No abstract

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.

C hiari malformation type I (CMI) is defined by cerebellar tonsillar descent of at least 5 mm below the foramen magnum (1-3). Patients with CMI present with a variety of neurologic symptoms, including suboccipital headaches and/or neck pain, cognitive and/or neurologic deficits, and a range of nonspecific symptoms. Patients with CMI may undergo posterior fossa decompression (PFD) surgery to reduce symptoms, restore posterior fossa cerebrospinal fluid flow, reduce tonsillar crowding, and relieve brainstem and/or cerebellar tissue compression (4,5). Although preoperative assessment includes the evaluation of tonsillar descent and other morphologic features, there are conflicting results within the literature as to whether tonsillar descent is related to CMI symptoms (1,6-8).Patients with CMI demonstrate altered brain tissue biomechanics, characterized by increased neural tissue motion within the craniocervical region (6,(9)(10)(11)(12)(13)(14)(15)(16). Brain tissue displacement is thought to be primarily caused by changes in pressure during each heartbeat (17). Past studies have shown cerebrospinal fluid dynamics at the craniocervical region to be abnormal in participants with CMI when compared with healthy controls (18). Several studies have examined neural tissue dynamics by using phase-contrast MRI velocity measurements (6,9,10,12,13,15,16). Tissue displacement has also been quantified by integrating the phase-contrast MRI-measured velocity waveforms with respect to time over the cardiac cycle (6,9,10,13). However, phase-contrast MRI indirect measurement of 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 6 standard deviation, 37 years 6 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-mm...

The goal of this study was to determine the accuracy of displacementencoding with stimulated echoes (DENSE) MRI in a tissue motion phantom with displacements representative of those observed in human brain tissue. Methods: The phantom was comprised of a plastic shaft rotated at a constant speed. The rotational motion was converted to a vertical displacement through a camshaft. The phantom generated repeatable cyclical displacement waveforms with a peak displacement ranging from 92 µm to 1.04 mm at 1-Hz frequency. The surface displacement of the tissue was obtained using a laser Doppler vibrometer (LDV) before and after the DENSE MRI scans to check for repeatability. The accuracy of DENSE MRI displacement was assessed by comparing the laser Doppler vibrometer and DENSE MRI waveforms. Results: Laser Doppler vibrometer measurements of the tissue motion demonstrated excellent cycle-to-cycle repeatability with a maximum root mean square error of 9 µm between the ensemble-averaged displacement waveform and the individual waveforms over 180 cycles. The maximum difference between DENSE MRI and the laser Doppler vibrometer waveforms ranged from 15 to 50 µm. Additionally, the peak-to-peak difference between the 2 waveforms ranged from 1 to 18 µm. Conclusion: Using a tissue phantom undergoing cyclical motion, we demonstrated the percent accuracy of DENSE MRI to measure displacement similar to that observed for in vivo cardiac-induced brain tissue.

No abstract

No abstract

No abstract

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.