) result in the manifestation of Chiari malformation type 1 (CM1) signs and symptoms, we conducted a morphometric study of the posterior cranial fossa (PCF) and cervical canal in patients with CM1 and CM0. This retrospective study had a STROBE design and included 120 adult patients with MRI evidence of a small PCF (SPCF), typical clinical symptoms of CM1, and a diagnosis of CM1, CM0, or SPCF-TH0-only (SPCF with cerebellar ectopia less than 2mm and without syringomyelia). Patients were divided by MRI findings into 4 groups: SPCF-TH0-only, SPCF-TH0-syr (CM0 with SPCF and syringomyelia), SPCF-CM1-only (SPCF with cerebellar ectopia 5mm or more without syringomyelia), and SPCF-CM1-syr (CM1 with syringomyelia). Neurological examination data and MRI parameters were analyzed. All patient cohorts had morphometric evidence of a small, flattened, and overcrowded PCF. The PCF phenotype of the SPCF-TH0-only group differed from that of other CM cohorts in that the length of clivus and supraocciput and the height of the PF were longer, the upper CSF spaces of PCF were taller, and the area of the foramen magnum was smaller. The SPCF-TH0 groups had a more significant narrowing of the superior cervical canal and a smaller decrease in PCF height than the SPCF-CM1 groups. Patients with SPCF-TH0 with and without syringomyelia developed Chiari 1 symptoms and signs. Patients with SPCF-TH0-syr (Chiari 0) had more constriction of their CSF pathways in and around the foramen magnum than patients with SPCF-TH0-only.

Ventral Tonsillar Herniation Predicts Headaches in Adults With Chiari Malformation

Radiographic characterization of Chiari malformation (CM) has historically focused on caudal tonsillar herniation (CH) below the foramen magnum. Previously, we published evidence linking ventral tonsillar herniation (VH) and medullary symptoms in very young children. We sought to extend that investigation by studying the radiographic and clinical significance of VH in adults diagnosed with CM. We retrospectively reviewed adults with cerebellar ectopia who underwent posterior fossa decompression with or without duraplasty (PFD/D) at our institution. VH was defined as tonsils crossing a line bisecting the caudal medulla at the level of the foramen magnum on axial MRI. Degree of VH was measured as distance between this bisecting line and the ventral tip of the herniated tonsil. Dorsal brainstem compression was qualitatively determined by assessing for obliteration of CSF space between the dorsal brainstem and the tonsils. Out of 89 cases reviewed, 54 had some degree of VH. Compared with those without VH, the VH group was significantly older in age and more likely to also present with dorsal brainstem compression and headaches. No correlation was observed between degrees of CH and VH in the VH group. The degree of VH significantly decreased 3 months after PFD/D. VH is relatively common in CM patients and might be an important independent radiographic metric to evaluate and consider as part of the decision-making process, especially in those presenting with Chiari-like symptomatology referable to the medulla but who do not meet the traditional criteria of cerebellar ectopia greater than 5 mm.

CSF-space volumetric change following posterior fossa decompression in paediatric Chiari type-I malformation: a correlation with outcome.

We have previously reported inferior post-operative clinical outcomes in younger children with Chiari type-I malformation (CIM). We sought to quantify the CSF volumetric changes pre- and post-decompression, in a paediatric cohort, to determine whether cisternal volume change is associated with clinical outcomes. In this retrospective clinical study, the CSF spaces of the posterior fossa (supracerebellar/quadrigeminal, prepontine, fourth ventricle, cisterna magna) were measured on magnetic resonance images pre- and post-operatively using a semi-automated method. Additionally, we describe a novel CSF space of the upper cervical canal incorporating the subarachnoid space from the foramen magnum to the inferior cortex of the C2 body, FM-C2 cistern. Morphometric measurements included the pB-C2 distance, clivoaxial angle, clival length, clival angle and Boogard's angle. Volumetric and morphometric data were correlated with clinical outcomes at 4-12months post-operatively as measured by the Chicago Chiari Outcome Scale (CCOS). Of 59 adequate clinical cases, 57 and 36 patients had acceptable imaging for morphometric and volumetric analysis respectively. All CSF spaces measured had a significant increase in volume post-operatively (p < 0.05). There was no correlation between the change in volume or post-operative CSF volumes and CCOS. The pre-operative volume of the FM-C2 was positively correlated with total CCOS (Wald [Formula: see text], [Formula: see text]) and was significantly smaller in the 0-6-year age group (2.38 ± 1.27ml vs. 3.67 ± 1.56ml, p = 0.014). No morphometric measurement changed significantly after surgery or demonstrated a relationship with CCOS. Volumetric changes in the CSF cisterns of the posterior cranial fossa and upper cervical canal do not correlate with the age-related differences in clinical outcomes in paediatric CIM. The pre-operative volume of the FM-C2 cistern may have a role in predicting the likelihood of a beneficial post-operative outcome in paediatric CIM.

Neuroradiologic Evaluation of MRI in High-Contact Sports.

Background and Purpose: Athletes participating in high-contact sports experience repeated head trauma. Anatomical findings, such as a cavum septum pellucidum, prominent CSF spaces, and hippocampal volume reductions, have been observed in cases of mild traumatic brain injury. The extent to which these neuroanatomical findings are associated with high-contact sports is unknown. The purpose of this study was to determine whether there are subtle neuroanatomic differences between athletes participating in high-contact sports compared to low-contact athletic controls.Materials and Methods: We performed longitudinal structural brain MRI scans in 63 football (high-contact) and 34 volleyball (low-contact control) male collegiate athletes with up to 4 years of follow-up, evaluating a total of 315 MRI scans. Board-certified neuroradiologists performed semi-quantitative visual analysis of neuroanatomic findings, including: cavum septum pellucidum type and size, extent of perivascular spaces, prominence of CSF spaces, white matter hyperintensities, arterial spin labeling perfusion asymmetries, fractional anisotropy holes, and hippocampal size.Results: At baseline, cavum septum pellucidum length was greater in football compared to volleyball controls (p = 0.02). All other comparisons were statistically equivalent after multiple comparison correction. Within football at baseline, the following trends that did not survive multiple comparison correction were observed: more years of prior football exposure exhibited a trend toward more perivascular spaces (p = 0.03 uncorrected), and lower baseline Standardized Concussion Assessment Tool scores toward more perivascular spaces (p = 0.02 uncorrected) and a smaller right hippocampal size (p = 0.02 uncorrected).Conclusion: Head impacts in high-contact sport (football) athletes may be associated with increased cavum septum pellucidum length compared to low-contact sport (volleyball) athletic controls. Other investigated neuroradiology metrics were generally equivalent between sports.

LMD-19. Anatomic and Surgical Factors Predict Development of Leptomeningeal Disease in Patients with Metastatic Melanoma

BackgroundLeptomeningeal disease (LMD) is a devastating complication of systemic malignancy, portending a poor prognosis with an estimated median survival of 4–6 weeks if left untreated. Several reports have suggested surgical resection, particularly piecemeal resection, as a potential causative factor. Herein, we explore if surgical and anatomical factors are correlated with development of LMD in patients with melanoma brain metastases.MethodsPatients treated at our institution between 1999–2019 for primary melanoma with brain metastasis were compiled into a database based on ICD9/10 coding. 1,079 patients with melanoma brain metastases and appropriate imaging were identified, and 834 patients with a minimum of 3 months’ follow up were included. Patients were dichotomized by development of LMD or lack thereof. General demographic information, surgical and anatomic data, and ventricular access during surgery were investigated as possible correlative factors for the development of LMD.ResultsOn univariate analysis, female gender (p=0.033), presence of dural metastasis (p=0.018), presence of periventricular lesions (p<.001), presence of intraventricular lesions (p<.001), and ventricular access during surgery (p<.001) were significantly associated with LMD. Patients undergoing surgery, or those undergoing surgery without ventricular access, were not at higher risk of LMD. Administration of immunotherapy, either as first-line or salvage therapy, did not impact rates of LMD. On multivariate analysis, female gender (p=.033), presence of periventricular lesions (p<.001), presence of intraventricular lesions (p<.002), and presence of dural metastasis (p=0.032) were significantly associated with development of LMD. In patients who had surgery, iatrogenic ventricular access (p<.001) was significantly correlated with LMD.ConclusionsIn a retrospective cohort of patients with melanoma metastatic to the brain, those patients with pre-existing lesions in contact with the CSF space are more likely to develop LMD than those who do not. In addition, iatrogenic access to the CSF space during surgery is highly correlated with LMD development.

Imaging and health metrics in incidental cerebellar tonsillar ectopia: findings from the Adolescent Brain Cognitive Development Study (ABCD).

Incidental cerebellar tonsillar ectopia (ICTE) that meets the radiographic criterion for Chiari malformation type I (CMI) is an increasingly common finding in the clinical setting, but its significance is unclear. The present study examined posterior cranial fossa (PCF) morphometrics and a broad range of health instruments of pediatric ICTE cases and matched controls extracted from the Adolescent Brain Cognitive Development (ABCD) dataset. One-hundred-six subjects with ICTE and 106 matched controls without ICTE were identified from 11,411 anatomical MRI of healthy screened pediatric subjects from the ABCD project. Subjects were matched by sex, age, body mass index, race, and ethnicity. Twenty-two brain morphometrics and 22 health instruments were compared between the two groups to identify unrecognized CMI symptoms and assess the general health impact of ICTE. Twelve and 15 measures were significantly different between the ICTE and control groups for females and males, respectively. Notably, for females, the anterior CSF space was significantly smaller (p = 0.00005) for the ICTE group than controls. For males, the clivus bone length was significantly shorter (p = 0.0002) for the ICTE group compared to controls. No significant differences were found among the 22 health instruments between the two groups. This study demonstrated that pediatric ICTE subjects have similar PCF morphometrics to adult CMI. ICTE alone did not appear to cause any unrecognized CMI symptoms and had no impact on the subjects' current mental, physical, or behavioral health. Still, given their cranial and brain morphology, these cases may be at risk for adult-onset symptomatic CMI.

Adult Age Differences in Self-Reported Pain and Anterior CSF Space in Chiari Malformation.

Chiari malformation type I (CMI) is a neural disorder with sensory, cognitive, and motor defects, as well as headaches. Radiologically, the cerebellar tonsils extend below the foramen magnum. To date, the relationships among adult age, brain morphometry, surgical status, and symptom severity in CMI are unknown. The objective of this study was to better understand the relationships among these variables using causal modeling techniques. Adult CMI patients (80% female) who either had (n = 150) or had not (n = 151) undergone posterior fossa decompression surgery were assessed using morphometric measures derived from magnetic resonance images (MRI). MRI-based morphometry showed that the area of the CSF pocket anterior to the cervico-medullary junction (anterior CSF space) correlated with age at the time of MRI (r = - .21). Also, self-reported pain increased with age (r = .11) and decreased with anterior CSF space (r = - .18). Age differences in self-reported pain were mediated by anterior CSF space in the cervical spine area-and this effect was particularly salient for non-decompressed CMI patients. As CMI patients age, the anterior CSF space decreases, and this is associated with increased pain-especially for non-decompressed CMI patients. It is recommended that further consideration of age-related decreases in anterior CSF space in CMI patients be given in future research.

Blind spots on CT imaging of the head: Insights from 5 years of report addenda at a single institution

BackgroundErrors of detection (“misses”) are the major source of error in radiology. There is sparse prior literature describing patterns of detection error on CT head imaging. PurposeThe objective of this study was to gain insight to areas on CT head imaging where radiologists are most likely to miss clinically relevant findings. MethodsWe performed a cross-sectional study of consecutive reports of CT imaging of the head at a single institution spanning 5/1/2013–5/1/2018 (5 years). Detection errors described in addenda were categorized according to anatomic location, type of pathology, and potential impact on management. Blind spots were defined by the most common sites of missed findings. ResultsA total of 165,943 reports for CT head imaging were obtained. Addenda were found in 1658 (~1%) of reports, of which 359 (21.7%) described errors of detection. Within the extracranial soft tissues (n = 73) the most common “misses” were at incidentally imaged parotid glands and the frontal scalp. Within osseous structures (n = 149), blind spots included the nasal and occipital bones. Vascular lesions (n = 47) which passed detection were most common at the distal MCA, carotid terminus and sigmoid sinus/jugular bulb. No predisposition was seen for anatomic subsites within the CSF space (n = 60) and brain parenchyma (n = 65). ConclusionsConsistent patterns of blind spots are revealed. Radiologic teaching and search patterns to account for these sites of error may accelerate trainee competence and improve accuracy in the practice of radiology.

P0619 / #1524: ACUTE NECROTIZING ENCEPHALOPATHY (ANE) RELATED TO INFLUENZA AND MYCOPLASMA PNEUMONIAE IN A CHILD

Aims & Objectives: Background: ANE is a rare neurologic complication of influenza which lead to profound disability or death.The co-infection role of M. pneumoniae on this condition has not been previously reported. Aims: Clinical case presentation. Methods: Methodology: Chart review and literature search. Results: A previously healthy 5-year-old female, 3 hours before admission presents with fever, restlessness, and an involuntary bowel movement. She became confused and developed right hemiparesis and dysarthria. Soon after arrival to the ER, she developed right-sided clonic seizures, coma, bilateral areactive mydriasis with no corneal reflex, and hypotension. An admission brain-CT (Fig. 1)scan reported no intracranial abnormalities, but a MRI (Fig. 2), performed 2 hours after stabilization, reported diffuse hyperintensity with cytotoxic edema in cerebellar hemispheres, posterior brainstem, thalamus, basal ganglia, gray and white supra and infratentorial substance, findings consistent with ANE. Despite decompressive craniotomy and maximum therapeutic efforts, intracranial hypertension was severe and refractory to treatment. A PCR respiratory panel was positive for Influenza A/H1-2009, A/H1-pan, A/pan1, A/pan2 and M. pneumoniae. Blood and urine cultures were negative. A CT scan after 12 hours reported total obliteration of the CSF spaces, occipital transcranial herniation, and absence of intracranial circulation. The patient died after 36 hours. A post-mortem CSF analysis, including a PCR panel and culture, were negative.Conclusions: ANE is a consequence of an inappropriate immune response to virus and bacteria associated with poor outcomes or severe complications. More research is needed to stablish an association with a genetic predisposition.

CSF dynamics as a predictor of cognitive progression

Disproportionately enlarged subarachnoid-space hydrocephalus (DESH), characterized by tight high convexity CSF spaces, ventriculomegaly, and enlarged Sylvian fissures, is thought to be an indirect marker of a CSF dynamics disorder. The clinical significance of DESH with regard to cognitive decline in a community setting is not yet well defined. The goal of this work is to determine if DESH is associated with cognitive decline. Participants in the population-based Mayo Clinic Study of Aging (MCSA) who met the following criteria were included: age ≥ 65 years, 3T MRI, and diagnosis of cognitively unimpaired or mild cognitive impairment at enrollment as well as at least one follow-up visit with cognitive testing. A support vector machine based method to detect the DESH imaging features on T1-weighted MRI was used to calculate a “DESH score”, with positive scores indicating a more DESH-like imaging pattern. For the participants who were cognitively unimpaired at enrollment, a Cox proportional hazards model was fit with time defined as years from enrollment to first diagnosis of mild cognitive impairment or dementia, or as years to last known cognitively unimpaired diagnosis for those who did not progress. Linear mixed effects models were fit among all participants to estimate annual change in cognitive z scores for each domain (memory, attention, language, and visuospatial) and a global z score. For all models, covariates included age, sex, education, APOE genotype, cortical thickness, white matter hyperintensity volume, and total intracranial volume. The hazard of progression to cognitive impairment was an estimated 12% greater for a DESH score of +1 versus −1 (HR 1.12, 95% CI 0.97–1.31, p = 0.11). Global and attention cognition declined 0.015 (95% CI 0.005–0.025) and 0.016 (95% CI 0.005–0.028) z/year more, respectively, for a DESH score of +1 vs −1 (p = 0.01 and p = 0.02), with similar, though not statistically significant DESH effects in the other cognitive domains. Imaging features of disordered CSF dynamics are an independent predictor of subsequent cognitive decline in the MCSA, among other well-known factors including age, cortical thickness, and APOE status. Therefore, since DESH contributes to cognitive decline and is present in the general population, identifying individuals with DESH features may be important clinically as well as for selection in clinical trials.

Spinal Compliance Curves: Preliminary Experience with a New Tool for Evaluating Suspected CSF Venous Fistulas on CT Myelography in Patients with Spontaneous Intracranial Hypotension.

Craniospinal space compliance reflects the distensibility of the spinal and intracranial CSF spaces as a system. Craniospinal space compliance has been studied in intracranial pathologies, but data are limited in assessing it in spinal CSF leak. This study describes a method to estimate craniospinal space compliance using saline infusion during CT myelography and explores the use of craniospinal space compliance and pressure-volume curves in patients with suspected cerebrospinal-venous fistula. Patients with suspected cerebrospinal-venous fistula underwent dynamic CT myelography. During the procedure, 1- to 5-mL boluses of saline were infused, and incremental changes in CSF pressure were recorded. These data were used to plot craniospinal space compliance curves. We calculated 3 quantitative craniospinal space compliance parameters: overall compliance, compliance at opening pressure, and the pressure volume index. These variables were compared between patients with confirmed cerebrospinal-venous fistula and those with no confirmed source of CSF leak. Thirty-four CT myelograms in 22 patients were analyzed. Eight of 22 (36.4%) patients had confirmed cerebrospinal-venous fistulas. Bolus infusion was well-tolerated with no complications and transient headache in 2/34 (5.8%). Patients with confirmed cerebrospinal-venous fistulas had higher compliance at opening pressure and overall compliance (2.6 versus 1.8 mL/cm H20, P < .01). There was no difference in the pressure volume index (77.5 versus 54.3 mL, P = .13) between groups. A method of deriving craniospinal space compliance curves using saline intrathecal infusion is described. Preliminary analysis of craniospinal space compliance curves provides qualitative and quantitative information about pressure-volume dynamics and may serve as a diagnostic tool in patients with known or suspected cerebrospinal-venous fistulas.

Novel in vivo depiction of optic nerves hemorrhages in child abuse: a 3D-SWI pilot study

PurposeUntil now, the diagnosis of optic nerves hemorrhages in abusive head trauma (AHT) has been obtained only in the postmortem setting. The aim of the IRB-approved study was to assess the presence of optic nerves hemorrhages in AHT patients using 3D-SWI.MethodsThirteen children with a final confirmed multidisciplinary diagnosis of AHT underwent coronal and axial 3D-SWI imaging of the orbits. The presence of optic nerve sheath (ONS) hemorrhages was defined by thickening and marked 3D-SWI hypointensity of the ONS, resulting in mass effect upon the CSF space. Optic nerve (ON) hemorrhages were defined by areas of susceptibility artifacts in the ON parenchyma. Superficial siderosis was defined by susceptibility artifact coating the ON. Furthermore, data about post-traumatic deformity of the ONS at the head of the optic nerve were collected.ResultsThe average age of the population was 7.9 ± 5.9 months old. The average GCS was 11.8 ± 4.5. The male to female ratio was 7:6. ONS hemorrhages were identified in 69.2% of cases. Superficial siderosis and ON hemorrhages were identified in 38.5 and 76.9% of cases, respectively. 3D-SWI also depicted traumatic deformity of the ONS at the level of the optic nerve head in 10 cases (76.9%). No statistical correlations were identified between RetCam findings and 3D-SWI findings or GCS and ON hemorrhages.ConclusionThis research shows that dedicated MRI with volumetric SWI of the orbits can depict hemorrhages in the ON, ONS, and ONS injury, in AHT victims.

GCT-61. CORRELATION OF PATTERNS OF DISEASE RECURRENCE WITH RADIOTHERAPY TECHNIQUES AND DOSE IN INTRACRANIAL GERM CELL TUMOURS (icGCT): LESSONS FROM THE UK COHORT OF SIOP GCT96 STUDY

BACKGROUNDThere are global variations in radiotherapy approaches for icGCT. An understanding of patterns of disease recurrence correlated with radiation techniques and doses is important in standardising and improving the quality of radiotherapy using high-precision techniques.METHODS AND RESULTSData from 20 patients with tumour recurrence after treatment within the SIOP GCT96 study in the UK were analysed. Seven (35%) patients had germinoma and 13 (65%) had non-germinoma. Twelve patients had local recurrence, 5 had metastatic and 3 had local and metastatic disease. Radiotherapy details were retrieved in only 8 patients (40%). Six patients had received focal radiotherapy and two craniospinal radiotherapy. Of the patients who received focal radiotherapy, 4 had recurrence within the radiation portal, one had periventricular recurrence and one had marker-positive recurrence with no radiological lesions. Both patients who received CSI recurred within the CSF space. The main reasons for poor retrieval of treatment details were difficulty in retrieving archived information and that the study was conducted during a period before PACS or electronic radiotherapy records.CONCLUSIONThis study highlights the importance prospective data collection and analysis to understand the patterns of recurrence in icGCT. Even within a prospective study, radiotherapy techniques varied between centres. There is therefore an urgent need for centralised radiological review and prospective radiotherapy quality assurance measures in future clinical trials.

White matter alteration and cerebellar atrophy are hallmarks of brain MRI in alpha-mannosidosis

ObjectiveDespite profound neurological symptomatology there are only few MRI studies focused on the brain abnormalities in alpha-mannosidosis (AM). Our aim was to characterize brain MRI findings in a large cohort of AM patients along with clinical manifestations. MethodsTwenty-two brain MRIs acquired in 13 untreated AM patients (8 M/5F; median age 17 years) were independently assessed by three experienced readers and compared to 16 controls. ResultsFocal and/or diffuse hyperintense signals in the cerebral white matter were present in most (85%) patients. Cerebellar atrophy was common (62%), present from the age of 5 years. Progression was observed in two out of 6 patients with follow-up scans. Cortical atrophy (62%) and corpus callosum thinning (23%) were already present in a 13-month-old child. The presence of low T2 signal intensity in basal ganglia and thalami was excluded by the normalized signal intensity profiling. The enlargement of perivascular spaces in white matter (38%), widening of perioptic CSF spaces (62%), and enlargement of cisterna magna (85%) were also observed. Diploic space thickening (100%), mucosal thickening (69%) and sinus hypoplasia (54%) were the most frequent non-CNS abnormalities. ConclusionWhite matter changes and cerebellar atrophy are proposed to be the characteristic brain MRI features of AM. The previously reported decreased T2 signal intensity in basal ganglia and thalami was not detected in this quantitative study. Rather, this relative MR appearance seems to be related to the diffuse high T2 signal in the adjacent white matter and not the gray matter iron deposition that has been hypothesized.

Scratching your head over extra-axial CSF spaces?

Scratching your head over extra-axial CSF spaces?