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Journal of Neurology, Neurosurgery, and... Jan 1996To assess the incidence of post-traumatic syringomyelia (PTS), to correlate the presence of PTS with its most common signs and symptoms, and to compare results from the... (Review)
Review
OBJECTIVES
To assess the incidence of post-traumatic syringomyelia (PTS), to correlate the presence of PTS with its most common signs and symptoms, and to compare results from the Swiss Paraplegic Centre with those reported in the medical literature.
METHODS
A total of 449 recent traumatic paraplegic and tetraplegic patients admitted to the Swiss Paraplegic Centre in Zurich between 1 January 1987 and 31 December 1993 were prospectively analysed. Yearly clinical tests with conventional radiographs and additional T1 and T2 weighted images were performed as soon as PTS was diagnosed.
RESULTS
Of these 449 patients 20 patients displayed symptoms of PTS (4.45%). Ten non-operated patients remained clinically stable (average time: 37 months). Ten worsened--three refused operation, seven were operated on. Mean worsening time was 97 months. Deterioration was closely related to the enlargement of the cyst whereas in operated patients neurological improvement or stabilisation correlated with collapse of the cyst.
CONCLUSIONS
Delay between appearance of the first symptoms of PTS and deterioration making surgery necessary may be long (mean five years in the seven operated patients) underlining the need for regular tests. "Slosh" and "suck" mechanisms could explain cyst enlargement as surgical realignment of the spine resulted in a complete cyst collapse in two of the operated patients (normalisation of CSF flow? ). Cord compression, tense syrinx at the fracture site, and kyphosis seemed to be closely linked to the enlargement of the cyst with subsequent further neurological deterioration.
Topics: Adolescent; Adult; Aged; Child; Female; Humans; Incidence; Kyphosis; Male; Middle Aged; Paraplegia; Prospective Studies; Quadriplegia; Radiography; Spinal Cord Injuries; Syringomyelia; Treatment Outcome
PubMed: 8558154
DOI: 10.1136/jnnp.60.1.61 -
Journal of Veterinary Internal Medicine Jul 2021The Chihuahua dog breed is known for frequent occurrence of a bregmatic fontanelle on the dorsal skull. A common conception is that this skull defect is a clinically...
BACKGROUND
The Chihuahua dog breed is known for frequent occurrence of a bregmatic fontanelle on the dorsal skull. A common conception is that this skull defect is a clinically irrelevant finding. No studies, however, describe its prevalence or whether it is accompanied by other persistent fontanelles (PFs). Although Chihuahuas are predisposed to Chiari-like malformation (CM) and syringomyelia (SM), it is unknown whether PFs occur more commonly in dogs with clinical signs that are caused by CM or SM.
HYPOTHESIS/OBJECTIVES
To describe the number and location of PFs at cranial sutures (CSs) and to compare the occurrence of these PFs in dogs with and without CM/SM-related clinical signs. We hypothesized that PFs also occur commonly at lateral and caudal cranial surfaces, affect a higher number of CSs, and are larger in dogs with CM/SM-related clinical signs.
ANIMALS
Fifty client-owned Chihuahuas with or without CM/SM-related clinical signs.
RESULTS
Of the 50 dogs evaluated, 46 (92%) had either 1 or several PFs. The mean ± SD number of PFs was 2.8 ± 3.0 (range, 0-13). A total of 138 PFs occupied 118 CSs with 57 (48%) located dorsally, 44 (37%) caudally, and 17 (14%) laterally. The number of CSs affected by PFs was significantly higher (P ≤ .001) and total PF area was significantly larger (P = .003) in dogs with CM/SM-related clinical signs.
CONCLUSIONS AND CLINICAL IMPORTANCE
Persistent fontanelles are very common in this group of Chihuahuas and appear at dorsal, lateral, and caudal cranial surfaces. They are more numerous and larger in Chihuahuas with CM/SM-related clinical signs.
Topics: Animals; Arnold-Chiari Malformation; Dog Diseases; Dogs; Magnetic Resonance Imaging; Skull; Syringomyelia
PubMed: 34028887
DOI: 10.1111/jvim.16151 -
Journal of the Chinese Medical... Jan 2005We present the case of a 20-year-old male with intermittent right upper extremity numbness for 3 months. His pain perception and temperature sensation were severely... (Review)
Review
We present the case of a 20-year-old male with intermittent right upper extremity numbness for 3 months. His pain perception and temperature sensation were severely disturbed. An incidental magnetic resonance imaging (MRI) finding of one small intramedullary enhancing nodule at spinal cord level T10-11 with long-segment syrinx formation suggested the diagnosis of spinal hemangioblastoma with syringomyelia. Surgical removal of the tumor and decompression of the spinal cord with opening of the syrinx were performed smoothly, and the pathology confirmed the diagnosis of spinal hemangioblastoma. Reviewing the literature, MRI is the examination of choice for spinal hemangioblastomas, and is helpful in preoperative planning and the differential diagnosis of spinal cord neoplasms and vascular lesions.
Topics: Adult; Hemangioblastoma; Humans; Magnetic Resonance Imaging; Male; Spinal Cord; Spinal Cord Neoplasms; Syringomyelia; Treatment Outcome
PubMed: 15742863
DOI: 10.1016/S1726-4901(09)70131-5 -
Journal of Neurosurgery. Spine Nov 2012The pathogenesis of syringomyelia in patients with an associated spinal lesion is incompletely understood. The authors hypothesized that in primary spinal syringomyelia,...
OBJECT
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. After a syrinx is formed, enlarged subarachnoid pressure waves compress the external surface of the spinal cord, propel the syrinx fluid, and promote syrinx progression.
METHODS
To elucidate the pathophysiology, the authors prospectively studied 36 adult patients with spinal lesions obstructing the spinal SAS. Testing before surgery included clinical examination; evaluation of anatomy on T1-weighted MRI; measurement of lumbar and cervical subarachnoid mean and pulse pressures at rest, during Valsalva maneuver, during jugular compression, and after removal of CSF (CSF compliance measurement); and evaluation with CT myelography. During surgery, pressure measurements from the SAS above the level of the lesion and the lumbar intrathecal space below the lesion were obtained, and cardiac-gated ultrasonography was performed. One week after surgery, CT myelography was repeated. Three months after surgery, clinical examination, T1-weighted MRI, and CSF pressure recordings (cervical and lumbar) were repeated. Clinical examination and MRI studies were repeated annually thereafter. Findings in patients were compared with those obtained in a group of 18 healthy individuals who had already undergone T1-weighted MRI, cine MRI, and cervical and lumbar subarachnoid pressure testing.
RESULTS
In syringomyelia patients compared with healthy volunteers, cervical subarachnoid pulse pressure was increased (2.7 ± 1.2 vs 1.6 ± 0.6 mm Hg, respectively; p = 0.004), pressure transmission to the thecal sac below the block was reduced, and spinal CSF compliance was decreased. Intraoperative ultrasonography confirmed that pulse pressure waves compressed the outer surface of the spinal cord superior to regions of obstruction of the subarachnoid space.
CONCLUSIONS
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. These findings are similar to the results of the authors' previous studies that examined the pathophysiology of syringomyelia associated with obstruction of the SAS at the foramen magnum in the Chiari Type I malformation and indicate that a common mechanism, rather than different, separate mechanisms, underlies syrinx formation in these two entities. Clinical trial registration no.: NCT00011245.
Topics: Adult; Arnold-Chiari Malformation; Cerebrospinal Fluid Pressure; Diagnostic Imaging; Disease Progression; Female; Humans; Male; Middle Aged; Prospective Studies; Subarachnoid Space; Syringomyelia
PubMed: 22958075
DOI: 10.3171/2012.8.SPINE111059 -
Neurologia Medico-chirurgica Sep 1998The clinical and neuroimaging characteristics of 22 consecutive patients with Chiari type I malformations were evaluated to investigate the pathogenesis of syrinx...
The clinical and neuroimaging characteristics of 22 consecutive patients with Chiari type I malformations were evaluated to investigate the pathogenesis of syrinx formation. All patients underwent magnetic resonance imaging and x-ray tomography before surgery. The electric manometric Queckenstedt test was performed on 16 patients pre- and postoperatively. Syringomyelia was present in 17 patients and absent in five patients. All patients without syringomyelia suffered from foramen magnum compression syndrome, with a wider basal angle, more acute clivo-axial angle, shorter clivus, and more prominent tonsillar ectopia than patients with syringomyelia. Low brain stem position, basilar impression, and beaking of the cervicomedullary junction were also more prominent in patients without syringomyelia. Marked to complete block with the neck in flexed position by Queckenstedt test was present in all patients except one. Patients with Chiari malformation not associated with syringomyelia have more pronounced compression of the brain stem at the foramen magnum. Therefore, despite a block of the cerebrospinal fluid pathway at the foramen magnum, syrinx formation may be prevented by severe compression.
Topics: Adolescent; Adult; Aged; Arnold-Chiari Malformation; Brain; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Spinal Cord; Syringomyelia; Tomography, X-Ray Computed
PubMed: 9805898
DOI: 10.2176/nmc.38.541 -
PloS One 2017To characterize and compare the phenotypic variables of the hindbrain and craniocervical junction associated with syringomyelia (SM) in the Chihuahua, Affenpinscher and...
Craniometric Analysis of the Hindbrain and Craniocervical Junction of Chihuahua, Affenpinscher and Cavalier King Charles Spaniel Dogs With and Without Syringomyelia Secondary to Chiari-Like Malformation.
OBJECTIVES
To characterize and compare the phenotypic variables of the hindbrain and craniocervical junction associated with syringomyelia (SM) in the Chihuahua, Affenpinscher and Cavalier King Charles Spaniel (CKCS).
METHOD
Analysis of 273 T1-weighted mid-sagittal DICOM sequences of the hindbrain and craniocervical junction from 99 Chihuahuas, 42 Affenpinschers and 132 CKCSs. The study compared 22 morphometric features (11 lines, eight angles and three ratios) of dogs with and without SM using refined techniques based on previous studies of the Griffon Bruxellois (GB) using Discriminant Function Analysis and ANOVA with post-hoc corrections.
RESULTS
The analysis identified 14/22 significant traits for SM in the three dog breeds, five of which were identical to those reported for the GB and suggest inclusion of a common aetiology. One ratio, caudal fossa height to the length of the skull base extended to an imaginary point of alignment between the atlas and supraoccipital bones, was common to all three breeds (p values 0.029 to <0.001). Associated with SM were a reduced occipital crest and two acute changes in angulation i) 'sphenoid flexure' at the spheno-occipital synchondrosis ii) 'cervical flexure' at the foramen magnum allied with medulla oblongata elevation. Comparing dogs with and without SM, each breed had a unique trait: Chihuahua had a smaller angle between the dens, atlas and basioccipital bone (p value < 0.001); Affenpinschers had a smaller distance from atlas to dens (p value 0.009); CKCS had a shorter distance between the spheno-occipital synchondrosis and atlas (p value 0.007).
CONCLUSION
The selected morphometries successfully characterised conformational changes in the brain and craniocervical junction that might form the basis of a diagnostic tool for all breeds. The severity of SM involved a spectrum of abnormalities, incurred by changes in both angulation and size that could alter neural parenchyma compliance and/or impede cerebrospinal fluid channels.
Topics: Animals; Arnold-Chiari Malformation; Breeding; Cephalometry; Cervical Vertebrae; Craniosynostoses; Discriminant Analysis; Dog Diseases; Dogs; Female; Foramen Magnum; Genetic Predisposition to Disease; Magnetic Resonance Imaging; Male; Phenotype; Retrospective Studies; Rhombencephalon; Skull; Skull Base; Syringomyelia
PubMed: 28121988
DOI: 10.1371/journal.pone.0169898 -
Journal of Veterinary Internal Medicine Sep 2019Diagnosis of Chiari-like malformation-associated pain (CM-P) or clinically relevant syringomyelia (SM) is challenging. We sought to determine common signs.
BACKGROUND
Diagnosis of Chiari-like malformation-associated pain (CM-P) or clinically relevant syringomyelia (SM) is challenging. We sought to determine common signs.
ANIMALS
One hundred thirty client-owned Cavalier King Charles spaniels with neuroaxis magnetic resonance imaging (MRI) and diagnosis of CM-P/SM. Dogs with comorbidities causing similar signs were excluded with exception of otitis media with effusion (OME).
METHODS
Retrospective study of medical records relating signalment, signs, and MRI findings. Dogs were grouped by SM maximum transverse diameter (1 = no SM; 2 = 0.5-1.99 mm; 3 = 2-3.9 mm: 4 = ≥4 mm). Differences between all groups-groups 1 versus 2-4 and groups 1-3 versus 4-were investigated. Continuous variables were analyzed using 2-sample t-tests and analysis of variance. Associations between categorical variables were analyzed using Fisher's exact or chi-square tests.
RESULTS
Common signs were vocalization (65.4%), spinal pain (54.6%), reduced activity (37.7%), reduced stairs/jumping ability (35.4%), touch aversion (30.0%), altered emotional state (28.5%), and sleep disturbance (22%). Head scratching/rubbing (28.5%) was inversely associated with syrinx size (P = .005), less common in group 4 (P = .003), and not associated with OME (P = .977). Phantom scratching, scoliosis, weakness, and postural deficits were only seen in group 4 (SM ≥4 mm; P = .004).
CONCLUSIONS AND CLINICAL IMPORTANCE
Signs of pain are common in CM/SM but are not SM-dependent, suggesting (not proving) CM-P causality. Wide (≥4 mm) SM is associated with signs of myelopathy and, if the dorsal horn is involved, phantom scratching (ipsilateral) and torticollis (shoulder deviated ipsilateral; head tilt contralateral).
Topics: Animals; Arnold-Chiari Malformation; Behavior, Animal; Dog Diseases; Dogs; Female; Magnetic Resonance Imaging; Male; Otitis Media with Effusion; Pain; Retrospective Studies; Syringomyelia
PubMed: 31290195
DOI: 10.1111/jvim.15552 -
Fluids and Barriers of the CNS Feb 2024Syringomyelia (SM) is characterized by the development of fluid-filled cavities, referred to as syrinxes, within the spinal cord tissue. The molecular etiology of SM...
BACKGROUND
Syringomyelia (SM) is characterized by the development of fluid-filled cavities, referred to as syrinxes, within the spinal cord tissue. The molecular etiology of SM post-spinal cord injury (SCI) is not well understood and only invasive surgical based treatments are available to treat SM clinically. This study builds upon our previous omics studies and in vitro cellular investigations to further understand local fluid osmoregulation in post-traumatic SM (PTSM) to highlight important pathways for future molecular interventions.
METHODS
A rat PTSM model consisting of a laminectomy at the C7 to T1 level followed by a parenchymal injection of 2 μL quisqualic acid (QA) and an injection of 5 μL kaolin in the subarachnoid space was utilized 6 weeks after initial surgery, parenchymal fluid and cerebrospinal fluid (CSF) were collected, and the osmolality of fluids were analyzed. Immunohistochemistry (IHC), metabolomics analysis using LC-MS, and mass spectrometry-based imaging (MSI) were performed on injured and laminectomy-only control spinal cords.
RESULTS
We demonstrated that the osmolality of the local parenchymal fluid encompassing syrinxes was higher compared to control spinal cords after laminectomy, indicating a local osmotic imbalance due to SM injury. Moreover, we also found that parenchymal fluid is more hypertonic than CSF, indicating establishment of a local osmotic gradient in the PTSM injured spinal cord (syrinx site) forcing fluid into the spinal cord parenchyma to form and/or expand syrinxes. IHC results demonstrated upregulation of betaine, ions, water channels/transporters, and enzymes (BGT1, AQP1, AQP4, CHDH) at the syrinx site as compared to caudal and rostral sites to the injury, implying extensive local osmoregulation activities at the syrinx site. Further, metabolomics analysis corroborated alterations in osmolality at the syrinx site by upregulation of small molecule osmolytes including betaine, carnitine, glycerophosphocholine, arginine, creatine, guanidinoacetate, and spermidine.
CONCLUSIONS
In summary, PTSM results in local osmotic disturbance that propagates at 6 weeks following initial injury. This coincides with and may contribute to syrinx formation/expansion.
Topics: Rats; Animals; Syringomyelia; Osmoregulation; Betaine; Rats, Sprague-Dawley; Spinal Cord Injuries; Magnetic Resonance Imaging
PubMed: 38409031
DOI: 10.1186/s12987-024-00514-y -
PloS One 2014This study aimed to develop a system of quantitative analysis of canine Chiari-like malformation and syringomyelia on variable quality MRI. We made a series of...
This study aimed to develop a system of quantitative analysis of canine Chiari-like malformation and syringomyelia on variable quality MRI. We made a series of measurements from magnetic resonance DICOM images from Griffon Bruxellois dogs with and without Chiari-like malformation and syringomyelia and identified several significant variables. We found that in the Griffon Bruxellois dog, Chiari-like malformation is characterized by an apparent shortening of the entire cranial base and possibly by increased proximity of the atlas to the occiput. As a compensatory change, there appears to be an increased height of the rostral cranial cavity with lengthening of the dorsal cranial vault and considerable reorganization of the brain parenchyma including ventral deviation of the olfactory bulbs and rostral invagination of the cerebellum under the occipital lobes.
Topics: Animals; Arnold-Chiari Malformation; Brain; Dogs; Female; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Male; Predictive Value of Tests; Prevalence; Reproducibility of Results; Sensitivity and Specificity; Syringomyelia
PubMed: 24533070
DOI: 10.1371/journal.pone.0088120 -
Revista de Neurologia Jun 2013Chiari malformations (CM) include a series of congenital anomalies involving the descent of the cerebellar tonsils below the foramen magnum, which may be associated with... (Review)
Review
INTRODUCTION
Chiari malformations (CM) include a series of congenital anomalies involving the descent of the cerebellar tonsils below the foramen magnum, which may be associated with compression in the brainstem, upper spinal cord, and cranial nerves, consequently altering the responses of brainstem auditory evoked potentials (BAEP) and somatosensory evoked potentials (SSEP). However, only a small number of authors have described the indications of these tests in CM, and study groups to date have been small and heterogeneous.
AIM
To review the results of BAEPs and SSEPs in published studies of patients with Chiari type 1 malformation (CM-1) or Chiari type 2 malformation (CM-2) as well as the indications of both tests in the diagnosis, treatment, and monitoring of both diseases, especially CM-1.
DEVELOPMENT
We present a review article analyzing data from all published studies in Medline starting in 1966, located through PubMed, using combinations of the following keywords: 'Chiari malformation', 'Arnold-Chiari malformation', 'Chiari type 1 malformation', 'Arnold-Chiari type 1 malformation', 'evoked potentials', 'brainstem auditory evoked potentials' and 'somatosensory evoked potentials' as well as records of patients with CM-1 from the neurosurgery and neurophysiology departments at the Hospital Universitari Vall d'Hebron.
CONCLUSIONS
Common findings of SSEP are a reduction in cortical amplitude from the posterior tibial nerve, a reduction or absence of cervical median nerve potential, and an increased N13-N20 interval. In BAEP, the most frequent findings are an increased I-V interval and a peripheral or cochlear auditory disturbance.
Topics: Adolescent; Adult; Age of Onset; Arnold-Chiari Malformation; Child; Child, Preschool; Cochlear Nerve; Decompression, Surgical; Encephalocele; Evoked Potentials, Auditory, Brain Stem; Evoked Potentials, Somatosensory; Humans; Magnetic Resonance Imaging; Median Nerve; Monitoring, Intraoperative; Reaction Time; Syringomyelia; Tibial Nerve; Young Adult
PubMed: 23744249
DOI: No ID Found