Materiały źródłowe

• #1 Syringomyelia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537110/

  Syringomyelia is a condition characterized by abnormal cerebrospinal fluid circulation, leading to the formation of fluid-filled cavities (syrinx) within the spinal cord parenchyma or central canal. […] Syringomyelia develops due to a disturbance in CSF flow dynamics, often resulting from spinal subarachnoid space obstruction in most cases. […] Authors have proposed various theories to explain the origin and progression of syringomyelia. […] Gardner and Angel introduced the „hydrodynamic theory,” suggesting that hindbrain hernias obstruct normal CSF egress, causing fluid to divert into the central spinal canal and pulsate with each heartbeat, which is also known as the „water-hammer” effect. […] Williams observed pressure gradients exceeding 100 mm Hg across the foramen magnum during Valsalva maneuvers in patients with hindbrain hernias.

• #2 Syringomyelia: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1151685-overview

  Syringomyelia is the development of a fluid-filled cavity or syrinx within the spinal cord. […] Although many mechanisms for syrinx formation have been postulated, the exact pathogenesis is still unknown. Frequently cited theories are those of Gardner, William, and Oldfield. […] This theory proposes that syringomyelia results from a „water hammer”-like transmission of pulsatile CSF pressure via a communication between the fourth ventricle and the central canal of the spinal cord through the obex. A blockage of the foramen of Magendie initiates this process. […] This theory proposes that syrinx development, particularly in patients with Chiari malformation, follows a differential between intracranial pressure and spinal pressure caused by a valvelike action at the foramen magnum. […] Downward movement of the cerebellar tonsils during systole can be visualized with dynamic MRI. This oscillation creates a piston effect in the spinal subarachnoid space that acts on the surface of the spinal cord and forces CSF through the perivascular and interstitial spaces into the syrinx raising intramedullary pressure.

• #2 Update on the pathophysiology and management of syringomyelia unrelated to Chiari malformation | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-update-on-pathophysiology-management-syringomyelia-S2173580818301561

  Much has been published on syringomyelia related to Chiari malformation. In contrast, little is known about the condition when it is not associated with this malformation, but this presentation of syringomyelia constitutes a different entity and therefore requires specific management. […] Syringomyelia is almost inherently thought to be accompanied by Chiari malformation, due to the large body of literature on the association between these 2 entities. However, syringomyelia is a distinct entity that may be caused by a wide range of conditions. This literature review focuses on the aetiopathogenesis of syringomyelia and the different treatment options for syringomyelia unrelated to Chiari malformation. […] Over the years, numerous theories have attempted to explain the pathogenesis of syringomyelia. We still lack a theory capable of explaining all the possible scenarios that may cause syringomyelia. Although the intramedullary pulse pressure theory proposed by Greitz seems to fulfil this goal, it is yet to be universally accepted; some researchers continue to support other theories that have already been rejected by the scientific community.

• #3 Update on the pathophysiology and management of syringomyelia unrelated to Chiari malformation | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-avance-resumen-update-on-pathophysiology-management-syringomyelia-S2173580818301561

  Much has been published on syringomyelia related to Chiari malformation. In contrast, little is known about the condition when it is not associated with this malformation, but this presentation of syringomyelia constitutes a different entity and therefore requires specific management. […] Syringomyelia is a distinct entity that may be caused by a wide range of conditions. This literature review focuses on the aetiopathogenesis of syringomyelia and the different treatment options for syringomyelia unrelated to Chiari malformation. […] Over the years, numerous theories have attempted to explain the pathogenesis of syringomyelia. We still lack a theory capable of explaining all the possible scenarios that may cause syringomyelia. […] Traditional and recent theories agree that syringomyelia results from abnormal CSF circulation within the spinal subarachnoid space.

• #3 Syringomyelia intermittens: highlighting the complex pathophysiology of syringomyelia. Illustrative case in: Journal of Neurosurgery: Case Lessons Volume 2 Issue 11 (2021) Journals

  https://thejns.org/caselessons/view/journals/j-neurosurg-case-lessons/2/11/article-CASE21341.xml

  Chiari Type I malformation (CM1) is a disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Syringomyelia is frequently found in patients with CM1, but the pathophysiology of syringomyelia remains an enigma. […] The unusual course of this patient highlights the importance of guiding treatment by clinical symptoms, not radiological findings. Furthermore it reflects the complexity of the pathophysiology and the uncertain natural history of syringomyelia. […] The exact pathophysiology of syringomyelia due to CM1 remains frustratingly uncertain. Five theories have been proposed. The first theory was proposed by Gardner and Angel in 1958; the water-hammer theory states that partial obstruction of the outflow of cerebrospinal fluid (CSF) from the fourth ventricle directs the systolic pulsations of CSF from the fourth ventricle through a patent central canal.

• #4 Syringomyelia – wikidoc

  https://www.wikidoc.org/index.php/Syringomyelia

  The exact pathogenesis of syringomyelia remains unknown. Several theories have been postulated about the development of a syrinx in the spinal cord. The underlying mechanisms of the disease involves disruption of CNS flow, subsequent formation of a syrinx, and enlargement of the lesion to impinge on the surrounding nerve fibers resulting in a symptomatic presentation. […] The pathogenesis of a syrinx is largely unknown. There are five primary theories of intra and extra cannilicular […] Gardner’s hydrodynamic theory: Blockage of the foramen of Magendie at the obex results in a pulsatile „water-hammer” effect of the mass lesion on the spinal cord CSF. This pressure differential results in extravasation of fluid into the perivascular and extracellular spaces. […] Oldfield’s theory: The pulsatile waves of the CSF which occur during systole result in increased ICP. This increased pressure herniates the cerebellum to obstruct the subarachnoid space at the level of the foramen magnum. This pulsatile fluid wave against the surface of the spinal cord results in extravasation of fluid into the parenchyma and creates a syrinx.

• #5 Pathophysiology – SCIRE Professional

  https://scireproject.com/evidence/syringomyelia/introduction/pathophysiology/

  The pathophysiology of syringomyelia following SCI is not completely understood. The most supported theory is Williams Cranial-Spinal Pressure Dissociation Theory which involves formation of the cavity and its enlargement and extension. Initially, at the site of SCI, a cavity forms after liquefaction of cord tissue or hematoma. The liquefaction and cyst formation at the site has been linked to microinfarcts and the release of cellular enzymes. Cyst formation results in partial obstruction of cerebral spinal fluid movement, creating a pressure gradient between the intracranial space and spinal space. The second phase, cyst enlargement and extension, is the result of this pressure gradient which has been linked to two mechanisms affecting fluid dynamics, slosh and suck. The slosh is due to increased epidural venous pressure and CSF movement which are triggered by everyday activities such as coughing and sneezing. This pressure causes areas of structural weakness in the cord leading to proximal and distal extension of the syrinx. The second mechanism suck is the result of a partial subarachnoid block. As the fluid is initially forced up due to increased epidural venous pressure, it returns slowly creating a pressure gradient across the partial subarachnoid block with negative pressure caudal to it. This contributes to the syrinx formation and progression.

• #6 Syringomyelia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537110/

  Oldfield et al proposed that a CSF pressure wave generated by the systolic „piston-like” caudal displacement contracts the cord, forcing fluid into the syrinx cavity from the spinal subarachnoid space. […] Gretiz, Koyanagi, and Houkinkoya have proposed a theory supporting the extracellular nature of syrinx fluid rather than CSF. […] Classic communicating theory posits that there is anatomical communication between the syrinx and the fourth ventricle. […] Gardner and Angel postulated that atresia of the foramina of the fourth ventricle causes systolic water hammer from pulse wave, causing hydromyelia and herniating structures through the foramen magnum to act as a one-way pump and valve. […] In contrast, Williams argued against systolic pulse waves directly causing syringomyelia. […] Ball and Dayan observed that CSF pulse waves are typically insufficient to form a syrinx by entering an open central canal.

• #7 Syringomyelia: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1151685-overview

  The here-proposed intramedullary pulse pressure theory instead suggests that syringomyelia is caused by increased pulse pressure in the spinal cord and that the syrinx consists of extracellular fluid. A new principle is introduced implying that the distending force in the production of syringomyelia is a relative increase in pulse pressure in the spinal cord compared to that in the nearby subarachnoid space. The formation of a syrinx then occurs by the accumulation of extracellular fluid in the distended cord.

• #8 Update on the pathophysiology and management of syringomyelia unrelated to Chiari malformation | Neurología (English Edition)

  https://www.elsevier.es/en-revista-neurologia-english-edition–495-articulo-update-on-pathophysiology-management-syringomyelia-S2173580818301561

  Traditional and recent theories agree that syringomyelia results from abnormal CSF circulation within the spinal subarachnoid space. Early theories postulated that abnormal CSF circulation was caused by spinal trauma secondary to an ischaemic lesion, leading to spinal cystic degeneration and the formation of a large cavity. […] The previously mentioned intramedullary pulse pressure theory, postulated by Greitz in 1995, is probably the most widely accepted and serves as the basis for most current articles. In addition to explaining the symptoms independently of the cause, this theory presents the best performing and currently most widely used treatment option. […] Though rare, syringomyelia unrelated to Chiari malformation is a distinct entity whose aetiopathogenesis requires further study. A deeper understanding of the condition may help physicians determine which patients are eligible for surgery and choose the most appropriate option for aetiological treatment, as this approach has been found to be more effective than other options in the long term.

• #9 Pathophysiology of primary spinal syringomyelia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3787878/

  The pathogenesis of syringomyelia in patients with an associated spinal lesion is incompletely understood. The authors hypothesized that in primary spinal syringomyelia, a subarachnoid block effectively shortens the length of the spinal subarachnoid space (SAS), reducing compliance and the ability of the spinal theca to dampen the subarachnoid CSF pressure waves produced by brain expansion during cardiac systole. This creates exaggerated spinal subarachnoid pressure waves during every heartbeat that act on the spinal cord above the block to drive CSF into the spinal cord and create a syrinx. […] These findings are consistent with the theory that a spinal subarachnoid block increases spinal subarachnoid pulse pressure above the block, producing a pressure differential across the obstructed segment of the SAS, which results in syrinx formation and progression.

• #10 Pathophysiology of primary spinal syringomyelia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3787878/

  We postulate that the development and progression of PSS results from a mechanism of several steps that begins with obstruction of CSF flow within the SAS. This obstruction affects spinal CSF dynamics because the SAS accepts the fluid that is displaced from the intracranial subarachnoid cisterns as the brain expands during cardiac systole. In PSS, a subarachnoid block shortens the length of the SAS, reducing its compliance and impairing its ability to dampen the subarachnoid CSF pressure waves produced by brain expansion during cardiac systole. Spinal subarachnoid pressure waves that occur with every heartbeat would consequently have greater amplitude (pulse pressure) than normal and would act on the spinal cord above the block to drive CSF into the spinal cord, creating spinal cord edema that later coalesces into a syrinx.

• #11 Syringomyelia Is Associated with a Reduction in Spinal Canal Compliance, Venous Outflow Dilatation and Glymphatic Fluid Obstruction

  https://www.mdpi.com/2077-0383/12/20/6646

  It has now been recognised that this same phenomenon could produce an increase in venous pressure within the cord in syringomyelia. […] The purpose of the current paper to describe the interaction between spinal canal compliance, venous dilatation and interstitial fluid outflow obstruction in syringomyelia. […] The findings from patients with multiple sclerosis (MS) appear to be similar to those found for patients with hydrocephalus. […] The finding of reduced compliance in syringomyelia is not new. […] In Chiari 1 malformation with syrinx formation, local cervical compliance is reduced by 45% with a 44% increase in CSF pulse pressure. […] The reduction in craniospinal compliance within the CSF space surrounding the spinal cord alters the impedance matching between the venule and the extradural veins dilating the venule due to an alteration in impedance pumping.

• #12 Syringomyelia Is Associated with a Reduction in Spinal Canal Compliance, Venous Outflow Dilatation and Glymphatic Fluid Obstruction

  https://www.mdpi.com/2077-0383/12/20/6646

  The dilated vein narrows the perivenular space and increases the resistance to interstitial glymphatic fluid flow. […] The back-up of interstitial fluid increases the pressure in the interstitial space and there is a development of edema. […] The edema fluid begins to coalesce, forming small cystic spaces which eventually join up into a larger syrinx. […] The purpose of this proposed mechanism of syringomyelia is to highlight that treatments for this condition should be aimed at maximising craniospinal compliance. […] There is a correlation between hydrocephalus, multiple sclerosis and syringomyelia, with each having a reduction in craniospinal compliance.

• #13 The Biochemistry of Syringomyelia | Neupsy Key

  https://neupsykey.com/the-biochemistry-of-syringomyelia/

  The presence of endothelial barrier antigen and loss of functional integrity, as assessed by extravasation of intravascular horseradish peroxidase, found a prolonged structural and functional disruption of the blood-spinal cord barrier in this animal model. […] This disturbed blood-spinal cord barrier may contribute to fluid ingress into a syrinx. […] The stenotic sections of the central canal so formed have been postulated to act as an initial site for fluid build-up into a cyst. […] The functions of ependyma and tanycytes in human spinal cord may be less important than in the brain but may still play a role after cellular destruction in the spinal cord and provide an interesting avenue for further investigation. […] Inflammation, therefore, probably contributes to syrinx pathogenesis in some cases by leading to arachnoid adhesion formation and the hydrodynamic effects that this produces, rather than by acting as the main pathogenic mechanism.

• #14 Syringomyelia | Neurosurgery Inselspital Bern

  https://neurochirurgie.insel.ch/en/diseases-specialities/spinal-disorders/syringomyelia

  Syringomyelia is always caused by a cerebrospinal fluid circulation disorder, i.e., an impairment of cerebrospinal fluid flow. […] It is assumed that a blockage of the CSF passage between the spinal cord membranes or also disturbances of the mobility of the spinal cord due to scars or other malformations are the triggering factors. As a result, cavities form in the spinal cord, predominantly in the cervical spine. […] Congenital syringomyelia is usually due to a Chiari malformation. In this developmental disorder, the cerebellum is displaced into the foramen magnum, the exit canal of the spinal cord from the base of the skull. As a result, the cerebrospinal fluid circulation in the craniocervical region is disturbed. […] Acquired or secondary syringomyelia occurs as a result of trauma.

• #15 Syringohydromyelia and Syringomyelia Imaging: Practice Essentials, Radiography, Magnetic Resonance Imaging

  https://emedicine.medscape.com/article/344599-overview

  Syringomyelia develops as a consequence of intramedullary cystic lesions and cerebrospinal fluid (CSF) flow disturbance around the spinal cord. […] The presence of fluid flow voids in the syrinx cavity is not a differentiating characteristic of syringomyelia; flow voids may be seen in cases associated with Chiari malformations, trauma, and adhesive arachnoiditis. Most cavities associated with intramedullary tumors do not demonstrate a flow void, although rare exceptions have been reported. […] Obstruction of the foramen magnum in patients with Chiari I malformation causes an abrupt systolic downward displacement of the spinal cord and impairs the recoil of CSF during diastole. These observations may have pathophysiologic implications. […] Because there is an impact of Chiari I malformation-associated syringohydromyelia on morbidity and surgical intervention, its diagnosis is critical. Identifying related variables on the basis of imaging may also help identify patients at risk of syrinx formation during their course of disease. A retrospective analysis of the MR imaging studies of 108 consecutive cases of Chiari I malformation considered multiple factors associated with syrinx formation. Craniocervical junction osseous anomalies and a skull base angle of more than 135 were most predictive of syrinx formation. Patients younger than 18 years who developed syringohydromyelia were more likely to have associated skull base osseous anomalies than older individuals. […] The appearance of these flow voids may vary, depending on the imaging parameters used. As proposed by some authors, these dynamic movements may cause syrinx propagation; therefore, the observation of fluid flow voids within a syrinx cavity may have prognostic significance.

• #16

  https://journals.lww.com/neurosurgery/fulltext/2017/09001/pathogenesis_of_chiari_i___pathophysiology_of.16.aspx

  I focus here on 4 important issues related to the Chiari I malformation and syringomyelia. By what mechanism does a Chiari I malformation produce syringomyelia? What is the pathogenesis of the Chiari I malformation? Are the mechanisms underlying development of a Chiari I malformation and syringomyelia similar? […] The optimal treatment is the least surgery that is safe and that eliminates the mechanism underlying the symptoms and progression. And that begs the question of what mechanism underlies the pathogenesis of the Chiari I malformation and of development and progression of syringomyelia. […] Our observations during our initial clinical study suggested a previously unrecognized mechanism for progression of syringomyelia associated with occlusion of the SAS at the foramen magnum. […] Thus, successful treatment requires only that we eliminate that block to the rapid movement of CSF in the SAS at the foramen magnum. The study indicated that the mechanism of the development and progression of syringomyelia is on the outside, not the inside, of the spinal cord.

• #17 Syringomyelia | Neurosurgery Inselspital Bern

  https://neurochirurgie.insel.ch/en/diseases-specialities/spinal-disorders/syringomyelia

  Chronic inflammation can occur after meningitis, hemorrhage, or surgery within the meninges, also with arachnoiditis, an inflammation of the arachnoid membrane surrounding the brain. […] Extramedullary tumors, osteophytes, or chronic large disc hernias cause compression of the CSF drainage pathways. […] The symptoms of syringomyelia usually develop slowly and depend more on whether the cavity is in the cervical or thoracic spine and less on its size. […] The leading symptom is a dissociated sensory disturbance. […] Damage to motor neurons results in muscle weakness and incomplete paralysis (paresis) of the upper extremities at the level of syringomyelia. […] If the syringomyelia is not due to a definite cause, the spinal cord is decompressed and freed from any membranes that impede CSF flow and cause the syrinx. […] In order for CSF circulation to occur unimpeded, duraplasty is performed in some patients.

• #18 Syringomyelia – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/syringomyelia/symptoms-causes/syc-20354771

  Cerebrospinal fluid usually flows around the outside of the brain and spinal cord. In people with syringomyelia, cerebrospinal fluid collects inside the spinal cord and forms a fluid-filled cyst. The cyst is sometimes called a syrinx. […] Syringomyelia has several possible causes. Many cases are associated with a Chiari malformation. This is a condition in which brain tissue pushes into the spinal canal. […] It’s not clear how and why syringomyelia happens. When it develops, the fluid that surrounds, cushions and protects the brain and spinal cord collects within the spinal cord itself. This fluid is called cerebrospinal fluid. If it collects and forms a fluid-filled cyst, it is called a syrinx. […] Several conditions and diseases can lead to syringomyelia, including: Chiari malformation, a condition in which brain tissue pushes into the spinal canal and blocks the regular flow of cerebrospinal fluid. […] Spinal cord injury, which can cause symptoms months or years later.

• #19 The Physiological Occlusion of the Central Canal May Be a Prerequisite for Syringomyelia Formation

  https://www.e-neurospine.org/journal/view.php?doi=10.14245/ns.2346834.417

  Syringomyelia is a common central nervous system disease characterized by the dilation of the central canal (CC). […] The precise pathogenesis remains unknown. […] However, clinical cases like spinal tumors/stenosis with SAS obstruction often lack syringomyelia. […] These findings suggest SAS obstruction alone may not underlie syringomyelia pathogenesis. […] CC occlusion may represent a physiological prerequisite for syringomyelia formation, while SAS obstruction serves to initiate disease onset. […] The impairment of ependymal cilia appears to facilitate progression of syringomyelia. […] Collectively, these findings challenge the notion that SAS obstruction alone underlies syringomyelia pathogenesis. […] CC occlusion may represent a necessary condition for syringomyelia formation by disrupting CSF dynamics. […] Overall, concurrent with CC occlusion, extensive cilia impairment along the CC critically slowed flow and may promote CC dilation and syringomyelia development. […] This work highlights the need to look beyond SAS CSF flow and consider the multifaceted roles of CC morphology and ciliary function.

• #20 The Biochemistry of Syringomyelia | Neupsy Key

  https://neupsykey.com/the-biochemistry-of-syringomyelia/

  Plasma ECF/ISF ICF CSF Syrinxa Sodium (mM) 135145 138142 515 135154 145150 Potassium (mM) 3.44.7 3.85 135155 2.63.1 2.62.8 pH 7.357.45 7.44 7.37.6 Chloride (mM) 99108 118 9.0 115130 120123 Calcium (mM) 2.12.6 11.5 1.051.7 Protein (g/L) 5080 0.152 0.350.89 Glucose (mM) 3.96.1 2.84.2 36.1 Magnesium (mM) 0.71.0 1.02.3 1.092.1 Osmolality (mM) 280296 280296 280296 280296 280296 Amino acids (g/L) 2.62 0.72 […] The only significant difference between CSF and syrinx fluid was a higher protein content in the syrinx. […] The influence of these neuroactive substances on spinal structures and any link with syrinx pathogenesis is not well established, although a major role in syrinx formation is unlikely. […] Syrinx fluid has been reported as having different protein content from that of CSF or plasma, and this may have implications for syrinx pathogenesis.

• #21 The Biochemistry of Syringomyelia | Neupsy Key

  https://neupsykey.com/the-biochemistry-of-syringomyelia/

  The observed degree of difference may be influenced by whether the CSF is taken from the lumbar cistern, rather than adjacent to the syrinx. […] This difference in CSF content may be related to syrinx-induced cell damage and an inflammatory response in these animals. […] The protein content of syrinx fluid may be slightly higher than CSF, except in some post-traumatic cases where this difference is much greater. […] Although much has been published regarding the makeup of plasma and CSF protein compartments, there is nothing published on the specific proteins and amino acids that are present in syrinx fluid. […] These proteins may have implications in syrinx initiation, development or maintenance. […] As we understand the biochemical processes underlying the pathogenesis of syringomyelia, new biomarkers in the CSF or serum for syrinx propagation or development may occur.

• #22 Importance of the Mechanical Forces in the Pathogenesis – American Syringomyelia & Chiari Alliance Project – ASAP

  https://asap.org/importance-of-the-mechanical-forces-in-the-pathogenesis-of-syringomyelia/

  A simplified in vitro model of the spinal canal, based on in vivo magnetic resonance imaging (MRI), was used to examine the hydrodynamics of the human spinal cord and subarachnoid space (SAS) with syringomyelia. […] Overall, the in vitro study of the unsteady pressure and flow environment within the syrinx and SAS, provides insight into the complex biomechanical forces present in syringomyelia. […] Thus, the proposed work would examine this potential mechanism under various configurations thought to cause syringomyelia. […] We will focus on obtaining measurements that provide a more complete understanding of the role of hydrodynamic forces in syringomyelia pathogenesis.

• #23 The pathogenesis of syringomyelia : a re-evaluation of the elastic-jump hypothesis – WRAP: Warwick Research Archive Portal

  https://wrap.warwick.ac.uk/id/eprint/28335/

  Syringomyelia is a disease in which fluid-filled cavities, called syrinxes, form in the spinal cord causing progressive loss of sensory and motor functions. […] However, only recently have models begun to appear based on the principles of mechanics. […] They suggested that a pressure wave due to a cough or sneeze could form a shocklike elastic jump, which when incident at a stenosis, such as a hindbrain tonsil, would generate a transient region of high pressure within the spinal cord and lead to fluid accumulation. […] It was found that, while the spinal geometry does allow for elastic jumps to occur, their effects are likely to be weak and subsumed by the small amount of viscous damping present in the subarachnoid space. […] Furthermore, the polarity of the pressure differential set up by cough-type impulses opposes the tenets of the elastic-jump hypothesis. […] The analysis presented here does not support the elastic-jump hypothesis or any theory reliant on cough-based pressure impulses as a mechanism for the pathogenesis of syringomyelia.

• #24 Syringomyelia in the Tethered Spinal Cords

  https://www.jkns.or.kr/journal/view.php?doi=10.3340/jkns.2020.0097

  Cases of syringomyelia associated with spinal dysraphism are distinct from those associated with hindbrain herniation or arachnoiditis in terms of the suspected pathogenetic mechanism. […] Various theories on the pathogenesis of syringomyelia have been proposed, but the majority have focused on syringomyelia associated with hindbrain herniation. In terms of cases of an accompanying tethered cord, a recent group postulated that tensile radial stress on the spinal cord may cause syrinx, as the transient lower pressure of the cord parenchyma may draw in interstitial fluid, causing enlargement of the syrinx. […] Another study established a rat model of syringomyelia using kaolin material to produce epidural compression.

• #25 The Pathophysiology of Syringomyelia | SpringerLink

  https://link.springer.com/chapter/10.1007/978-3-642-56023-1_2

  We have analyzed more than 500 patients with syringomyelia who were treated within the past 20 years. Either at the craniocervical junction or in the spinal canal, an associated pathology could be found in each patient. Each associated pathology was in close anatomical relation to the syrinx cavity and caused CSF flow obstruction and/or spinal cord tethering to some degree. The best long-term results for treatment of syringomyelia were obtained with successful treatment of the associated pathology. […] On the pathogenesis of syringomyelia: a review. […] Pathological basis of spinal cord cavitation in syringomyelia: analysis of 105 autopsy cases. […] The distending force in the production of communicating syringomyelia. […] Spinal cord intramedullary pressure. A probable factor in syrinx growth.

• #26 The pathophysiology of syringomyelia – historical overview and current concept. | CoLab

  https://colab.ws/articles/10.1007%2Fs00701-002-0944-3

  Various ideas and hypotheses have been brought forward to explain the development of syringomyelia in the past two centuries. […] Syringomyelia is understood as a state of chronic interstitial edema of the spinal cord due to accumulation of extracellular fluid (ECF). This accumulation is caused by a cascade of events starting with obstruction of cerebrospinal fluid (CSF) flow and/or spinal cord tethering which ultimately alter ECF flow and increase ECF volume. […] Treatment should be targeted against the pathological process which causes CSF flow obstruction and cord tethering to inhibit this pathophysiological process at a decisive point.

• #27 A unifying hypothesis for hydrocephalus, Chiari malformation, syringomyelia, anencephaly and spina bifida | Fluids and Barriers of the CNS | Full Text

  https://fluidsbarrierscns.biomedcentral.com/articles/10.1186/1743-8454-5-7

  Chiari malformations cause obstruction to CSF flow that elevates CNS pressure and damages neural tissue by ischemic and mechanical forces. […] It is proposed that hydrocephalus is an oedema of the central nervous system. […] The mechanism for neural injury in the original theory had a requirement for hydrocephalus with CSF flow from the head into the cord parenchyma via the fourth ventricle. Hydrocephalus is not always present in the two conditions and fluid flow from the fourth ventricle into spinal cord cavities in syringomyelia is uncommon. It is proposed that the disease continuum relates to posterior fossa hypoplasia that causes reduced CNS compliance before or after birth. […] Surgery that improves CSF pathways at the foramen magnum may cause collapse of the syrinx cavity, improve cord blood flow and the clinical features of syringomyelia. This indicates that neural injury results from impaired flow of CSF at the foramen magnum. […] Syringomyelia occurs in association with posterior fossa restriction of Chiari I and other causes of reduced CNS compliance, particularly if they directly affect the spine.

• #28 Syringomyelia: What It Is, Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/6126-syringomyelia

  Acquired (developed later in life) causes of syringomyelia include spinal cord injuries, spinal cord tumors, inflammation of the arachnoid membrane (arachnoiditis), and meningitis. […] Treatment to fix the underlying cause and prevent further damage to your spinal cord usually involves some type of surgery. General therapies to manage symptoms include pain management and medications, physical therapy and rehabilitation, and limiting certain activities, especially those that cause strain on your spine. […] If you have syringomyelia that’s causing symptoms or the syrinx is getting bigger, your neurologist or neurosurgeon will likely recommend surgery. There are two general forms of surgery: restoration of normal CSF flow around your spinal cord and direct drainage of the syrinx. The type of surgical treatment depends on what’s causing the symptoms. […] Surgery for syringomyelia can often lead to an improvement of symptoms and stabilization of the condition for many people. However, syringomyelia can recur (come back) after successful treatment, which may require more procedures.

• #29 Syringomyelia – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK537110/

  The pressure within the syrinx is observed to be equal to or higher than that in the spinal subarachnoid space, contradicting the transmedullary theory. […] The natural history of CM-1 with syringomyelia is usually benign, as only a small fraction of patients treated non-surgically eventually require surgery. […] The most common symptoms of syringomyelia include radicular pain, gait ataxia, sensory disturbances, dysesthesias, motor weakness, spasticity, autonomic dysreflexia, and neuropathic pain. […] Despite undergoing posterior fossa decompression, persistent, recurrent, or worsening syringomyelia may occur in 10% to 50% of cases.

• #30 Syringomyelia > Treatment, symptoms, therapy, surgery

  https://avicenna-klinik.com/en/spine-symptoms-disorders/syringomyelia/

  A frequent cause is, for example, the Chiari malformation. […] The causes of the acquired form of syringomyelia vary. […] Syringomyelia can also develop after treatment by spinal cord surgery. […] In order to prevent the progression of the deficits, it is necessary to enlarge the space for the spinal cord in an operation (surgery) or to drain the fluid that is in the cavity from there. […] By reducing the pressure, we relieve the spinal cord and in this way relieve the pain. […] Syringomyelia cannot be prevented. […] However, the progression of the disease can be stopped or slowed down and severe accompanying symptoms, pain and disorders can be alleviated.

• #31 Syringomyelia | PM&R KnowledgeNow

  https://now.aapmr.org/syringomyelia/

  There is limited research on the pathophysiology of syringomyelia unrelated to Chiari I malformation. […] Exploration of the factors and their role in the development and progression of syringomyelia is researched in the following areas: Role of genetic factors related to a Chiari I malformation, Learning the role of birth defects in the development of hindbrain malformations, Abnormal CSF flow that may contribute to the progression, Developing preventive treatment to stop the formation of birth defects, Role for the cardiovascular system, as evidenced by forceful downward movement of the CSF with heartbeat, Improvement in surgical techniques. […] The exact mechanism of syrinx development remains unknown and no prevention or cure exists.

• #32 Familial syringomyelia: Incidental or hereditary? | 2025, Volume 31 – Issue 1 | Turkish Journal of Neurology

  https://tjn.org.tr/full-text/2148/eng

  Syringomyelia is a rare disease of the spinal cord, and its familial occurrence is even rarer. […] Both genetic and environmental factors appear to be involved in familial syringomyelia. […] The presence of another affected individual in the family history suggests that genetic predisposition plays a more important role in the pathogenesis of this condition. […] Familial aggregation of SM suggests a genetic basis. […] The high coincidence of SM with CM1 and other skeletal abnormalities, particularly at the skull base, as well as the familial aggregation of the two, implies that there could be a genetic culprit. […] Avar et al. mention about the significant linkage between chromosome 8, 9, 11, 12 and 15 and CM1 with or without SM detected in some contemporary studies. […] Regarding the high coincidence of SM with CM1 malformation, both might have a shared genetic etiology, and these genes may also have a role in syrinx formation.

• #33 Syringomyelia: Current Concepts in Pathogenesis and Management | SpringerLink

  https://link.springer.com/book/10.1007/978-4-431-67893-9

  Surgical skill and imaging technology in the field of neurosurgery have developed remarkably during the past decade. […] Recent advances in dynamic MRI techniques have made it possible to evaluate normal and abnormal cerebrospinal fluid (CSF) flow at the foramen magnum, within the syrinx, and in the spinal subarachnoid space, without invasion of the closed CSF system. […] However, many issues related to the pathophysiology and treatment of syringomyelia remain controversial. […] This volume thus represents a comprehensive description of the state of the art regarding this disease in the hope that it may help to define comprehensively what is already known and to find new ways toward a better understanding of the pathophysiology, diagnosis, and treatment of syringomyelia. […] Some Further Thoughts on the Etiopathology of Syringomyelia and Experience with Surgical Management.

• #34 Engineering models to explain the origins of syringomyelia – Dr. Serge Cirovic | Mechanical and Aerospace Engineering

  https://mae.ncsu.edu/event/engineering-models-explain-origins-syringomyelia-dr-serge-cirovic/

  Syringomyelia is a severe progressive pathological condition in which fluid-filled cavities form and grow in the spinal cord at the expense of the normal nervous tissue. […] There is strong evidence that syringomyelia is linked to obstructions to the movement of cerebrospinal fluid (CSF), a clear water-like fluid that bathes the brain and the spinal cord. […] However, the exact mechanism of cyst formation and growth has defied explanation for decades. […] The lack of understanding of the origins of the condition limits the success of currently available surgical treatments. […] With the lack of clinical explanation for the aetiology of this condition, engineers and mathematicians resorted to computer models in order to identify possible physical mechanisms that can lead to this pathology. […] While engineering models have helped in dismissing old theories and generating new ones on the origin of syringomyelia, no clear answers have emerged as yet and efforts to understand this enigmatic condition continue.

• #35 Syringomyelia Treatment and Causes| Institut Chiari de Barcelona

  https://institutchiaribcn.com/en/diseases-we-treat/syringomyelia/

  Idiopathic Syringomyelia is defined by the presence of an intramedullary cyst filled with clear fluid, within the spinal cord. […] The cause of its formation is unknown. […] Secondary Syringomyelia is an intramedullary cyst filled with interstitial fluid or serum, the cause of which is tumorous, traumatic, infectious or other. Its cause could produce a necrosis of the spinal cord parenchyma, exerting an invasive effect by compression, traction or a combination of the 3 mechanisms. […] Dr. Miguel B. Royo-Salvador’s theory considers the syringomyelic cavitation as the result of nervous tissue necrosis, caused by the ischemia-oedema produced by the traction from an overly tense film terminale (not detectable on imaging). […] Dr. Royo-Salvador proved the theory that considers several diseases of unknown cause (Arnold-Chiari Syndrome Type I, idiopathic Syringomyelia, idiopathic Scoliosis, platybasia, basilar invagination, odontoid retroflexion, brain stem kinking, and others) as part of a new disease concept (the Filum Disease) and that they share a common cause (spinal cord and entire nervous system traction).

• #36 Pathophysiology of primary spinal syringomyelia

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3787878/

  After a syrinx is formed, the enlarged waves compress the external surface of the spinal cord, propel the syrinx fluid, and promote syrinx progression. […] The mechanism of PSS was evaluated by clinical, radiographic, and physiological tests performed before, during, and after surgery. Magnetic resonance imaging, CT myelography, and intraoperative ultrasonography identified obstruction of the subarachnoid space in all patients. […] All of these findings are consistent with a mechanism of syringomyelia that is extramedullary, not intramedullary. […] The goal of this study was to establish the mechanism of progression of PSS. We hypothesized that PSS results from obstruction of CSF flow in the SAS; this obstruction affects spinal CSF dynamics because the SAS accepts the CSF that is rapidly displaced from the intracranial subarachnoid space as the brain expands during cardiac systole. In PSS, a subarachnoid block effectively shortens the length of the SAS, reducing CSF compliance and the capacity of the spinal theca to dampen the subarachnoid CSF pressure waves produced by brain expansion during cardiac systole. As a result, exaggerated spinal subarachnoid pressure waves occur with every heartbeat and act on the spinal cord above the block to drive CSF into the spinal cord and create a syrinx.

• #37

  https://journals.lww.com/neurosurgery/fulltext/2017/09001/pathogenesis_of_chiari_i___pathophysiology_of.16.aspx

  The findings are consistent with our proposal that a spinal subarachnoid block increases spinal subarachnoid pulse pressure above the block of the spinal SAS, producing a pressure differential across the obstructed segment of the SAS, and underlies syrinx formation and progression, and are similar to the results of our previous studies that examined the pathophysiology of syringomyelia associated with the Chiari I malformation. […] The results of all our studies were consistent with our original hypothesis. We should keep in mind that patients with increased intracranial pressure, increased intracranial pressure waves, and reduced CSF compliance from tumors, hydrocephalous, etc do not have syringomyelia unless there is tonsil herniation. The development of a syrinx requires increased absolute pressure and pulse pressure in the spinal CSF and at least a partial obstruction of the SAS with a partially isolated spinal CSF compartment.

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