Transient Decrease in Cerebrospinal Fluid Motion Is Related to Cough-Associated Headache in Chiari I Malformation.

BACKGROUND: Short-lasting cough-associated headache (CAH) in patients with Chiari I malformation (CMI) is believed to be due to transient worsening of cerebrospinal flow (CSF) obstruction at the foramen magnum. We assessed changes in CSF flow in response to coughing in CMI patients with CAH and compared with those without CAH and healthy participants (HPs) using real-time magnetic resonance imaging. METHODS: Seventeen CMI patients (12 with CAH, 5 without CAH) and 6 HPs were prospectively assessed using real-time pencil-beam imaging magnetic resonance sequence. A 64-mm length pencil-beam imaging cylinder was placed at the craniocervical junction. CSF stroke volume (SVCSF) was assessed during resting, postcoughing, and relaxation phases via a 90-second scan. SVCSF was measured at 6 levels at 5-mm intervals between 10 and 35 mm below the foramen magnum. During each phase, SVCSF was compared between CMI with and without CAH and HPs and corrected for multiple comparisons. RESULTS: At multiple consecutive levels, postcoughing SVCSF was significantly lower in CMI with CAH compared with both CMI without CAH and HP (P < 0.05). No differences in postcoughing SVCSF were seen between CMI without CAH and HP. At rest or relaxation phase, no differences in SVCSF were seen between patients with and without CAH but minimal differences were seen between CMI with CAH and HP. CONCLUSIONS: A decrease in CSF flow after coughing in CMI patients with CAH supports the notion that CAH is caused by transient worsening of CSF flow obstruction at the foramen magnum.

Relationship of Morphometrics and Symptom Severity in Female Type I Chiari Malformation Patients with Biological Resilience.

In the present study we report the relationship among MRI-based skull and cervical spine morphometric measures as well as symptom severity (disability-as measured by Oswestry Head and Neck Pain Scale and social isolation-as measured by the UCLA Loneliness scale) on biomarkers of allostatic load using estrogen, interleukin-6, C-reactive protein, and cortisol in a sample of 46 CMI patients. Correlational analyses showed that McRae line length was negatively associated with interleukin-6 and C-reactive protein levels, and Analysis of Variance (ANOVA) showed joint effects of morphometric measures (McRae line length, anterior CSF space) and symptom severity (disability and loneliness) on estrogen and intereukin-6 levels. These results are consistent with allostatic load. That is, when the combination of CSF crowding and self-report symptom (disability and loneliness) severity exceed the capacity of biological resilience factors, then biomarkers such as neuroprotective estrogen levels drop, rather than rise, with increasing symptom severity.

The Effect of Posterior Fossa Decompression Surgery on Brainstem and Cervical Spinal Cord Dimensions in Adults with Chiari Malformation Type 1.

OBJECTIVE: Posterior fossa decompression (PFD) surgery creates more space at the skull base, reduces the resistance to the cerebrospinal fluid motion, and alters craniocervical biomechanics. In this paper, we retrospectively examined the changes in neural tissue dimensions following PFD surgery on Chiari malformation type 1 adults. METHODS: Measurements were performed on T2-weighted brain magnetic resonance images acquired before and 4 months after surgery. Measurements were conducted for neural tissue volume and spinal cord/brainstem width at 4 different locations; 2 width measurements were made on the brainstem and 2 on the spinal cord in the midsagittal plane. Cerebellar tonsillar position (CTP) was also measured before and after surgery. RESULTS: Twenty-five adult patients, with a mean age of 38.9 ± 8.8 years, were included in the study. The cervical cord volume increased by an average of 2.3 ± 3.3% (P = 0.002). The width at the pontomedullary junction increased by 2.2 ± 3.5% (P < 0.01), while the width 10 mm caudal to this junction increased by 4.2 ± 3.9% (P < 0.0001). The spinal cord width at the base of second cervical vertebra and third cervical vertebra did not significantly change after surgery. The CTP decreased by 60 ± 37% (P < 0.0001) after surgery, but no correlation was found between CTP change and dimension change. CONCLUSIONS: The brainstem width and cervical cord volume showed a modest increase after PFD surgery, although standard deviations were large. A reduction in compression after PFD surgery may allow for an increase in neural tissue dimension. However, clinical relevance is unclear and should be assessed in future studies with high-resolution imaging.

Prospective, Longitudinal Study of Clinical Outcome and Morphometric Posterior Fossa Changes after Craniocervical Decompression for Symptomatic Chiari I Malformation.

BACKGROUND AND PURPOSE: The time course of changes in posterior fossa morphology, quality of life, and neurologic function of patients with Chiari I malformation after craniocervical decompression requires further elaboration. To better understand the pace of these changes, we longitudinally studied patients with Chiari I malformation, with or without syringomyelia, before and after the operation for up to 5 years. MATERIALS AND METHODS: Thirty-eight symptomatic adult patients (35 women, 3 men) diagnosed with Chiari I malformation only (n = 15) or Chiari I malformation and syringomyelia (n = 23) and without previous Chiari I malformation surgery were enrolled in a clinical study. Patients underwent outpatient study visits and MR imaging at 7 time points (ie, initial [before the operation], 3 months, 1 year, 2 years, 3 years, 4 years, and 5 years) during 5 years. The surgical procedure for all patients was suboccipital craniectomy, C1 laminectomy, and autologous duraplasty. RESULTS: Morphometric measurements demonstrated an enlargement of the CSF areas posterior to the cerebellar tonsils after the operation, which remained largely stable through the following years. There was a decrease in pain and improved quality of life after the operation, which remained steady during the following years. Reduction in pain and improved quality of life correlated with CSF area morphometrics. CONCLUSIONS: Most changes in MR imaging morphometrics and quality of life measures occurred within the first year after the operation. A 1-year follow-up period after Chiari I malformation surgery is usually sufficient for evaluating surgical efficacy and postoperative MR imaging changes.

Association between resistance to cerebrospinal fluid flow and cardiac-induced brain tissue motion for Chiari malformation type I.

PURPOSE: Chiari malformation type I (CMI) patients have been independently shown to have both increased resistance to cerebrospinal fluid (CSF) flow in the cervical spinal canal and greater cardiac-induced neural tissue motion compared to healthy controls. The goal of this paper is to determine if a relationship exists between CSF flow resistance and brain tissue motion in CMI subjects. METHODS: Computational fluid dynamics (CFD) techniques were employed to compute integrated longitudinal impedance (ILI) as a measure of unsteady resistance to CSF flow in the cervical spinal canal in thirty-two CMI subjects and eighteen healthy controls. Neural tissue motion during the cardiac cycle was assessed using displacement encoding with stimulated echoes (DENSE) magnetic resonance imaging (MRI) technique. RESULTS: The results demonstrate a positive correlation between resistance to CSF flow and the maximum displacement of the cerebellum for CMI subjects (r = 0.75, p = 6.77 × 10-10) but not for healthy controls. No correlation was found between CSF flow resistance and maximum displacement in the brainstem for CMI or healthy subjects. The magnitude of resistance to CSF flow and maximum cardiac-induced brain tissue motion were not statistically different for CMI subjects with and without the presence of five CMI symptoms: imbalance, vertigo, swallowing difficulties, nausea or vomiting, and hoarseness. CONCLUSION: This study establishes a relationship between CSF flow resistance in the cervical spinal canal and cardiac-induced brain tissue motion in the cerebellum for CMI subjects. Further research is necessary to understand the importance of resistance and brain tissue motion in the symptomatology of CMI.

The Relationship Between Imbalance Symptom and Cardiac Pulsation Induced Mechanical Strain in the Brainstem and Cerebellum for Chiari Malformation Type I.

Chiari malformation Type I (CMI) is known to have an altered biomechanical environment for the brainstem and cerebellum; however, it is unclear whether these altered biomechanics play a role in the development of CMI symptoms. We hypothesized that CMI subjects have a higher cardiac-induced strain in specific neurological tracts pertaining to balance, and postural control. We measured displacement over the cardiac cycle using displacement encoding with stimulated echoes magnetic resonance imaging in the cerebellum, brainstem, and spinal cord in 37 CMI subjects and 25 controls. Based on these measurements, we computed strain, translation, and rotation in tracts related to balance. The global strain on all tracts was small (<1%) for CMI subject and controls. Strain was found to be nearly doubled in three tracts for CMI subjects compared to controls (p < 0.03). The maximum translation and rotation were ∼150 µm and ∼1 deg, respectively and 1.5-2 times greater in CMI compared to controls in four tracts (p < 0.005). There was no significant difference between strain, translation, and rotation on the analyzed tracts in CMI subjects with imbalance compared to those without imbalance. A moderate correlation was found between cerebellar tonsillar position and strain on three tracts. The lack of statistically significant difference between strain in CMI subjects with and without imbalance could imply that the magnitude of the observed cardiac-induced strain was too small to cause substantial damage to the tissue (<1%). Activities such as coughing, or Valsalva may produce a greater strain.

Cerebrospinal Fluid Flow and Brain Motion in Chiari I Malformation: Past, Present, and Future.

Cranio-spinal volume and pressure changes associated with the cardiac-cycle and respiration are altered in Chiari I malformation (CMI) due to obstruction of cerebrospinal fluid (CSF) flow at the foramen magnum. With the introduction of motion-sensitive MRI sequences, it was envisioned that these could provide noninvasive information about volume-pressure dynamics at the cranio-cervical junction in CMI hitherto available only through invasive pressure measurements. Since the early 1990s, multiple studies have assessed CSF flow and brain motion in CMI. However, differences in design and varied approaches in the presentation of results and conclusions makes it difficult to fully comprehend the role of MR imaging of CSF flow and brain motion in CMI. In this review, a cohesive summary of the current status of MRI assessment of CSF flow and brain motion in CMI is presented. Simplified versions of the results and conclusions of previous studies are presented by dividing the studies in distinct topics: 1) comparing CSF flow and brain motion between healthy subjects (HS) and CMI patients (before and after surgery), 2) comparing CSF flow and brain motion to CMI severity and symptoms, and 3) comparing CSF flow and brain motion in CMI with and without syringomyelia. Finally, we will discuss our vision of the future directions of MR imaging in CMI patients. EVIDENCE LEVEL: 2. TECHNICAL EFFICACY: 5.

Pain and Depression Account for More Than One Half of the Neck Disability Variance Among Adult Women with Chiari I.

OBJECTIVE: We sought to quantify the neck-related disability in adult women with Chiari malformation type I and identify the significantly related variables. METHODS: A total of 70 variables were selected from the self-report history questionnaires (12 variables; n = 474), standardized scales (15 variables; n = 474), and morphometric data (43 variables; n = 293-474) of adult women with Chiari malformation type I. The variables were tested independently to identify those with a significant relationship to the Neck Disability Index (NDI; P < 0.00071) and those that might be associated with the NDI (P < 0.05). A forward selection regression model was constructed to identify the variables contributing unique variance to the NDI. In addition, a mediation analysis was performed to determine whether depression mediated the relationship between pain and disability. RESULTS: Overall, 79.5% of the patients had scored at a moderate level of disability or higher. Independent testing identified 16 significant variables, including symptom duration, tonsillar position, and measures of psychological distress. The short-form McGill pain questionnaire-2 (r = 0.69; P < 0.00001) and CES-D scale for depression (r = 0.56; P < 0.00001) exhibited the highest correlations with the NDI. The forward selection regression model produced an R2 of 0.6178. Pain and depression accounted for more than one half of the NDI variance. CONCLUSIONS: We found that high levels of disability are common among adult women with Chiari malformation type I, independent of surgical status. Pain and depression were the primary factors related to this disability. Depression mediated the relationship between pain intensity and disability at a modest level (5%). Patients who had experienced symptoms for >2 years before diagnosis had had, on average, 77% higher NDI scores, highlighting the importance of a timely diagnosis.

Modifying the ICP pulse wave: effects on parenchymal blood flow pulsatility.

Pulsation of the cerebral blood flow (CBF) produces intercranial pressure (ICP) waves. The aim of this study is to determine whether externally modifying ICP pulsatility alters parenchymal blood flow pulsatility. A cardiac-gated inflatable device was inserted in the lateral epidural space of 12 anesthetized canines (canis familiaris) and used to cause reduction, inversion, and augmentation of the ICP pulse. CBF in each hemisphere was measured using laser Doppler velocimetry. A significant increase in both mean CBF and its amplitude was observed for reduction as well as inversion of the ICP pulse, with larger changes observed for the inversion protocol. Significant increases in the mean CBF were also observed ipsilaterally for the augmentation protocol together with indications of reduced CBF amplitude contralaterally. External alteration of the ICP pulse thus caused significant changes in parenchymal blood flow pulsatility. The inverse relationship between the ICP and CBF amplitude suggests that the changes did not occur via modification of the intracranial Windkessel mechanism. Thus, the effects likely occurred in the low-pressure vessels, i.e., capillaries and/or venules, rather than the high-pressure arteries. Future MRI studies are however required to map and quantify the effects on global cerebral blood flow.NEW & NOTEWORTHY This study demonstrated that external modification of ICP pulsatility, using a cardiac-gated inflatable device implanted epidurally in canines, alters brain tissue blood flow pulsatility. Specifically, decreasing systolic ICP increased blood flow pulsatility in brain tissue. The results suggest that the altered CBF pulsatility is unlikely to depend on modification of the Windkessel effect on the feeding arterial system but was rather an effect directly on tissue and the lower pressure distal vessels.

Impact of Blood Rheology on Transition to Turbulence and Wall Vibration Downstream of a Stenosis.

Previous experimental flow studies have demonstrated a delay (∼20%) in transition to turbulence for whole blood compared to a Newtonian analog fluid in both a straight pipe and eccentric stenosis model with ridged walls. The impact of wall compliance on the transition to turbulence of blood compared to Newtonian analog and on wall vibration is unknown. The present study employed flexible walls downstream of an eccentric stenosis model and examined the wall vibration during the transition to turbulence with whole blood and a Newtonian analog. Measurements of tube wall vibration velocity (WVV) were used as an indicator of the turbulence level within the flexible tube. WVV was measured at 5, 10, and 15 diameters downstream of the stenosis using a laser Doppler vibrometer at Reynolds numbers 0, 200, 300, 350, 400, 450, 500, 550, 600, 650, 700, and 750. The root mean squares (RMS) of the measured WVV were utilized as an indirect measure of fluid velocity fluctuations present at that location, and hence, an indicator of transition to turbulence. WVV RMS was near-constant until approximately Reynolds number 400. It increased monotonically with Reynolds number for both whole blood and the Newtonian fluid. No differences in the transition to turbulence were observed between whole blood and the Newtonian fluid, as the WVV RMS curves were remarkably similar in shape. This result suggests that rheology had minimal impact on the WVV downstream of a stenosis for transition to turbulence since the fluids had a similar level of vibration.

The importance of precise plane selection for female adult Chiari Type I malformation midsagittal morphometrics.

INTRODUCTION: Morphometric assessment of Chiari malformation type I (CMI) is typically performed on a midsagittal MRI. However, errors arising from an imprecise selection of the midsagittal plane are unknown. We define absolute parasagittal error as the absolute difference between morphometric measurements at the midsagittal and parasagittal planes. Our objective was to determine the absolute parasagittal error at various lateral distances for morphometric parameters commonly used in CMI research. METHODS: Sagittal T1-weighted MRI scans of 30 CMI adult female subjects were included. Image sets were evaluated to assess 14 CMI morphometric parameters in the midsagittal plane and four parasagittal planes located 1 and 2 mm lateral (left and right). Comparisons between measurements at the midsagittal and parasagittal planes were conducted to determine the mean individual absolute and mean group parasagittal errors for all 14 parameters. RESULTS: The mean individual absolute parasagittal error was > 1 unit (1 mm for lengths and 1 degree for angles) for 9/14 parameters within a lateral distance of 2 mm. No significant group parasagittal errors were seen in 14/14 parameters, including tonsillar position within a lateral distance of 2 mm. CONCLUSION: Our results show that the absolute errors for imprecise midsagittal plane selection may impact the clinical assessment of an individual patient. However, the impact on group measurements, such as in a research setting, will be minimal.

Influence of Pain on Cognitive Dysfunction and Emotion Dysregulation in Chiari Malformation Type I.

It has been well demonstrated that the cerebellum is associated with cognitive and affective processing as well as the traditionally conceptualized motor function. In the present chapter, we explore the behavioral and neurobiological implications of a common congenital cerebellar condition, Chiari malformation Type I, on cognitive and affective processing. We also emphasize the associations between Chiari-related chronic pain, cognitive dysfunction, and emotion dysregulation. Based on our review of the literature, we argue that chronic pain can account for a substantial amount of the cognitive dysfunction and emotion dysregulation in Chiari malformation Type I. Yet, there also exists aspects of Chiari-related cognitive dysfunction and emotion dysregulation that appear to be at least partially independent of chronic pain and more directly associated with abnormalities in cerebrospinal fluid flow dynamics and cerebro-cerebellar communication pathways.

MRI findings differentiating tonsillar herniation caused by idiopathic intracranial hypertension from Chiari I malformation.

PURPOSE: Some patients with idiopathic intracranial hypertension (IIH) have cerebellar tonsillar herniation ≥ 5 mm mimicking Chiari malformation I (CMI), which can result in misdiagnosis and unjustified treatment. Our purpose was to identify IIH patients with tonsillar herniation ≥ 5 mm (IIHTH) and compare with CMI patients to assess imaging findings that could distinguish the two conditions. METHODS: Ninety-eight patients with IIH, 81 patients with CMI, and 99 controls were retrospectively assessed. Two neuroradiologists blindly reviewed MR images. IIHTH patients were compared with CMI patients and controls regarding the extent of tonsillar herniation (ETH), bilateral transverse sinus stenosis (BTSS), hypophysis-sella ratio (HSR), and bilateral tortuosity of optic nerve (BTON). RESULTS: 13/98 (13.2%) IIH patients had tonsillar herniation ≥ 5 mm (IIHTH) and were significantly younger and had higher BMI compared with CMI patients and controls. ETH was significantly less in the IIHTH than CMI (6.5 ± 2.4 mm vs. 10.9 ± 4.4 mm; p < 0.001). BTSS and HSR < 0.5 were more common in IIHTH than CMI (p < 0.001 and p = 0.003, respectively). No differences were seen between CMI and controls. BTON was significantly more common in IIHTH compared to control (p = 0.01) but not to the CMI (p = 0.36). Sensitivity and specificity to differentiate IIHTH from CMI were 69.2% and 96.1% for BTSS and 69.2% and 75.3% for HSR < 0.5. CONCLUSION: The presence of BTSS and/or HSR < 0.5 in patients with ETH ≥ 5 mm should suggest further evaluation to exclude IIH before considering CMI surgery.

Factors Associated With Patient-Reported Postsurgical Symptom Improvement in Adult Females with Chiari Malformation Type I: A Report from the Chiari1000 Dataset.

OBJECTIVE: The goal of this study was to assess if a broad array of factors is predictive of patient-reported surgical impact on symptoms. METHODS: Sixty-five presurgical factors were selected from the self-report history questionnaires (12 variables, N = 653), standardized scales (14 variables, N = 494-581), and morphometric measurements from magnetic resonance imaging (39 variables, N = 137) data of adult women in the Chiari1000 dataset. The factors were tested independently to identify those that were either significantly different in terms of, or significantly related to, patient-reported surgical impact on symptoms (P < 0.05). These results were then used to build a forward selection regression model. RESULTS: Overall, 74.4% of the patients reported either some level of improvement or complete resolution of symptoms. Independent testing identified 12 significant factors, including having a family member diagnosed with Chiari malformation type I (CMI), Ehlers-Danlos syndrome, migraines, duration of symptoms >2 years, multiple surgeries, symptom severity, depression, anxiety, stress, and loneliness. Depression showed the highest correlation (r = 0.36; P < 0.00001) with a negative outcome. The regression model produced an R2 = 0.346 and identified 5 factors with significant unique variance. CONCLUSIONS: The regression model accounted for more than a third of the surgical impact variance. Having a family member diagnosed with CMI contributed the largest unique variance to the model, suggesting that hereditary CMI may represent a unique subset of patients with poorer outcomes. Independent testing showed that psychological factors, such as depression and anxiety, were significant negative predictors, indicating that presurgical screening and treatment for these psychological conditions may improve outcomes.

A new hypothesis for the pathophysiology of symptomatic adult Chiari malformation Type I.

Chiari malformation Type I (CMI) is characterized by herniation of the cerebellar tonsils through the foramen magnum. The pathophysiology of CMI is not well elucidated; however, the prevailing theory focuses on the underdevelopment of the posterior cranial fossa which results in tonsillar herniation. Symptoms are believed to be due to the herniation causing resistance to the natural flow of cerebrospinal fluid (CSF) and exerting a mass effect on nearby neural tissue. However, asymptomatic cases vastly outnumber symptomatic ones and it is not known why some people become symptomatic. Recently, it has been proposed that CMI symptoms are primarily due to instability of either the atlanto-axial (AA) or the atlanto-occipital (AO) joint and the cerebellar tonsils herniate to prevent mechanical pinching. However, only a small percentage of patients exhibit clinical instability and these theories do not account for asymptomatic herniations. We propose that the pathophysiology of adult CMI involves a combination of craniocervical abnormalities which leads to tonsillar herniation and reduced compliance of the cervical spinal canal. Specifically, abnormal AO and/or AA joint morphology leads to chronic cervical instability, often subclinical, in a large portion of CMI patients. This in turn causes overwork of the suboccipital muscles as they try to compensate for the instability. Over time, the repeated, involuntary activation of these muscles leads to mechanical overload of the myodural bridge complex, altering the mechanical properties of the dura it merges with. As a result, the dura becomes stiffer, reducing the overall compliance of the cervical region. This lower compliance, combined with CSF resistance at the same level, leads to intracranial pressure peaks during the cardiac cycle (pulse pressure) that are amplified during activities such as coughing, sneezing, and physical exertion. This increase in pulse pressure reduces the compliance of the cervical subarachnoid space which increases the CSF wave speed in the spinal canal, and further increases pulse pressure in a feedback loop. Finally, the abnormal pressure environment induces greater neural tissue motion and strain, causing microstructural damage to the cerebellum, brainstem, and cervical spinal cord, and leading to symptoms. This hypothesis explains how the combination of craniocervical bony abnormalities, anatomic CSF restriction, and reduced compliance leads to symptoms in adult CMI.

Diagnostic utility of parasagittal measurements of tonsillar herniation in Chiari I malformation.

BACKGROUND AND PURPOSE: Although the cerebellar tonsils are parasagittal structures, the extent of tonsillar herniation (ETH) in Chiari I malformation (CMI) is currently measured in the midsagittal plane. We measured the ETH of each cerebellar tonsil in the parasagittal plane and assessed their diagnostic utility by comparing them to the midsagittal ETH measurements in predicting cough-associated headache (CAH), an indicator of clinically significant disease in CMI. METHODS: Eighty-five CMI patients with 3D-MPRAGE images were included. Neurosurgeons determined the presence of CAH. Sagittal images were used to measure ETH in the midsagittal (MS_ETH) and parasagittal planes (by locating tonsillar tips on each side on reformatted coronal images). Given the parasagittal ETH (PS_ETH) asymmetry in the majority of cases, they were considered Smaller_PS_ETH or Larger_PS_ETH. The accuracy of ETH measurements was assessed by the receiver operating characteristic (ROC) curve. RESULTS: Of 85 patients, 46 reported CAH. ROC analysis showed an area under the curve (AUC) of 0.78 for Smaller_PS_ETH significantly better than 0.65 for MS-ETH in predicting CAH (p = 0.001). An AUC of 0.68 for Larger_PS_ETH was not significantly different from MS_ETH. The sensitivity and specificity of predicting CAH were 87% and 28% for MS_ETH >6 mm versus 90% and 46% for Smaller_PS_ETH >6 mm, and 52% and 67% for MS_ETH >9 mm versus 48% and 87% for Smaller_PS_ETH >9 mm. At ETH >15 mm, no differences were seen between the measurements. CONCLUSIONS: Diagnostic utility of ETH measurements in detecting clinically significant CMI can be improved by parasagittal measurements of the cerebellar tonsillar herniation.

Are Two-Dimensional Morphometric Measures Reflective of Disease Severity in Adult Chiari I Malformation?

BACKGROUND: Two-dimensional (2D) morphometric measures have been previously used to diagnose Chiari I malformation (CMI) and distinguish patients from healthy subjects. There is, however, a paucity of literature regarding whether morphometric differences exist across the range of CMI disease severity. We evaluated whether 2D morphometrics demonstrate significant changes across standardized grades of CMI severity in adults. METHODS: This retrospective observational study comprised 76 patients with symptomatic CMI with or without syringomyelia. Patients matched for age, sex, and body mass index were selected from each of the 3 severity grades of the Chiari Severity Index (CSI). The study used 17 2D craniocervical and posterior fossa morphometric variables that were previously found to differentiate patients with CMI from healthy subjects. The measurements were performed on midsagittal T1-weighted magnetic resonance imaging sequences using 2 custom in-house software tools, MorphPro and CerePro, and compared across CSI grades. RESULTS: Analysis of variance showed that none of the 17 morphometric variables were significantly different across the 3 CSI grades (P > 0.003). Bayes factor 10 scores ranged from 0.11 to 0.82. Of variables, 9 had Bayes factor 10 scores between 0.10 and 0.30, while 8 had scores between 0.30 and 1. None of the Bayes factor 10 scores provided support for the alternative hypothesis that the morphometric measures differed across the CSI grades. CONCLUSIONS: Our study indicated that 2D morphometrics are not reflective of CMI disease severity as indicated by the CSI grading system. The findings of our study support the need for further investigation into whether non-2D morphometric variables can be used as markers of disease severity.

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.

Transition to Turbulence Downstream of a Stenosis for Whole Blood and a Newtonian Analog Under Steady Flow Conditions.

Blood, a multiphase fluid comprised of plasma, blood cells, and platelets, is known to exhibit a shear-thinning behavior at low shear rates and near-Newtonian behavior at higher shear rates. However, less is known about the impact of its multiphase nature on the transition to turbulence. In this study, we experimentally determined the critical Reynolds number at which the flow began to transition to turbulence downstream of eccentric stenosis for whole porcine blood and a Newtonian blood analog (water-glycerin mixture). Velocity profiles for both fluids were measured under steady-state flow conditions using an ultrasound Doppler probe placed 12 diameters downstream of eccentric stenosis. Velocity was recorded at 21 locations along the diameter at 11 different flow rates. Normalized turbulent kinetic energy was used to determine the critical Reynolds number for each fluid. Blood rheology was measured before and after each experiment. Tests were conducted on five samples of each fluid inside a temperature-controlled in vitro flow system. The viscosity at a shear rate of 1000 s-1 was used to define the Reynolds number for each fluid. The mean critical Reynolds numbers for blood and water-glycerin were 470 ± 27.5 and 395 ± 10, respectively, indicating a ∼19% delay in transition to turbulence for whole blood compared to the Newtonian fluid. This finding is consistent with a previous report for steady flow in a straight pipe, suggesting some aspect of blood rheology may serve to suppress, or at least delay, the onset of turbulence in vivo.

Cerebellar and Brainstem Displacement Measured with DENSE MRI in Chiari Malformation Following Posterior Fossa Decompression Surgery.

Background Posterior fossa decompression (PFD) surgery is a treatment for Chiari malformation type I (CMI). The goals of surgery are to reduce cerebellar tonsillar crowding and restore posterior cerebral spinal fluid flow, but regional tissue biomechanics may also change. MRI-based displacement encoding with stimulated echoes (DENSE) can be used to assess neural tissue displacement. Purpose To assess neural tissue displacement by using DENSE MRI in participants with CMI before and after PFD surgery and examine associations between tissue displacement and symptoms. Materials and Methods In a prospective, HIPAA-compliant study of patients with CMI, midsagittal DENSE MRI was performed before and after PFD surgery between January 2017 and June 2020. Peak tissue displacement over the cardiac cycle was quantified in the cerebellum and brainstem, averaged over each structure, and compared before and after surgery. Paired t tests and nonparametric Wilcoxon signed-rank tests were used to identify surgical changes in displacement, and Spearman correlations were determined between tissue displacement and presurgery symptoms. Results Twenty-three participants were included (mean age ± standard deviation, 37 years ± 10; 19 women). Spatially averaged (mean) peak tissue displacement demonstrated reductions of 46% (79/171 µm) within the cerebellum and 22% (46/210 µm) within the brainstem after surgery (P < .001). Maximum peak displacement, calculated within a circular 30-mm2 area, decreased by 64% (274/427 µm) in the cerebellum and 33% (100/300 µm) in the brainstem (P < .001). No significant associations were identified between tissue displacement and CMI symptoms (r < .74 and P > .012 for all; Bonferroni-corrected P = .0002). Conclusion Neural tissue displacement was reduced after posterior fossa decompression surgery, indicating that surgical intervention changes brain tissue biomechanics. For participants with Chiari malformation type I, no relationship was identified between presurgery tissue displacement and presurgical symptoms. © RSNA, 2021 Online supplemental material is available for this article.