Dix-Hallpike Maneuver Triggered Pulsatile Occipital Headache with Geotropic Nystagmus in a Case of Type I Chiari Malformation

Objective Broader access to magnetic resonance imaging (MRI) has increased the diagnosis of tonsillar ectopia, with most of these patients being asymptomatic. The early diagnosis and treatment of type I Chiari malformation (CM I) patients has impact on the prognosis. This study supplements information about the neurologic exam of symptomatic patients with CM I. Methods The sample was composed of 32 symptomatic patients with CM I diagnosed by a combination of tonsil herniation of more than 5 mm below the magnum foramen (observed in the sagittal T2 MRI) and at least one of the following alterations: intractable occipital headache, ataxia, upper or lower motor neuron impairment, sensitivity deficits (superficial and deep) or lower cranial nerves disorders. Results Occipital headache was the most frequent symptom (53.12%). During the physical exam, the most common dysfunctions were those from the pyramidal system (96.87%), followed by posterior cord syndrome (87.5%). Discussion In this study, patients became symptomatic around the fifth decade of life, which is compatible with previous descriptions. Patients with more than 2 years of evolution have worse responses to treatment. Occipital headache, symptoms in the upper limbs, gait and proprioceptive disorders are common findings in patients with CM I. Conclusion Deep tendinous reflexes and proprioception disorders were the main neurologic features found in symptomatic CM I patients.

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Intraoperative ultrasonography used to determine the extent of surgery necessary during posterior fossa decompression in children with Chiari malformation Type I

Yearbook of Diagnostic Radiology ◽

10.1016/s0098-1672(08)70086-9 ◽

2007 ◽

Vol 2007 ◽

pp. 116-117

Author(s):

A.E. Oestreich

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Intraoperative Ultrasonography ◽

Posterior Fossa Decompression ◽

Type I ◽

Chiari Malformation Type I ◽

Extent Of Surgery

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Management of Chiari Malformation Type I with Posterior Fossa Decompression without Dural Opening

Journal of Neurological Surgery Part A Central European Neurosurgery ◽

10.1055/s-0035-1566365 ◽

2015 ◽

Vol 76 (S 02) ◽

Author(s):

Walid Elhalaby

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Posterior Fossa Decompression ◽

Type I ◽

Chiari Malformation Type I ◽

Dural Opening

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Radiological and clinical associations with scoliosis outcomes after posterior fossa decompression in patients with Chiari malformation and syrinx from the Park-Reeves Syringomyelia Research Consortium

Journal of Neurosurgery Pediatrics ◽

10.3171/2020.1.peds18755 ◽

2020 ◽

Vol 26 (1) ◽

pp. 53-59 ◽

Cited By ~ 2

Author(s):

Jennifer M. Strahle ◽

Rukayat Taiwo ◽

Christine Averill ◽

James Torner ◽

Jordan I. Gewirtz ◽

...

Keyword(s):

Posterior Fossa ◽

Chiari Malformation ◽

Craniocervical Junction ◽

Curve Progression ◽

Posterior Fossa Decompression ◽

Type I ◽

Younger Age ◽

Curve Magnitude ◽

The Mean

OBJECTIVEIn patients with Chiari malformation type I (CM-I) and a syrinx who also have scoliosis, clinical and radiological predictors of curve regression after posterior fossa decompression are not well known. Prior reports indicate that age younger than 10 years and a curve magnitude < 35° are favorable predictors of curve regression following surgery. The aim of this study was to determine baseline radiological factors, including craniocervical junction alignment, that might predict curve stability or improvement after posterior fossa decompression.METHODSA large multicenter retrospective and prospective registry of pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and a syrinx (≥ 3 mm in width) was reviewed for clinical and radiological characteristics of CM-I, syrinx, and scoliosis (coronal curve ≥ 10°) in patients who underwent posterior fossa decompression and who also had follow-up imaging.RESULTSOf 825 patients with CM-I and a syrinx, 251 (30.4%) were noted to have scoliosis present at the time of diagnosis. Forty-one (16.3%) of these patients underwent posterior fossa decompression and had follow-up imaging to assess for scoliosis. Twenty-three patients (56%) were female, the mean age at time of CM-I decompression was 10.0 years, and the mean follow-up duration was 1.3 years. Nine patients (22%) had stable curves, 16 (39%) showed improvement (> 5°), and 16 (39%) displayed curve progression (> 5°) during the follow-up period. Younger age at the time of decompression was associated with improvement in curve magnitude; for those with curves of ≤ 35°, 17% of patients younger than 10 years of age had curve progression compared with 64% of those 10 years of age or older (p = 0.008). There was no difference by age for those with curves > 35°. Tonsil position, baseline syrinx dimensions, and change in syrinx size were not associated with the change in curve magnitude. There was no difference in progression after surgery in patients who were also treated with a brace compared to those who were not treated with a brace for scoliosis.CONCLUSIONSIn this cohort of patients with CM-I, a syrinx, and scoliosis, younger age at the time of decompression was associated with improvement in curve magnitude following surgery, especially in patients younger than 10 years of age with curves of ≤ 35°. Baseline tonsil position, syrinx dimensions, frontooccipital horn ratio, and craniocervical junction morphology were not associated with changes in curve magnitude after surgery.

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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

Journal of Neurosurgery Spine ◽

10.3171/2020.6.spine20544 ◽

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.

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

Annals of Biomedical Engineering ◽

10.1007/s10439-020-02695-7 ◽

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

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In vitro evaluation of cerebrospinal fluid velocity measurement in type I Chiari malformation: repeatability, reproducibility, and agreement using 2D phase contrast and 4D flow MRI

Fluids and Barriers of the CNS ◽

10.1186/s12987-021-00246-3 ◽

2021 ◽

Vol 18 (1) ◽

Author(s):

Gwendolyn Williams ◽

Suraj Thyagaraj ◽

Audrey Fu ◽

John Oshinski ◽

Daniel Giese ◽

...

Keyword(s):

Chiari Malformation ◽

Phase Contrast ◽

Patient Specific ◽

Type I ◽

4D Flow ◽

Csf Dynamics ◽

Flow Measurements ◽

Repeatability And Reproducibility ◽

Flow Techniques

Abstract Background Phase contrast magnetic resonance imaging, PC MRI, is a valuable tool allowing for non-invasive quantification of CSF dynamics, but has lacked adoption in clinical practice for Chiari malformation diagnostics. To improve these diagnostic practices, a better understanding of PC MRI based measurement agreement, repeatability, and reproducibility of CSF dynamics is needed. Methods An anatomically realistic in vitro subject specific model of a Chiari malformation patient was scanned three times at five different scanning centers using 2D PC MRI and 4D Flow techniques to quantify intra-scanner repeatability, inter-scanner reproducibility, and agreement between imaging modalities. Peak systolic CSF velocities were measured at nine axial planes using 2D PC MRI, which were then compared to 4D Flow peak systolic velocity measurements extracted at those exact axial positions along the model. Results Comparison of measurement results showed good overall agreement of CSF velocity detection between 2D PC MRI and 4D Flow (p = 0.86), fair intra-scanner repeatability (confidence intervals ± 1.5 cm/s), and poor inter-scanner reproducibility. On average, 4D Flow measurements had a larger variability than 2D PC MRI measurements (standard deviations 1.83 and 1.04 cm/s, respectively). Conclusion Agreement, repeatability, and reproducibility of 2D PC MRI and 4D Flow detection of peak CSF velocities was quantified using a patient-specific in vitro model of Chiari malformation. In combination, the greatest factor leading to measurement inconsistency was determined to be a lack of reproducibility between different MRI centers. Overall, these findings may help lead to better understanding for application of 2D PC MRI and 4D Flow techniques as diagnostic tools for CSF dynamics quantification in Chiari malformation and related diseases.

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