Cognitive-Affective Improvement on Cerebellar Neuropsychiatric Rating Scale Scores in Adults and Children After Decompression of Chiari Malformation Type I.

Background And Objectives: This study evaluates the Cerebellar Neuropsychiatric Rating Scale (CNRS) in the preoperative and postoperative assessment of Chiari Malformation Type I (CM1).

Methods: Adults and children with symptomatic CM1 undergoing surgical decompression completed the CNRS preoperatively (N = 87) and 8-24 months postoperatively (N = 56). Preoperative and postoperative total scores, as well as subdomains of attentional control, emotional control, autism spectrum, psychosis spectrum, and social skills, were compared using a paired t-test.

Correlation of anterior CSF space in the cervical spine with Chicago Chiari Outcome Scale score in adult females.

Objective: Craniocervical junction morphology has been associated with Chiari malformation type I (CMI) symptom severity; however, little is known about its deterministic effect on surgical outcomes in patients across age and sex differences. The goal of the present study was to assess the effects of age and sex on surgical outcomes in CMI.

Methods: In the present study, the authors examined MRI-based morphometric data from 115 individuals diagnosed with CMI (54 adults including 39 women and 15 men, and 61 children including 24 girls and 37 boys) and correlated them with Chicago Chiari Outcome Scale (CCOS) scores obtained 1 year after posterior fossa decompression.

High levels of patient self advocacy may confound clinical research in understudied patient populations.

To examine the levels of patient self-advocacy in a sample of participants with Chiari Malformation (CM) and to explore how they relate to clinical outcomes. As part of a larger clinical trial addressing chronic pain in patients with CM, 111 participants completed the Patient Self Advocacy Scale (PSAS), the Depression, Anxiety, and Stress Scale (DASS-21), and the Brief Pain Inventory (BPI). PSAS scores indicated a moderately high level of patient self-advocacy (Mean = 3.

Clinical criteria for filum terminale resection in occult tethered cord syndrome.

Objective: Tethered cord syndrome (TCS) comprises three symptom categories: back/leg pain, bowel/bladder, and neurological complaints. MRI typically reveals a low-lying conus medullaris, filum terminale (FT) pathology, or lumbosacral abnormalities. FT resection is established in TCS but not in radiologically occult TCS (OTCS).

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.

Cognition in Chiari Malformation Type I: an Update of a Systematic Review.

Chiari malformation has been classified as a group of posterior cranial fossa disorders characterized by hindbrain herniation. Chiari malformation type I (CM-I) is the most common subtype, ranging from asymptomatic patients to those with severe disorders. Research about clinical manifestations or medical treatments is still growing, but cognitive functioning has been less explored.

Revisiting the factor structure of the Short-Form McGill Pain Questionnaire-2 (SF-MPQ-2): Evidence for a bifactor model in individuals with Chiari malformation.

The Short-Form McGill Pain Questionnaire-2 (SF-MPQ-2; Dworkin et al., 2009) is intended to measure the multidimensional qualities of pain (i.e.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Functional connectivity abnormalities in Type I Chiari: associations with cognition and pain.

There is initial evidence of microstructural abnormalities in the fibre-tract pathways of the cerebellum and cerebrum of individuals diagnosed with Type I Chiari malformation. However, it is unclear whether abnormal white matter architecture and macro-level morphological deviations that have been observed in Chiari translate to differences in functional connectivity. Furthermore, common symptoms of Chiari include pain and cognitive deficits, but the relationship between these symptoms and functional connectivity has not been explored in this population.

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

Purpose: 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.

Methods: 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.

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.

Impact of Surgical Status, Loneliness, and Disability on Interleukin 6, C-Reactive Protein, Cortisol, and Estrogen in Females with Symptomatic Type I Chiari Malformation.

Chiari malformation type I (CMI) provides an opportunity for examining possible moderators of allostatic load. CMI patients who had (n = 43) and had not (n = 19) undergone decompression surgery completed questionnaires regarding pain, disability, and loneliness, and provided serum samples for IL-6, CRP, estrogen, and free estradiol assays, and saliva samples to assess diurnal cortisol curves. ANOVAs examining surgical status (decompressed versus non-decompressed), loneliness (high vs.

Association Between Resistance to Cerebrospinal Fluid Flow Near the Foramen Magnum and Cough-Associated Headache in Adult Chiari Malformation Type I.

Cough-associated headaches (CAHs) are thought to be distinctive for Chiari malformation type I (CMI) patients and have been shown to be related to the motion of cerebrospinal fluid (CSF) near the foramen magnum (FM). We used computational fluid dynamics (CFD) to compute patient-specific resistance to CSF motion in the spinal canal for CMI patients to determine its accuracy in predicting CAH. Fifty-one symptomatic CMI patients with cerebellar tonsillar position (CTP) ≥ 5 mm were included in this study.

Regional Brain Tissue Displacement and Strain is Elevated in Subjects with Chiari Malformation Type I Compared to Healthy Controls: A Study Using DENSE MRI.

While the degree of cerebellar tonsillar descent is considered the primary radiologic marker of Chiari malformation type I (CMI), biomechanical forces acting on the brain tissue in CMI subjects are less studied and poorly understood. In this study, regional brain tissue displacement and principal strains in 43 CMI subjects and 25 controls were quantified using a magnetic resonance imaging (MRI) methodology known as displacement encoding with stimulated echoes (DENSE). Measurements from MRI were obtained for seven different brain regions-the brainstem, cerebellum, cingulate gyrus, corpus callosum, frontal lobe, occipital lobe, and parietal lobe.

An Electrophysiological Study of Aging and Perceptual Letter-Matching.

: Previous studies on perceptual letter-matching have found that younger and older adults showed "fast-same" effects for response time and "false-different" effects for errors but the effects were more pronounced for older adults. According to the , internal noise in visual processing distorts "same" trials into appearing different whereas distortion for "different" trials does not affect performance. Older adults have a "noisier" representation of items within perceptual processing which can impact perceptual matching.

Clivus length distinguishes between asymptomatic healthy controls and symptomatic adult women with Chiari malformation type I.

Purpose: While the presence of cerebellar tonsillar descent in radiological images has been used as evidence of Chiari malformation type I (CMI), tonsillar ectopia alone is insufficient to identify individuals with symptomatic CMI. This study sought to identify differences in brain morphology between symptomatic CMI and healthy controls in adult females.

Methods: Two hundred and ten adult females with symptomatic CMI and 90 age- and body mass index-matched asymptomatic female controls were compared using seven brain morphometric measures visible on magnetic resonance images.