scholarly journals Imaging and health metrics in Incidental Cerebellar Tonsillar Ectopia: Findings from the Adolescent Brain Cognitive Development Study (ABCD)

2021 ◽  
Author(s):  
Blaise Simplice Talla Nwotchouang ◽  
Alaaddin Ibrahimy ◽  
Dorothy M. Loth ◽  
Edward Labuda ◽  
Nicholas Labuda ◽  
...  
Keyword(s):  
Cognitive Development ◽  
Race And Ethnicity ◽  
Health Impact ◽  
Brain Morphology ◽  
Common Finding ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Tonsillar Ectopia ◽  
Radiographic Criterion ◽  
And Control

Abstract 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. 106 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. Results. 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. Conclusion. 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.

scholarly journals Breaking Barriers for Cerebrospinal Fluid Flow in Chiari Malformation Type I: “What and How Much Is Enough?” A Retrospective Analysis of 74 Cases

2018 ◽  
Vol 07 (03) ◽  
pp. 196-208
Author(s):  
Manish Jaiswal ◽  
Amit Patil ◽  
Radhey Mittal
Keyword(s):  
Cerebrospinal Fluid ◽  
Natural History ◽  
Retrospective Analysis ◽  
Chiari Malformation ◽  
Foramen Magnum ◽  
Common Finding ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Compression Syndrome ◽  
History Of

Abstract Introduction: Chiari malformation type I is a collection of hindbrain abnormalities, for which natural history of the disease process is not clear. The challenge is to identify which patients will benefit most from posterior fossa decompression. Objectives: To identify important surgical implications that most likely benefit patients with Chiari malformation type I by analyzing and reviewing various operative interventions in these patients with appropriate symptoms and then following their course. Subjects & Methods: Retrospective analysis of 74 operated Chiari malformation type I with syrinx adult patients was done. Results: No definite pattern of progression in natural history of disease was noted. Most of the patients who were symptomatically stable for months to years presented with recent rapid progression. The most common symptom was suboccipital pain. The most common finding was lower extremity weakness. On clinical presentation basis, patients were divided into three categories: foramen magnum compression syndrome, central cord syndrome, and cerebellar syndrome. Most patients in our study fall in first category. Foramen magnum decompression with atlas posterior arch removal and sometimes partial C2 laminectomy depending on extent of tonsillar descent as well as augmentation duraplasty was done in most patients. Improvement was seen in foramen magnum compression syndrome group more significantly. Conclusions: Individualized surgical techniques for breaking the barriers of cerebrospinal fluid (CSF) flow in Chiari malformation type I with syrinx to restore normal CSF dynamics across craniocervical junction provide the pragmatic solution. The trend is toward balance between optimum wide decompression as compared with long craniocaudal decompression and preserving normal integrity.

scholarly journals Normalization of hindbrain morphology after decompression of Chiari malformation Type I

2012 ◽  
Vol 117 (5) ◽  
pp. 942-946 ◽  
Author(s):  
John D. Heiss ◽  
Giancarlo Suffredini ◽  
Kamran D. Bakhtian ◽  
Malisa Sarntinoranont ◽  
Edward H. Oldfield
Keyword(s):  
Chiari Malformation ◽  
Foramen Magnum ◽  
Control Group ◽  
Decompression Surgery ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Prospective Longitudinal Study ◽  
Tonsillar Ectopia ◽  
Prospective Longitudinal ◽  
Cerebellar Tonsils

Object Chiari malformation Type I (CM-I) is characterized by hindbrain deformity. We investigated the effects of craniocervical decompression surgery on the anatomical features of hindbrain deformity with a prospective MRI study of patients with CM-I. Methods A prospective longitudinal study was conducted in 48 patients with CM-I (39 with syringomyelia) treated with craniocervical decompression. Clinical examinations and cervical MRI were performed before surgery and 1 week, 3–6 months, and annually after surgery. Hindbrain deformity was defined by tonsillar ectopia, pointed cerebellar tonsils, and/or cervicomedullary protuberance. The length of the clivus, basiocciput (sphenooccipital synchondrosis to basion), supraocciput (internal occipital protuberance to opisthion), and anteroposterior (AP) width of CSF pathways at the foramen magnum were measured and compared with those from 18 healthy volunteers (control group). Results Before surgery, the patients' posterior fossa bones were short and their CSF pathways were narrow. All patients had tonsillar ectopia (mean [± SD] 12.3 ± 5.1 mm; normal 0.3 ± 1.0). The majority of patients had pointed tonsils and more than two-thirds exhibited a cervicomedullary protuberance. Clivus and basiocciput lengths were significantly shorter than the values obtained in the control group. However, the supraocciput length did not differ significantly from control measurements. The mean bulbopontine sulcus distance superior to the basion was 9.5 ± 2.6 mm (vs 13.6 ± 2.8 mm in controls; p < 0.0001). The AP widths of the CSF pathways at the level of the foramen magnum were significantly narrowed. After surgery, CSF pathways significantly expanded both ventrally and dorsally. By 3–6 months after surgery, pointed tonsils became round, cervicomedullary protuberance disappeared, and tonsillar ectopia diminished by 51% (to 6.0 ± 3.3 mm; p < 0.0001). Conclusions The cerebellar tonsils and brainstem assumed a normal appearance within 6 months after craniocervical decompression. These findings support the concept that the CM-I is not a congenital malformation of the neural elements but rather an acquired malformation that arises from pulsatile impaction of the cerebellar tonsils into the foramen magnum. Clinical trial registration no.: NCT00001327.

Effectiveness of the Chiari Health Index for Pediatrics instrument in measuring postoperative health-related quality of life in pediatric patients with Chiari malformation type I

2020 ◽  
pp. 1-6
Author(s):  
Georgina E. Sellyn ◽  
Alan R. Tang ◽  
Shilin Zhao ◽  
Madeleine Sherburn ◽  
Rachel Pellegrino ◽  
...  
Keyword(s):  
Surgical Intervention ◽  
Type I ◽  
Health Related Quality ◽  
Health Index ◽  
Chiari Malformation Type I ◽  
Related Quality ◽  
Health Related ◽  
Over Time

OBJECTIVEThe authors’ previously published work validated the Chiari Health Index for Pediatrics (CHIP), a new instrument for measuring health-related quality of life (HRQOL) for pediatric Chiari malformation type I (CM-I) patients. In this study, the authors further evaluated the CHIP to assess HRQOL changes over time and correlate changes in HRQOL to changes in symptomatology and radiological factors in CM-I patients who undergo surgical intervention. Strong HRQOL evaluation instruments are currently lacking for pediatric CM-I patients, creating the need for a standardized HRQOL instrument for this patient population. This study serves as the first analysis of the CHIP instrument’s effectiveness in measuring short-term HRQOL changes in pediatric CM-I patients and can be a useful tool in future CM-I HRQOL studies.METHODSThe authors evaluated prospectively collected CHIP scores and clinical factors of surgical intervention in patients younger than 18 years. To be included, patients completed a baseline CHIP captured during the preoperative visit, and at least 1 follow-up CHIP administered postoperatively. CHIP has 2 domains (physical and psychosocial) comprising 4 components, the 3 physical components of pain frequency, pain severity, and nonpain symptoms, and a single psychosocial component. Each CHIP category is scored on a scale, with 0 indicating absent and 1 indicating present, with higher scores indicating better HRQOL. Wilcoxon paired tests, Spearman correlations, and linear regression models were used to evaluate and correlate HRQOL, symptomatology, and radiographic factors.RESULTSSixty-three patients made up the analysis cohort (92% Caucasian, 52% female, mean age 11.8 years, average follow-up time 15.4 months). Dural augmentation was performed in 92% of patients. Of the 63 patients, 48 reported preoperative symptoms and 42 had a preoperative syrinx. From baseline, overall CHIP scores significantly improved over time (from 0.71 to 0.78, p < 0.001). Significant improvement in CHIP scores was seen in patients presenting at baseline with neck/back pain (p = 0.015) and headaches (p < 0.001) and in patients with extremity numbness trending at p = 0.064. Patients with syringomyelia were found to have improvement in CHIP scores over time (0.75 to 0.82, p < 0.001), as well as significant improvement in all 4 components. Additionally, improved CHIP scores were found to be significantly associated with age in patients with cervical (p = 0.009) or thoracic (p = 0.011) syrinxes.CONCLUSIONSThe study data show that the CHIP is an effective instrument for measuring HRQOL over time. Additionally, the CHIP was found to be significantly correlated to changes in symptomatology, a finding indicating that this instrument is a clinically valuable tool for the management of CM-I.

Prognostic significance of C1–C2 facet malalignment after surgical decompression in adult Chiari malformation type I: a pilot study based on the Chicago Chiari Outcome Scale

2020 ◽  
pp. 1-7
Author(s):  
Michael Lumintang Loe ◽  
Tito Vivas-Buitrago ◽  
Ricardo A. Domingo ◽  
Johan Heemskerk ◽  
Shashwat Tripathi ◽  
...  
Keyword(s):  
Pilot Study ◽  
Chiari Malformation ◽  
Prognostic Significance ◽  
Foramen Magnum ◽  
Bivariate Analysis ◽  
Type I ◽  
Foramen Magnum Decompression ◽  
Swallowing Function ◽  
Chiari Malformation Type I

OBJECTIVEThe authors assessed the prognostic significance of various clinical and radiographic characteristics, including C1–C2 facet malalignment, in terms of surgical outcomes after foramen magnum decompression of adult Chiari malformation type I.METHODSThe electronic medical records of 273 symptomatic patients with Chiari malformation type I who were treated with foramen magnum decompression, C1 laminectomy, and duraplasty at Mayo Clinic were retrospectively reviewed. Preoperative and postoperative Neurological Scoring System scores were compared using the Friedman test. Bivariate analysis was conducted to identify the preoperative variables that correlated with the patient Chicago Chiari Outcome Scale (CCOS) scores. Multiple linear regression analysis was subsequently performed using the variables with p < 0.05 on the bivariate analysis to check for independent associations with the outcome measures. Statistical software SPSS version 25.0 was used for the data analysis. Significance was defined as p < 0.05 for all analyses.RESULTSFifty-two adult patients with preoperative clinical and radiological data and a minimum follow-up of 12 months were included. Motor deficits, syrinx, and C1–C2 facet malalignment were found to have significant negative associations with the CCOS score at the 1- to 3-month follow-up (p < 0.05), while at the 9- to 12-month follow-up only swallowing function and C1–C2 facet malalignment were significantly associated with the CCOS score (p < 0.05). Multivariate analysis showed that syrinx presence and C1–C2 facet malalignment were independently associated with the CCOS score at the 1- to 3-month follow-up. Swallowing function and C1–C2 facet malalignment were found to be independently associated with the CCOS score at the 9- to 12-month follow-up.CONCLUSIONSThe observed results in this pilot study suggest a significant negative correlation between C1–C2 facet malalignment and clinical outcomes evaluated by the CCOS score at 1–3 months and 9–12 months postoperatively. Prospective studies are needed to further validate the prognostic value of C1–C2 facet malalignment and the potential role of atlantoaxial fixation as part of the treatment.

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

2021 ◽  
Author(s):  
Blaise Simplice Talla Nwotchouang ◽  
Maggie S. Eppelheimer ◽  
Soroush Heidari Pahlavian ◽  
Jack W. Barrow ◽  
Daniel L. Barrow ◽  
...  
Keyword(s):  
Brain Tissue ◽  
Chiari Malformation ◽  
Type I ◽  
Healthy Controls ◽  
Chiari Malformation Type I ◽  
Regional Brain

scholarly journals A genetically stable Zika virus vaccine candidate protects mice against virus infection and vertical transmission

npj Vaccines ◽  
2021 ◽  
Vol 6 (1) ◽  
Author(s):  
Awadalkareem Adam ◽  
Camila R. Fontes-Garfias ◽  
Vanessa V. Sarathy ◽  
Yang Liu ◽  
Huanle Luo ◽  
...  
Keyword(s):  
T Cell ◽  
Vertical Transmission ◽  
Zika Virus ◽  
Type I ◽  
Post Vaccination ◽  
Vaccine Trials ◽  
Specific Memory ◽  
Cell Immunity ◽  
Cell Antibody ◽  
And Control

AbstractAlthough live attenuated vaccines (LAVs) have been effective in the control of flavivirus infections, to date they have been excluded from Zika virus (ZIKV) vaccine trials due to safety concerns. We have previously reported two ZIKV mutants, each of which has a single substitution in either envelope (E) glycosylation or nonstructural (NS) 4B P36 and displays a modest reduction in mouse neurovirulence and neuroinvasiveness, respectively. Here, we generated a ZIKV mutant, ZE4B-36, which combines mutations in both E glycosylation and NS4B P36. The ZE4B-36 mutant is stable and attenuated in viral replication. Next-generation sequence analysis showed that the attenuating mutations in the E and NS4B proteins are retained during serial cell culture passages. The mutant exhibits a significant reduction in neuroinvasiveness and neurovirulence and low infectivity in mosquitoes. It induces robust ZIKV-specific memory B cell, antibody, and T cell-mediated immune responses in type I interferon receptor (IFNR) deficient mice. ZIKV-specific T cell immunity remains strong months post-vaccination in wild-type C57BL/6 (B6) mice. Vaccination with ZE4B-36 protects mice from ZIKV-induced diseases and vertical transmission. Our results suggest that combination mutations in E glycosylation and NS4B P36 contribute to a candidate LAV with significantly increased safety but retain strong immunogenicity for prevention and control of ZIKV infection.

Updated Evidence Regarding Chiari Malformation Type I and Management Options

2020 ◽  
Vol 42 (5) ◽  
pp. 1-5
Author(s):  
Jeffrey Hatef ◽  
Eric A. Sribnick ◽  
Jeffrey R. Leonard
Keyword(s):  
Chiari Malformation ◽  
Type I ◽  
Management Options ◽  
Chiari Malformation Type I
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