Spinal cord edema preceding syringomyelia associated with Chiari I malformation--case report.
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A 38-year-old woman with Chiari I malformation presented with spinal cord edema preceding syringomyelia manifesting as a 5-month history of nuchal pain and numbness of the upper extremities. Magnetic resonance imaging showed spinal cord edema, a poorly defined syrinx at the C-2 to T-2 levels, and distorted cerebellar tonsils. Computed tomography revealed cerebrospinal fluid (CSF) density in the center of spinal cord edema, and positron emission tomography revealed no uptake of L-[methyl-11C]methionine, indicating a non-neoplastic lesion. Craniocervical decompression achieved excellent clinical and neuroradiological outcomes. The success of surgical treatment supports the theory that patients with Chiari I malformation have increased transmural flow of CSF, causing spinal cord edema that progresses to syringomyelia. Early treatment of patients with spinal cord edema is indicated to prevent permanent spinal cord injury due to progressive syringomyelia. (+info)
A patient with optic pathway glioma, scoliosis, Chiari type I malformation and syringomyelia: is it Neurofibromatosis type 1?
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A 22 years old girl had features of optic pathway glioma, scoliosis, Chiari type 1 malformation and cervical syringomyelia. She had no cutaneous lesions. We considered this combination to be more than coincidental and argue in favour of considering the case as a variant form of Neurofibromatosis type 1. The relevent literature in favour of our contention has been reviewed. (+info)
Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics.
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OBJECTIVES: Despite many hypotheses, the pathophysiology of syringomyelia is still not well understood. In this report, the authors propose a hypothesis based on analysis of cerebrospinal fluid dynamics in the spine. METHODS: An electric circuit model of the CSF dynamics of the spine was constructed based on a technique of computational fluid mechanics. With this model, the authors calculated how a pulsatile CSF wave coming from the cranial side is propagated along the spinal cord. RESULTS: Reducing the temporary fluid storage capacity of the cisterna magna dramatically increased the pressure wave propagated along the central canal. The peak of this pressure wave resided in the mid-portion of the spinal cord. CONCLUSIONS: The following hypotheses are proposed. The cisterna magna functions as a shock absorber against the pulsatile CSF waves coming from the cranial side. The loss of shock absorbing capacity of the cisterna magna and subsequent increase of central canal wall pressure leads to syrinx formation in patients with Chiari I malformation. (+info)
Peak systolic and diastolic CSF velocity in the foramen magnum in adult patients with Chiari I malformations and in normal control participants.
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BACKGROUND AND PURPOSE: Abnormal flow of CSF through the foramen magnum has been implicated in the pathogenesis of clinical deficits in association with Chiari I malformation. The purpose of this study was to test the hypothesis that peak CSF velocities in the foramen magnum are increased in patients with Chiari I malformations. METHODS: Eight adult patients with symptomatic Chiari I malformations and 10 adult volunteers were studied with cardiac gated, phase-contrast MR imaging in the axial plane at the foramen magnum. The spatial uniformity of flow velocity in the foramen magnum was assessed at 14 time frames within the R-R interval. The velocity in each of the voxels at each of the time frames was calculated, and the peak systolic and diastolic velocities were tabulated for the patients and controls. RESULTS: For the normal volunteers, the CSF velocities in the subarachnoid space were relatively uniform throughout the subarachnoid space at each of the time frames. Peak systolic velocity ranged from 1.2 to 3.3 cm/s, and peak diastolic velocity ranged from 1.6 to 4.5 cm/s. In symptomatic patients with Chiari I, velocities in the foramen magnum did not appear as uniform throughout the subarachnoid space in the phase-contrast images. Peak systolic velocities ranged from 1.8 to 4.8 cm/s, and peak diastolic velocities ranged from 2.5 to 5.3 cm/s. Peak systolic velocity was significantly higher (P =.01) in the patients than in the control volunteers. CONCLUSION: Patients with Chiari I malformations have significant elevations of peak systolic velocity in the CSF in the foramen magnum. (+info)
Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients.
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The present study shows the results of 53 patients who have been treated surgically for basilar impression (BI), Chiari malformation (CM), and syringomyelia (SM). The patients were divided into two groups. Group I (24 patients) underwent osteodural decompression with large inferior occipital craniectomy, laminectomy from C 1 to C 3, dural opening in Y format, dissection of arachnoid adhesion between the cerebellar tonsils, medulla oblongata and spinal cord, large opening of the fourth ventricle and dural grafting with the use of bovine pericardium. Group II patients (29 patients) underwent osteodural-neural decompression with the same procedures described above plus dissection of the arachnoid adherences of the vessels of the region of the cerebellar tonsils, and tonsillectomy (amputation) in 10 cases, and as for the remainning 19 cases, intrapial aspiration of the cerebellar tonsils was performed. The residual pial sac was sutured to the dura in craniolateral position. After completion of the suture of the dural grafting, a thread was run through the graft at the level of the created cisterna magna and fixed to the cervical aponeurosis so as to move the dural graft on a posterior- caudal direction, avoiding, in this way, its adherence to the cerebellum. (+info)
Preoperative radiological and electrophysiological evaluation in 100 adolescent idiopathic scoliosis patients.
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This is a prospective study of spinal magnetic resonance imaging (MRI), electrophysiological recordings, and neurological examinations of 100 patients admitted for surgery for adolescent idiopathic scoliosis (AIS), which was conducted to assess the prevalence of structural and functional abnormalities within the spinal cord in patients with clinically normal neurologic condition. In all patients the clinical diagnosis and intact neurological condition was ascertained by a spinal orthopedic surgeon. Full-length spinal axis MRI studies (T1/T2 sequences) and somato-sensory evoked potentials of the tibial nerves (tSSEPs) were preoperatively assessed by independent evaluators blinded to the patients' medical histories. Structural spinal cord abnormalities were found in three of 100 AIS patients on MR imaging. In one patient a Chiari malformation type 1 with an accompanying syringomyelia was diagnosed, which required a suboccipital decompression. In the other two patients small thoracic syringomyelias were diagnosed. Abnormalities of spinal cord function were detected in 68% of the 100 patients: tSSEP latencies corrected for body height were increased in 56% of the patients; pathological differences between tSSEPs on the left and right sides were present in 17% (12% in combination with a prolongation of the latency). The findings of this study indicate that MRI and electrophysiological examinations are essential to assess spinal cord abnormalities that are clinically not detectable in AIS patients. Even in patients with intact neurologic condition and clinically typical right-curved thoracic scoliosis, the possibility of intraspinal pathologies should be ruled out by MRI. It is especially important to detect structural pathologies like syringomyelia and Chiari malformation before proceeding with scoliosis surgery, as these conditions are associated with a higher neurological risk during scoliosis surgery. The electrophysiological recordings made in the present study, with the high number of pathological tSSEPs, are indicative of functional abnormalities with a subclinical involvement of the recorded neuronal pathways. The relevance of the latter findings is not yet clear, but pre-operative tSSEP examinations offer the possibility of assessing alterations in spinal cord function that are undetectable by clinical examination. (+info)
Craniocervical CT and MR imaging of Schwartz-Jampel syndrome.
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Schwartz-Jampel syndrome is a rare, inherited disorder characterized by myotonia, skeletal deformities, facial dysmorphism, and growth retardation. In this report of an adolescent male patient with Schwartz-Jampel syndrome, CT and MR imaging revealed basilar invagination, platybasia, Chiari I malformation, hyperpneumatized mastoids with intramastoid dural sinuses, platyspondyly, bulbous zygoma, and blunted pterygoid processes. (+info)
Growth hormone insensitivity syndrome associated with syringomyelia and type I Chiari malformation.
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A 49-year-old man with syringomyelia and a Type I Arnold-Chiari malformation (Chiari-I) was diagnosed with growth hormone insensitivity syndrome (GHIS). He was short in stature, had high circulating levels of GH, and low circulating levels of insulin-like growth factor-I (IGF-I) and IGF binding protein-3 (IGFBP-3). His GH responses to the administration of growth hormone-releasing hormone (GHRH) and L-DOPA were normal, but his levels of IGF-I and IGFBP-3 did not increase after the administration of exogenous GH. Direct genomic DNA sequencing revealed neither a mutation nor deletion in this patient's GH receptor (GHR) gene, though one polymorphism was detected, indicating that his GHR gene was normal. This is the first reported case of an association of GHIS with syringomyelia and Chiari-I malformation. (+info)