Cough-associated headache in patients with Chiari I malformation: CSF Flow analysis by means of cine phase-contrast MR imaging.
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ABSTRACT:
SUBMITTER: Bhadelia RA
PROVIDER: S-EPMC7965870 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Background and purposeInvasive pressure studies have suggested that CSF flow across the foramen magnum may transiently decrease after coughing in patients with symptomatic Chiari I malformation. The purpose of this exploratory study was to demonstrate this phenomenon noninvasively by assessing CSF flow response to coughing in symptomatic patients with Chiari I malformation by using MR pencil beam imaging and to compare the response with that in healthy participants.Materials and methodsEight symptomatic patients with Chiari I malformation and 6 healthy participants were studied by using MR pencil beam imaging with a temporal resolution of ∼50 ms. Patients and healthy participants were scanned for 90 seconds (without cardiac gating) to continuously record cardiac cycle-related CSF flow waveforms in real-time during resting, coughing, and postcoughing periods. CSF flow waveform amplitude, CSF stroke volume, and CSF flow rate (CSF Flow Rate = CSF Stroke Volume × Heart Rate) in the resting and immediate postcoughing periods were determined and compared between patients and healthy participants.ResultsThere was no significant difference in CSF flow waveform amplitude, CSF stroke volume, and the CSF flow rate between patients with Chiari I malformation and healthy participants during rest. However, immediately after coughing, a significant decrease in CSF flow waveform amplitude (P < .001), CSF stroke volume (P = .001), and CSF flow rate (P = .001) was observed in patients with Chiari I malformation but not in the healthy participants.ConclusionsReal-time MR imaging noninvasively showed a transient decrease in CSF flow across the foramen magnum after coughing in symptomatic patients with Chiari I malformation, a phenomenon not seen in healthy participants. Our results provide preliminary evidence that the physiology-based imaging method used here has the potential to be an objective clinical test to differentiate symptomatic from asymptomatic patients with Chiari I malformation.
| S-EPMC7960326 | biostudies-literature
Project description:BackgroundChiari I malformation typically presents with cough headache. However, migraine-like or tension-type-like headaches may also occur. There are limited publications on Chiari I malformation-associated headache semiologies and the effect of foramen magnum decompression on different headache types.MethodsA retrospective analysis complemented by structured phone interviews was performed on 65 patients with Chiari I malformation, treated at our hospital between 2010 and 2021. Headache semiology (according to ICHD-3), frequency, intensity, and radiological characteristics were evaluated pre- and postoperatively.ResultsWe included 65 patients. 38 patients were female and 27 male. Mean age was 43.9 ± 15.7 years. Headache was predominant in 41 patients (63.0%). Twenty-one patients had cough headache and 20 had atypical headache (12 migrainous, eight tension-type headache-like). Thirty-five patients with headache underwent surgery. Frequency, intensity, and analgesic use was significantly reduced in cough headache (p < 0.001). Atypical headaches improved less (p = 0.004 to 0.176). Exploratory analysis suggested that larger preoperative tonsillar descent correlated with larger postoperative headache intensity relief (p = 0.025).ConclusionDecompression was effective in Chiari I malformation-related cough headache. Atypical headache responded less well, and the causal relation with Chiari I malformation remains uncertain. For atypical headache, decompression should only be considered after failed appropriate preventive therapy and within an interdisciplinary approach involving a neurologist.
| S-EPMC9315190 | biostudies-literature
Project description:Chiari malformation is a congenital deformity leading to herniation of cerebellar tonsils. Headache is a typical symptom of this condition, but patients with Chiari malformation often present with double vision and vertigo. Examination of eye movements in such patients often reveals nystagmus and strabismus. Eye movement deficits in the context of typical symptomatic presentation are critical clinical markers for the diagnosis of Chiari malformation. We will review eye movement deficits that seen in patients with type 1 Chiari malformation. We will then discuss the underlying pathophysiology and therapeutic options for such deficits.
| S-EPMC4733655 | biostudies-literature
Project description:Background & Aims:Hepatobiliary phase (HBP) images can discriminate between benign and malignant liver lesions, but it is unclear if this approach can be used in patients with Budd-Chiari syndrome (BCS). Thus, we aimed to assess the diagnostic utility of HBP images in patients with BCS. Methods:This retrospective study included all patients admitted to our institution with a diagnosis of BCS and focal liver lesions on hepatobiliary contrast agent-enhanced MR imaging (HBCA-MRI) from 2000 to 2019. MR images were reviewed by 2 radiologists blinded to the diagnosis of the lesions. Patient and lesion characteristics were recorded, focusing on HBP imaging features. Results:Twenty-six patients (mean 35 ± 11 years old [13-65]; 21 women [81%] 35 ± 12 years old [13-65]; 5 men [19%] 36 ± 10 years old [19-44]) with 99 benign liver lesions and 12 hepatocellular carcinomas (HCCs) were analyzed. Patients with HCC were significantly older than those with benign lesions (mean 50 ± 10 vs. 33 ± 9 years old, p = 0.003), with higher alpha-fetoprotein (AFP) levels (3/4 [75%] vs. 1/22 [5%] with AFP >15 ng/ml, p <0.001). Homogeneous hypointense signals were identified on HBP in 14 lesions, including 12/12 (100%) HCCs, and 2/99 (2%) benign lesions (p <0.001). Most benign liver lesions showed either peripheral (n = 52/99 [53%]) or homogeneous hyperintensity (n = 23/99 [23%]) on HBP. Lesions with signal hypointensity on HBP in patients with AFP serum levels >15 ng/ml were all HCCs. Conclusion:Most benign lesions showed homogeneous or peripheral hyperintensity on HBP images while all HCCs were homogeneously hypointense. HBP images are helpful to differentiate between benign lesions and HCCs and outperform other sequences. They should be systematically acquired for the characterization of focal lesions in patients with BCS. Lay summary:Hepatobiliary phase imaging is an approach that has recently been shown to discriminate between benign and malignant lesions in the liver. However, it was not known whether this imaging approach could be used effectively in patients with Budd-Chiari syndrome. Herein, we have shown that hepatobiliary phase imaging appears to be useful for differentiating between benign and malignant liver lesions in patients with Budd-Chiari syndrome.
| S-EPMC7232085 | biostudies-literature
Project description:PURPOSE:The accuracy of flow velocity and three-directional velocity components are important for the precise visualization of hemodynamics by 3D cine phase-contrast MRI (3D cine PC MRI, also referred to as 4D-flow). The aim of this study was to verify the accuracy of these measurements of prototype or commercially available 3D cine PC MRI obtained by three different manufactures' MR scanners. METHODS:The verification of the accuracy of flow velocity in 3D cine PC MRI was performed by circulating blood mimicking fluid through a straight-tube phantom in a slanting position, such that the three-directional velocity components were simultaneously measurable, using three 3T MR scanners from different manufacturers. The data obtained were processed by phase correction, and the velocity and three-directional velocity components in the center of the tube on the central cross section of a slab were calculated. The velocity profile in each three directions and the composite velocity profiles were compared with the calculated reference values, using the Hagen-Poiseuille equation. In addition, velocity profiles and the spatially time-averaged velocity perpendicular to the tube were compared with the theoretical values and measured values by a flowmeter, respectively. RESULTS:An underestimation of the maximum velocity in the center of the tube and an overestimation of the velocity near the tube wall due to partial volume effects were observed in all three scanners. A roughening and flattening of profiles in the center of the tube were observed in one scanner, due, presumably, to the low signal-to-noise ratio. However, the spatially time-averaged velocities corresponded well with the measured values by the flowmeter in all three scanners. CONCLUSION:In this study, we have demonstrated that the accuracy of flow velocity and three-directional velocity components in 3D cine PC MRI was satisfactory in all three MR scanners.
| S-EPMC6883082 | biostudies-literature
Project description:IntroductionThe Chiari II is a relatively common birth defect that is associated with open spinal abnormalities and is characterized by caudal migration of the posterior fossa contents through the foramen magnum. The pathophysiology of Chiari II is not entirely known, and the neurobiological substrate beyond posterior fossa findings remains unexplored. We aimed to identify brain regions altered in Chiari II fetuses between 17 and 26 GW.MethodsWe used in vivo structural T2-weighted MRIs of 31 fetuses (6 controls and 25 cases with Chiari II).ResultsThe results of our study indicated altered development of diencephalon and proliferative zones (ventricular and subventricular zones) in fetuses with a Chiari II malformation compared to controls. Specifically, fetuses with Chiari II showed significantly smaller volumes of the diencephalon and significantly larger volumes of lateral ventricles and proliferative zones.DiscussionWe conclude that regional brain development should be taken into consideration when evaluating prenatal brain development in fetuses with Chiari II.
| S-EPMC10149737 | biostudies-literature
Project description:Although it is not uncommon for patients with Chiari I malformations to present with respiratory complaints, cough syncope is a rare presenting symptom. We report an adult patient who harbored both a Chiari I malformation and atlanto-occipital assimilation who complained of cough syncope, orthopnea, and central sleep apnea. The patient underwent decompressive craniectomy of the posterior fossa and cervical level 2 laminectomy. However, due to a possible initial underappreciation of the profound narrowing of the foramen magnum as a result of these concomitant pathologies, the patient may have had continued impaired cerebrospinal fluid flow, leading to a symptomatic pseudomeningocele and requiring a more extensive decompression that included a cervical level 3 laminectomy as well as a temporary lumbar drain. On 2-year follow-up, he has remained asymptomatic.
| S-EPMC4110142 | biostudies-other
Project description:Compressive cervical myelopathy is a well-known life-threatening complication in mucopolysaccharidosis (MPS) patients. Glycosaminoglycan accumulation in the growing cartilage results in dens dysplasia, atlanto-axial instability, and subsequent periodontoid fibrocartilaginous tissue deposition with upper cervical stenosis.Chiari malformation type 1 (CM1) is a congenital downward cerebellar tonsil ectopia determined by clivus and posterior cranial fossa underdevelopment, possibly leading to progressive spinal cord cavitation (syringomyelia) and severe neurological impairment.We present a boy affected with Hunter syndrome (MPS II) and cerebellar tonsil ectopia who developed a holocord syringomyelia at the age of 6 years. The child underwent atlanto-occipital decompressive surgery with rapid clinical and neuroimaging improvement.Sharing a primary mesenchymal involvement of the cervical-occipital region, the coexistence of CM1 in MPS might be not unexpected and complicate further the disease course. In these patients, strict monitoring and prompt treatment might be of foremost importance for preventing major neurological complications.
| S-EPMC3897861 | biostudies-literature
Project description:ObjectivesBoth Chiari malformation type 1 (CMI, i.e., the idiopathic caudal ectopy of cerebellar tonsils into foramen magnum) and idiopathic intracranial hypertension (IIH) are characterized by reduced intracranial compliance (ICC) due to disturbed circulation of cerebrospinal fluid (CSF). An increasing body of evidence links cardiovascular disease to CSF circulation disturbances. The aim of this study was to explore whether the prevalence of cardiovascular risk factors in patients with CMI or IIH is higher than in the general population.Materials and methodsAmong the patients with CMI or IIH treated at our department during the period 2003-2014, we identified those with history of arterial hypertension (AH), myocardial infarction (MI), angina pectoris (AP), or diabetes mellitus (DM). For comparison with a control population, we retrieved information about the prevalence of AH, MI, AP, and DM among participants of the North-Trøndelag Health Study 3 (HUNT3).ResultsData from 48 CMI and 52 IIH cases were available. Compared to data from the 42,461 individuals participating in the HUNT3, we found increased prevalence of DM in male CMI as well as female IIH cases, and of AH in female IIH cases. Body mass index (BMI) was significantly increased in both female and male IIH cases. Prevalence of MI and AP in the CMI and IIH cohorts was extremely low and therefore not further studied.ConclusionsThis study provided evidence of an increased prevalence of DM in male CMI as well as female IIH cases and of AH in female IIH cases. Although requiring further exploration, these findings point to AH and DM as potential risk factors in the pathophysiology of CMI and IIH.
| S-EPMC5434183 | biostudies-literature
Project description:BackgroundPseudomeningoceles (PMCs) as abnormal collections of cerebrospinal fluid are quite common findings on follow-up MRI after Chiari decompression surgery (CDS). However, the importance of their identification has not been truly determined, especially when PMCs are described occasionally in the process of radiological follow-up. We retrospectively analyzed surgical outcomes and imaging findings after CDS depending upon the occurrence and thickness of PMCs.MethodsA total of 76 adult patients who underwent CDS were analyzed. The clinical and radiological outcomes of patients with a pseudomeningocele (wPMC) were evaluated and compared to those of patients without a pseudomeningocele (w/oPMC). Radiological morphometric measurements were performed and compared between groups. Comparisons of the maximal PMC thickness were made within the wPMC group.ResultsPMCs were recognized in 27 (35.5%) patients, of whom 3 (11.1%) required reoperation. Differences in satisfactory result rates regarding gestalt assessment and Chicago Chiari Outcome Scale were statistically insignificant between the w/oPMC and wPMC groups (p = 1 and p = 0.56, respectively). The postoperative syringomyelia decrease and cerebellar tonsil elevation were similar between the groups (p = 1 and p = 0.74, respectively) in the long-term follow-up. Additionally, the clinical or radiological outcomes with radiological details were not related to PMC thickness in the long-term follow-up. However, radiological details showed the cooccurrence of PMCs with a postsurgical of cerebello-tentorial distance increase (p < 0.05), basion-pontomedullary sulcus distance decrease (p < 0.05) and tonsillo-graft distance decrease (p < 0.05).ConclusionsWe found no significant relationships between PMC presence or thickness and clinical or radiological outcomes. However, postoperative changes within the posterior fossa associated with PMCs resemble brain sagging, which occurs in intracranial hypotension. Therefore, extradural cerebrospinal fluid escape may also be responsible for symptoms in some patients with PMCs after CDS.
| S-EPMC9406808 | biostudies-literature