Syringomyelia

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E I Bogdanov - One of the best experts on this subject based on the ideXlab platform.

  • clinical and neuroimaging features of idiopathic Syringomyelia
    Neurology, 2004
    Co-Authors: E I Bogdanov, John D Heiss, E G Mendelevich, I M Mikhaylov, A Haass
    Abstract:

    In some adult patients with cervical Syringomyelia, MRI studies do not identify primary disease within the foramen magnum or spinal canal. To identify the etiology of this idiopathic type of Syringomyelia, clinical features and posterior fossa (PF) measurements from 17 of these patients, 17 patients with Chiari I-type Syringomyelia, and 32 control subjects were compared. Idiopathic Syringomyelia and Chiari I-type Syringomyelia manifested central cervical myelopathy and a small PF with narrow CSF spaces, suggesting that they develop by the same mechanism.

  • spontaneous resolution of Syringomyelia report of two cases and review of the literature
    Neurosurgery, 2003
    Co-Authors: Kazuhiko Kyoshima, E I Bogdanov
    Abstract:

    OBJECTIVE AND IMPORTANCE: The natural history of Syringomyelia is highly unpredictable, and some patients experience improvement or stabilization without surgery. However, the mechanisms of the formation and spontaneous resolution of Syringomyelia remain controversial. This report concerns two patients with Syringomyelia who demonstrated spontaneous reductions in syrinx size, accompanied by symptomatic improvement. CLINICAL PRESENTATION: One patient was a 10-year-old girl with Syringomyelia associated with a tight cisterna magna and basilar impression, who demonstrated a spontaneous decrease in syrinx size, accompanied by symptomatic improvement, in 22 months. The other patient was a 39-year-old man with Syringomyelia associated with a Chiari I malformation, who demonstrated a spontaneous reduction in syrinx size and neurological improvement, accompanied by elevation of the cerebellar tonsils, 6 months after diagnosis. INTERVENTION: The patients were monitored. CONCLUSION: The mechanisms of spontaneous resolution of Syringomyelia, as well as the factors leading to the cerebrospinal fluid flow disturbances that cause Syringomyelia, may vary. Resolution of foramen magnum lesion-related Syringomyelia may be the result of spontaneous correction of the abnormal cerebrospinal fluid flow, as observed in our cases, or of cavity fluid drainage into the spinal arachnoid space because of spinal cord fissuring.

Victor Haughton - One of the best experts on this subject based on the ideXlab platform.

  • cervical spinal canal narrowing in idiopathic Syringomyelia
    Neuroradiology, 2016
    Co-Authors: Aaron F Struck, Carrie M Carr, Vinil Shah, John R Hesselink, Victor Haughton
    Abstract:

    Introduction The cervical spine in Chiari I patient with Syringomyelia has significantly different anteroposterior diameters than it does in Chiari I patients without Syringomyelia. We tested the hypothesis that patients with idiopathic Syringomyelia (IS) also have abnormal cervical spinal canal diameters. The finding in both groups may relate to the pathogenesis of Syringomyelia.

  • the cervical spinal canal tapers differently in patients with chiari i with and without Syringomyelia
    American Journal of Neuroradiology, 2016
    Co-Authors: A Thompson, John R Hesselink, N Madan, G Weinstein, Victor Haughton
    Abstract:

    BACKGROUND AND PURPOSE: The cause of Syringomyelia in patients with Chiari I remains uncertain. Cervical spine anatomy modifies CSF velocities, flow patterns, and pressure gradients, which may affect the spinal cord. We tested the hypothesis that cervical spinal anatomy differs between Chiari I patients with and without Syringomyelia. MATERIALS AND METHODS: We identified consecutive patients with Chiari I at 3 institutions and divided them into groups with and without Syringomyelia. Five readers measured anteroposterior cervical spinal diameters, tonsillar herniation, and syrinx dimensions on cervical MR images. Taper ratios for C1–C7, C1–C4, and C4–C7 spinal segments were calculated by linear least squares fitting to the appropriate spinal canal diameters. Mean taper ratios and tonsillar herniation for groups were compared and tested for statistical significance with a Kruskal-Wallis test. Inter- and intrareader agreement and correlations in the data were measured. RESULTS: One hundred fifty patients were included, of which 49 had Syringomyelia. C1–C7 taper ratios were smaller and C4–C7 taper ratios greater for patients with Syringomyelia than for those without it. C1–C4 taper ratios did not differ significantly between groups. Patients with Syringomyelia had, on average, greater tonsillar herniation than those without a syrinx. However, C4–C7 taper ratios were steeper, for all degrees of tonsil herniation, in patients with Syringomyelia. Differences among readers did not exceed differences among patient groups. CONCLUSIONS: The tapering of the lower cervical spine may contribute to the development of Syringomyelia in patients with Chiari I.

John D Heiss - One of the best experts on this subject based on the ideXlab platform.

  • origin of syrinx fluid in Syringomyelia a physiological study
    Neurosurgery, 2019
    Co-Authors: John D Heiss, Katie Jarvis, Rene Smith, Eric Eskioglu, Mortimer Gierthmuehlen, Nicholas J Patronas, John A Butman, Davis P Argersinger, Russell R Lonser, Edward H Oldfield
    Abstract:

    BACKGROUND: The origin of syrinx fluid is controversial. OBJECTIVE: To elucidate the mechanisms of Syringomyelia associated with cerebrospinal fluid pathway obstruction and with intramedullary tumors, contrast transport from the spinal subarachnoid space (SAS) to syrinx was evaluated in Syringomyelia patients. METHODS: We prospectively studied patients with Syringomyelia: 22 with Chiari I malformation and 16 with SAS obstruction-related Syringomyelia before and 1 wk after surgery, and 9 with tumor-related Syringomyelia before surgery only. Computed tomography-myelography quantified dye transport into the syrinx before and 0.5, 2, 4, 6, 8, 10, and 22 h after contrast injection by measuring contrast density in Hounsfield units (HU). RESULTS: Before surgery, more contrast passed into the syrinx in Chiari I malformation-related Syringomyelia and spinal obstruction-related Syringomyelia than in tumor-related Syringomyelia, as measured by (1) maximum syrinx HU, (2) area under the syrinx concentration-time curve (HU AUC), (3) ratio of syrinx HU to subarachnoid cerebrospinal fluid (CSF; SAS) HU, and (4) AUC syrinx/AUC SAS. More contrast (AUC) accumulated in the syrinx and subarachnoid space before than after surgery. CONCLUSION: Transparenchymal bulk flow of CSF from the subarachnoid space to syrinx occurs in Chiari I malformation-related Syringomyelia and spinal obstruction-related Syringomyelia. Before surgery, more subarachnoid contrast entered syringes associated with CSF pathway obstruction than with tumor, consistent with syrinx fluid originating from the subarachnoid space in Chiari I malformation and spinal obstruction-related Syringomyelia and not from the subarachnoid space in tumor-related Syringomyelia. Decompressive surgery opened subarachnoid CSF pathways and reduced contrast entry into syringes associated with CSF pathway obstruction.

  • pathophysiology of persistent Syringomyelia after decompressive craniocervical surgery clinical article
    Journal of Neurosurgery, 2010
    Co-Authors: John D Heiss, Rene Smith, Nicholas J Patronas, John A Butman, Hetty L Devroom, Giancarlo Suffredini, Francine Thomas, Edward H Oldfield
    Abstract:

    Object Craniocervical decompression for Chiari malformation Type I (CM-I) and Syringomyelia has been reported to fail in 10%–40% of patients. The present prospective clinical study was designed to test the hypothesis that in cases in which Syringomyelia persists after surgery, craniocervical decompression relieves neither the physiological block at the foramen magnum nor the mechanism of Syringomyelia progression. Methods The authors prospectively evaluated and treated 16 patients with CM-I who had persistent Syringomyelia despite previous craniocervical decompression. Testing before surgery included the following: 1) clinical examination; 2) evaluation of the anatomy using T1-weighted MR imaging; 3) assessment of the syrinx and CSF velocity and flow using cine phase-contrast MR imaging; and 4) appraisal of the lumbar and cervical subarachnoid pressures at rest, during a Valsalva maneuver, during jugular compression, and following the removal of CSF (CSF compliance measurement). During surgery, ultrasonog...

  • eLS - Syringomyelia and Related Diseases
    Encyclopedia of Life Sciences, 2010
    Co-Authors: John D Heiss, Edward H Oldfield
    Abstract:

    Syringomyelia is an uncommon medical condition in which a cyst (syrinx) develops in the spinal cord. Untreated, the syrinx will displace, injure and destroy the spinal cord, resulting in neurological deterioration in the affected region of the body. The Chiari I malformation is the most frequent cause of Syringomyelia. Symptoms of paralysis, sensory loss and chronic pain usually develop during the second through the fifth decades of life in these patients. Syringomyelia may also develop months to years after trauma or meningitis. The natural history of Syringomyelia is typically one of gradual, stepwise neurological deterioration over many years. The objective of surgical treatment of Syringomyelia is to eliminate the syrinx and prevent progression of spinal cord dysfunction, known as myelopathy. Syringomyelia usually resolves after surgical treatment that eliminates the effects of the underlying condition by opening the CSF (cerebrospinal fluid) pathways at the foramen magnum or spinal canal. Key concepts: Syringomyelia is a cyst within the spinal cord that results in dysfunction of the spinal cord, which is known as myelopathy. The fluid within the syrinx is chemically identical to normal cerebrospinal fluid (CSF) when there is an associated condition that obstructs the CSF pathway at the foramen magnum or within the spinal canal. The fluid within the syrinx has a protein content that is in excess to that of normal CSF when the syrinx is associated with a tumour within the spinal cord (an intramedullary tumour). The syrinx injures sensory nerve cell bodies (posterior horn cells) near the centre of the spinal cord and results in loss of sensation, uncomfortable sensations known as dysesthesias and perception of pain in the anatomic region supplied by the sensory nerves. The syrinx injures motor nerves cell bodies (anterior horn cells) near the centre of the spinal cord and results in loss of strength and atrophy in the muscles supplied by those nerves. The syrinx may expand over time to compress the long tracts of neurons that pass to the inferior part of the spinal cord and disrupt their function, resulting in spasticity, paralysis and loss of sensation in the lower extremities. Treatment of Syringomyelia requires a surgical procedure that successfully addresses the condition that causes Syringomyelia, by opening obstructed CSF pathways or by removing a spinal cord tumour. Successful treatment of Syringomyelia arrests the progression of Syringomyelia but results in limited improvement in signs and symptoms of spinal cord injury. Because treatment of Syringomyelia can prevent disease progression, but cannot restore neurons lost to the disease, early diagnosis and treatment is the key to maintain optimal function. Keywords: spinal cord diseases; nervous system malformations; cerebrospinal fluid; neurosurgical procedures; paralysis; intractable pain

  • clinical and neuroimaging features of idiopathic Syringomyelia
    Neurology, 2004
    Co-Authors: E I Bogdanov, John D Heiss, E G Mendelevich, I M Mikhaylov, A Haass
    Abstract:

    In some adult patients with cervical Syringomyelia, MRI studies do not identify primary disease within the foramen magnum or spinal canal. To identify the etiology of this idiopathic type of Syringomyelia, clinical features and posterior fossa (PF) measurements from 17 of these patients, 17 patients with Chiari I-type Syringomyelia, and 32 control subjects were compared. Idiopathic Syringomyelia and Chiari I-type Syringomyelia manifested central cervical myelopathy and a small PF with narrow CSF spaces, suggesting that they develop by the same mechanism.

  • Pathophysiology and Treatment of Syringomyelia
    Contemporary neurosurgery, 2003
    Co-Authors: John D Heiss, Edward H Oldfield
    Abstract:

    Learning Objectives:After reading this article, the participant should be able to:1. Identify the clinical characteristics of Syringomyelia.2. Describe the diagnostic studies and pathophysiologic concepts related to Syringomyelia.3. Recall the pathophysiological basis of treatment options for syring

Edward H Oldfield - One of the best experts on this subject based on the ideXlab platform.

  • origin of syrinx fluid in Syringomyelia a physiological study
    Neurosurgery, 2019
    Co-Authors: John D Heiss, Katie Jarvis, Rene Smith, Eric Eskioglu, Mortimer Gierthmuehlen, Nicholas J Patronas, John A Butman, Davis P Argersinger, Russell R Lonser, Edward H Oldfield
    Abstract:

    BACKGROUND: The origin of syrinx fluid is controversial. OBJECTIVE: To elucidate the mechanisms of Syringomyelia associated with cerebrospinal fluid pathway obstruction and with intramedullary tumors, contrast transport from the spinal subarachnoid space (SAS) to syrinx was evaluated in Syringomyelia patients. METHODS: We prospectively studied patients with Syringomyelia: 22 with Chiari I malformation and 16 with SAS obstruction-related Syringomyelia before and 1 wk after surgery, and 9 with tumor-related Syringomyelia before surgery only. Computed tomography-myelography quantified dye transport into the syrinx before and 0.5, 2, 4, 6, 8, 10, and 22 h after contrast injection by measuring contrast density in Hounsfield units (HU). RESULTS: Before surgery, more contrast passed into the syrinx in Chiari I malformation-related Syringomyelia and spinal obstruction-related Syringomyelia than in tumor-related Syringomyelia, as measured by (1) maximum syrinx HU, (2) area under the syrinx concentration-time curve (HU AUC), (3) ratio of syrinx HU to subarachnoid cerebrospinal fluid (CSF; SAS) HU, and (4) AUC syrinx/AUC SAS. More contrast (AUC) accumulated in the syrinx and subarachnoid space before than after surgery. CONCLUSION: Transparenchymal bulk flow of CSF from the subarachnoid space to syrinx occurs in Chiari I malformation-related Syringomyelia and spinal obstruction-related Syringomyelia. Before surgery, more subarachnoid contrast entered syringes associated with CSF pathway obstruction than with tumor, consistent with syrinx fluid originating from the subarachnoid space in Chiari I malformation and spinal obstruction-related Syringomyelia and not from the subarachnoid space in tumor-related Syringomyelia. Decompressive surgery opened subarachnoid CSF pathways and reduced contrast entry into syringes associated with CSF pathway obstruction.

  • pathophysiology of persistent Syringomyelia after decompressive craniocervical surgery clinical article
    Journal of Neurosurgery, 2010
    Co-Authors: John D Heiss, Rene Smith, Nicholas J Patronas, John A Butman, Hetty L Devroom, Giancarlo Suffredini, Francine Thomas, Edward H Oldfield
    Abstract:

    Object Craniocervical decompression for Chiari malformation Type I (CM-I) and Syringomyelia has been reported to fail in 10%–40% of patients. The present prospective clinical study was designed to test the hypothesis that in cases in which Syringomyelia persists after surgery, craniocervical decompression relieves neither the physiological block at the foramen magnum nor the mechanism of Syringomyelia progression. Methods The authors prospectively evaluated and treated 16 patients with CM-I who had persistent Syringomyelia despite previous craniocervical decompression. Testing before surgery included the following: 1) clinical examination; 2) evaluation of the anatomy using T1-weighted MR imaging; 3) assessment of the syrinx and CSF velocity and flow using cine phase-contrast MR imaging; and 4) appraisal of the lumbar and cervical subarachnoid pressures at rest, during a Valsalva maneuver, during jugular compression, and following the removal of CSF (CSF compliance measurement). During surgery, ultrasonog...

  • eLS - Syringomyelia and Related Diseases
    Encyclopedia of Life Sciences, 2010
    Co-Authors: John D Heiss, Edward H Oldfield
    Abstract:

    Syringomyelia is an uncommon medical condition in which a cyst (syrinx) develops in the spinal cord. Untreated, the syrinx will displace, injure and destroy the spinal cord, resulting in neurological deterioration in the affected region of the body. The Chiari I malformation is the most frequent cause of Syringomyelia. Symptoms of paralysis, sensory loss and chronic pain usually develop during the second through the fifth decades of life in these patients. Syringomyelia may also develop months to years after trauma or meningitis. The natural history of Syringomyelia is typically one of gradual, stepwise neurological deterioration over many years. The objective of surgical treatment of Syringomyelia is to eliminate the syrinx and prevent progression of spinal cord dysfunction, known as myelopathy. Syringomyelia usually resolves after surgical treatment that eliminates the effects of the underlying condition by opening the CSF (cerebrospinal fluid) pathways at the foramen magnum or spinal canal. Key concepts: Syringomyelia is a cyst within the spinal cord that results in dysfunction of the spinal cord, which is known as myelopathy. The fluid within the syrinx is chemically identical to normal cerebrospinal fluid (CSF) when there is an associated condition that obstructs the CSF pathway at the foramen magnum or within the spinal canal. The fluid within the syrinx has a protein content that is in excess to that of normal CSF when the syrinx is associated with a tumour within the spinal cord (an intramedullary tumour). The syrinx injures sensory nerve cell bodies (posterior horn cells) near the centre of the spinal cord and results in loss of sensation, uncomfortable sensations known as dysesthesias and perception of pain in the anatomic region supplied by the sensory nerves. The syrinx injures motor nerves cell bodies (anterior horn cells) near the centre of the spinal cord and results in loss of strength and atrophy in the muscles supplied by those nerves. The syrinx may expand over time to compress the long tracts of neurons that pass to the inferior part of the spinal cord and disrupt their function, resulting in spasticity, paralysis and loss of sensation in the lower extremities. Treatment of Syringomyelia requires a surgical procedure that successfully addresses the condition that causes Syringomyelia, by opening obstructed CSF pathways or by removing a spinal cord tumour. Successful treatment of Syringomyelia arrests the progression of Syringomyelia but results in limited improvement in signs and symptoms of spinal cord injury. Because treatment of Syringomyelia can prevent disease progression, but cannot restore neurons lost to the disease, early diagnosis and treatment is the key to maintain optimal function. Keywords: spinal cord diseases; nervous system malformations; cerebrospinal fluid; neurosurgical procedures; paralysis; intractable pain

  • Pathophysiology and Treatment of Syringomyelia
    Contemporary neurosurgery, 2003
    Co-Authors: John D Heiss, Edward H Oldfield
    Abstract:

    Learning Objectives:After reading this article, the participant should be able to:1. Identify the clinical characteristics of Syringomyelia.2. Describe the diagnostic studies and pathophysiologic concepts related to Syringomyelia.3. Recall the pathophysiological basis of treatment options for syring

Jeanvalery Coumans - One of the best experts on this subject based on the ideXlab platform.

  • spontaneous improvement of chiari i malformation and Syringomyelia in a patient with cystic fibrosis case report
    Neurosurgery, 2016
    Co-Authors: Arjun Khanna, Jeanvalery Coumans
    Abstract:

    BACKGROUND AND IMPORTANCE: Syringomyelia is highly associated with Chiari I malformation, but the pathophysiologic mechanism of syrinx formation and its relation to downward cerebellar tonsillar displacement remains elusive. Cough, Valsalva maneuver, and other physiological strains transiently exacerbate the clinical symptoms of these conditions, exert profound effects on the flow dynamics across the craniospinal junction, and are thought to play an important role in the pathogenesis of Syringomyelia. CLINICAL PRESENTATION: We report the case of a patient with cystic fibrosis who presented during an exacerbation of bronchiectasis and was found to have a Chiari I malformation with associated Syringomyelia. Eight months later, when the patient had returned to baseline pulmonary status, repeat imaging showed interval improvement in both the size of the syrinx and descent of cerebellar tonsils. CONCLUSION: This rare case of spontaneous improvement of Syringomyelia and Chiari I malformation attributable to relief from chronic cough offers interesting insight into the mechanism of these disorders. ABBREVIATIONS: FEV1, forced expiratory volume in 1 secondFVC, forced vital capacity.

  • volumetric analysis of Syringomyelia following hindbrain decompression for chiari malformation type i Syringomyelia resolution follows exponential kinetics
    Neurosurgical Focus, 2011
    Co-Authors: Jeanvalery Coumans, Brian P Walcott, William E Butler, Brian V Nahed, Kristopher T Kahle
    Abstract:

    Object Resolution of Syringomyelia is common following hindbrain decompression for Chiari malformation, yet little is known about the kinetics governing this process. The authors sought to establish the volumetric rate of Syringomyelia resolution. Methods A retrospective cohort of patients undergoing hindbrain decompression for a Chiari malformation Type I with preoperative cervical or thoracic Syringomyelia was identified. Patients were included in the study if they had at least 3 neuroimaging studies that detailed the entirety of their preoperative Syringomyelia over a minimum of 6 months postoperatively. The authors reconstructed the MR images in 3 dimensions and calculated the volume of the Syringomyelia. They plotted the Syringomyelia volume over time and constructed regression models using the method of least squares. The Akaike information criterion and Bayesian information criterion were used to calculate the relative goodness of fit. The coefficients of determination R2 (unadjusted and adjusted) ...