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European Neurology 2005
Topics: History, 16th Century; History, 18th Century; History, 19th Century; History, Ancient; Humans; Syringes; Syringomyelia; Terminology as Topic
PubMed: 16401904
DOI: 10.1159/000090723 -
Scientific Reports Jul 2023Posttraumatic spinal cord tethering and syringomyelia frequently lead to progressive neurological loss. Although several studies demonstrated favourable outcome...
Posttraumatic spinal cord tethering and syringomyelia frequently lead to progressive neurological loss. Although several studies demonstrated favourable outcome following spinal cord detethering with/without shunting, additional research is required as no clear consensus exists over the ideal treatment strategy and knowledge about prognostic demographic determinants is currently limited. In this investigation, we retrospectively investigated 67 patients (56 men, 11 women) who were surgically treated and followed for symptomatic spinal cord tethering and syringomyelia from 2012 to 2022 at our center. Age (B-coefficient 0.396) and severity of trauma to the spinal cord (B-coefficient - 0.462) have been identified as independent predictors for the rate of development of symptomatic spinal cord tethering and syringomyelia (p < 0.001). Following untethering surgery including expansion duraplasty with/without shunting, 65.9% of patients demonstrated an improvement of neurological loss (p < 0.001) whereas 50.0% of patients displayed amelioration of spasticity and/or neuropathic pain (p < 0.001). Conclusively, active screening for symptomatic spinal cord tethering and syringomyelia, particularly in younger patients with severe spinal trauma, is crucial as surgical untethering with/without shunting is able to achieve favourable clinical outcomes. This knowledge may enable clinicians to tailor treatment strategies in spinal cord injury patients suffering from progressive neurological loss towards a more optimal and personalized patient care.
Topics: Male; Humans; Female; Syringomyelia; Retrospective Studies; Spinal Cord; Spinal Cord Injuries; Treatment Outcome
PubMed: 37454226
DOI: 10.1038/s41598-023-38565-0 -
Journal of Veterinary Internal Medicine Nov 2017Chiari-like malformation (CM) and syringomyelia (SM) are widely reported in Cavalier King Charles Spaniels and Griffon Bruxellois dogs. Increasing evidence indicates...
BACKGROUND
Chiari-like malformation (CM) and syringomyelia (SM) are widely reported in Cavalier King Charles Spaniels and Griffon Bruxellois dogs. Increasing evidence indicates that CM and SM also occur in other small and toy breed dogs, such as Chihuahuas.
OBJECTIVES
To describe the presence of SM and craniocervical junction (CCJ) abnormalities in Chihuahuas and to evaluate the possible association of CCJ abnormalities with SM. To describe CM/SM-related clinical signs and neurologic deficits and to investigate the association of CM/SM-related clinical signs with signalment, SM, or CCJ abnormalities.
ANIMALS
Fifty-three client-owned Chihuahuas.
METHODS
Prospective study. Questionnaire analyses and physical and neurologic examinations were obtained before magnetic resonance and computed tomography imaging. Images were evaluated for the presence of SM, CM, and atlantooccipital overlapping. Additionally, medullary kinking, dorsal spinal cord compression, and their sum indices were calculated.
RESULTS
Scratching was the most common CM/SM-related clinical sign and decreased postural reaction the most common neurologic deficit in 73 and 87% of dogs, respectively. Chiari-like malformation and SM were present in 100 and 38% of dogs, respectively. Syringomyelia was associated with the presence of CM/SM-related clinical signs (P = 0.034), and medullary kinking and sum indices were higher in dogs with clinical signs (P = 0.016 and P = 0.007, respectively).
CONCLUSIONS AND CLINICAL IMPORTANCE
Syringomyelia and CCJ abnormalities are prevalent in Chihuahuas. Syringomyelia was an important factor for the presence of CM/SM-related clinical signs, but many dogs suffered from similar clinical signs without being affected by SM, highlighting the clinical importance of CCJ abnormalities in Chihuahuas.
Topics: Animals; Arnold-Chiari Malformation; Atlanto-Occipital Joint; Dog Diseases; Dogs; Female; Magnetic Resonance Imaging; Male; Medulla Oblongata; Prospective Studies; Species Specificity; Spinal Cord Compression; Syringomyelia; Tomography, X-Ray Computed
PubMed: 28892202
DOI: 10.1111/jvim.14826 -
BMC Veterinary Research Nov 2023Syringomyelia is a spinal cord cavity containing cerebrospinal fluid (CSF)-like fluid. If syringomyelia asymmetrically involves the dorsal horn grey matter of the spinal...
BACKGROUND
Syringomyelia is a spinal cord cavity containing cerebrospinal fluid (CSF)-like fluid. If syringomyelia asymmetrically involves the dorsal horn grey matter of the spinal cord, affected dogs show increased signs of dysesthesia and neuropathic pain, like increased itching behaviour. In the dorsal horn, amongst others, receptors for Interleukin-31 (IL-31) can be found. IL-31 is one of the main cytokines involved in the pathogenesis of pruritus in atopic dermatitis in different species. This study investigates suspected elevated levels of IL-31 in serum and CSF of dogs showing signs of pain or increased itching behaviour related to syringomyelia. The IL-31 were measured in archived samples (52 serum and 35 CSF samples) of dogs with syringomyelia (n = 48), atopic dermatitis (n = 3) and of healthy control dogs (n = 11) using a competitive canine IL-31 ELISA.
RESULTS
Mean serum IL-31 level in dogs with syringomyelia was 150.1 pg/ml (n = 39), in dogs with atopic dermatitis 228.3 pg/ml (n = 3) and in healthy dogs 80.7 pg/ml (n = 10). Mean CSF IL-31 value was 146.3 pg/ml (n = 27) in dogs with syringomyelia and 186.2 pg/ml (n = 8) in healthy dogs. Individual patients with syringomyelia (especially dogs with otitis media or otitis media and interna or intervertebral disc herniation) showed high IL-31 levels in serum and CSF samples, but the difference was not statistically significant. IL-31 serum and CSF levels did not differ significantly in dogs with syringomyelia with or without itching behaviour and with or without signs of pain.
CONCLUSION
Based on this study, increased IL-31 levels seem not to be correlated with itching behaviour or signs of pain in dogs with syringomyelia, but might be caused by other underlying diseases.
Topics: Dogs; Animals; Syringomyelia; Dermatitis, Atopic; Interleukins; Neuralgia; Spinal Cord Dorsal Horn; Pruritus; Otitis Media; Dog Diseases; Cerebrospinal Fluid
PubMed: 37993920
DOI: 10.1186/s12917-023-03817-8 -
Journal of Neurosurgery. Spine Aug 2014
Topics: Female; Humans; Male; Subarachnoid Space; Syringomyelia
PubMed: 25221803
DOI: No ID Found -
Spinal Cord Series and Cases Mar 2020Post-traumatic syringomyelia is a complication of traumatic spinal cord injury consisting in the development of a cavity within the spinal cord. Once considered an...
INTRODUCTION
Post-traumatic syringomyelia is a complication of traumatic spinal cord injury consisting in the development of a cavity within the spinal cord. Once considered an uncommon complication, its diagnosis has increased due to increased attention and advances in medical technology. Common symptoms of the syrinx are a sensory loss of the dissociated type with pain and temperature loss and the preservation of fine touch and vibratory sensation. Eventually, a deterioration of motor function with muscle wasting may occur.
CASE PRESENTATION
We present the case of a 36-year-old woman who sustained a sport accident in 1996, resulting in AIS A, T7 paraplegia. She underwent a magnetic resonance imaging (MRI) examination because of neck and left shoulder pain that resolved after a short anti-inflammatory treatment. The MRI showed a large cavity involving the cord beneath T6 and the medulla. Septations were present at both the spinal cord and medulla levels. With regard to vertebral status, the MRI showed the presence of severe kyphosis at the fracture level together with spinal cord compression. The neurological examination was normal except for the pre-existing paraplegia and of a slight heat and pain sensation deficit in the C8 dermatome.
DISCUSSION
We discuss the need of regular follow-up examinations as even large syrines with involvement of the brainstem may be asymptomatic. We also discuss the possible pathogenetic factors including the type of treatment of the vertebral lesion.
Topics: Accidental Falls; Adult; Cervical Vertebrae; Female; Humans; Spinal Cord Injuries; Syringomyelia; Thoracic Vertebrae
PubMed: 32170091
DOI: 10.1038/s41394-020-0264-y -
Neurosurgery Clinics of North America Oct 2015Despite the frequency of Chiari-associated spinal deformities, this disease process remains poorly understood. Syringomyelia is often present; however, this is not... (Review)
Review
Despite the frequency of Chiari-associated spinal deformities, this disease process remains poorly understood. Syringomyelia is often present; however, this is not necessary and scoliosis has been described in the absence of a syrinx. Decompression of the hindbrain is often recommended. In young patients (<10 years old) and/or those with small coronal Cobb measurements (<40°), decompression of the hindbrain may lead to resolution of the spinal deformity. Spinal fusion is reserved for those curves that progress to deformities greater than 50°. Further research is needed to understand the underlying pathophysiology to improve prognostication and treatment of this patient population.
Topics: Arnold-Chiari Malformation; Decompression, Surgical; Humans; Magnetic Resonance Imaging; Scoliosis; Spinal Fusion; Syringomyelia
PubMed: 26408068
DOI: 10.1016/j.nec.2015.06.005 -
Neurosurgical Review Jun 2023Spinal arachnoid web (SAW) is a rare disease entity characterized as band-like arachnoid tissue that can cause spinal cord compression and syringomyelia. This study...
Spinal arachnoid web (SAW) is a rare disease entity characterized as band-like arachnoid tissue that can cause spinal cord compression and syringomyelia. This study aimed to analyze the surgical management of the spinal arachnoid web in patients with syringomyelia, focusing on surgical strategies and outcomes. A total of 135 patients with syringomyelia underwent surgery at our department between November 2003 and December 2022. All patients underwent magnetic resonance imaging (MRI), with a special syringomyelia protocol (including TrueFISP and CINE), and electrophysiology. Among these patients, we searched for patients with SAW with syringomyelia following careful analysis of neuroradiological data and surgical reports. The criteria for SAW were as follows: displacement of the spinal cord, disturbed but preserved CSF flow, and intraoperative arachnoid web. Patients were evaluated for initial symptoms, surgical strategies, and complications by reviewing surgical reports, patient documents, neuroradiological data, and follow-up data. Of the 135 patients, 3 (2.22%) fulfilled the SAW criteria. The mean patient age was 51.67 ± 8.33 years. Two patients were male, and one was female. The affected levels were T2/3, T6, and T8. Excision of the arachnoid web was performed in all cases. No significant change in intraoperative monitoring was noted. Postoperatively, none of the patients presented new neurological symptoms. The MRI 3 months after surgery revealed that the syringomyelia improved in all cases, and caliber variation of the spinal cord could not be detected anymore. All clinical symptoms improved. In summary, SAW can be safely treated by surgery. Even though syringomyelia usually improves on MRI and symptoms also improve, residual symptoms might be observed. We advocate for clear criteria for the diagnosis of SAW and a standardized diagnostic (MRI including TrueFISP and CINE).
Topics: Humans; Male; Female; Adult; Middle Aged; Syringomyelia; Spinal Cord Compression; Magnetic Resonance Imaging; Arachnoid Cysts
PubMed: 37358703
DOI: 10.1007/s10143-023-02071-8 -
Annali Dell'Istituto Superiore Di Sanita 2020Syringomyelia and Chiari Syndrome are classified as rare diseases, but current known occurrence in Europe is missing. The increased ability to diagnose these pathologies...
BACKGROUND
Syringomyelia and Chiari Syndrome are classified as rare diseases, but current known occurrence in Europe is missing. The increased ability to diagnose these pathologies by magnetic resonance imaging and its widespread availability has led to an increase of reported cases, often asymptomatic, with the need to standardize definitions, diagnostic criteria and treatments.
AIMS
We present shared Interregional Recommendations developed with the primary aim to estimate Syringomyelia and Chiari Syndrome prevalence and incidence in North Western Italy, with special reference to symptomatic forms.
METHODS
An agreement for the standardization of definitions, classifications, diagnostic criteria and surgical Recommendations was reached by the multidisciplinary Interregional Piemonte and Valle d'Aosta Chiari-Syringomyelia Consortium (Delphi method); next, in 2011 a census for Syringomyelia and Chiari Malformation was performed through the Interregional Piemonte and Valle d'Aosta Rare Disease Registry, integrated by a dedicated form in order to estimate prevalence and incidence.
RESULTS
436 patients, 292 females, met shared interregional diagnostic criteria. Syringomyelia prevalence was estimated in 4.84:100 000; Chiari Malformation prevalence was 7.74:100 000; incidence was 0.82:100 000 and 3.08:100 000 respectively. Demographics, neuroradiological parameters and aetiology were reported (in symptomatic and asymptomatic forms). Finally, symptoms and signs, familiar and natural history were analyzed.
CONCLUSIONS
First Italian epidemiological data (prevalence, incidence) on Chiari and syringomyelia was collected, according to shared diagnostic Recommendations. Future perspectives include the adoption of these Recommendations at national level to standardize the access to diagnosis and care process and promote multicenter clinical trials.
Topics: Adolescent; Adult; Arnold-Chiari Malformation; Asymptomatic Diseases; Child; Child, Preschool; Decompression, Surgical; Disease Progression; Encephalocele; Female; Forms as Topic; Humans; Incidence; Italy; Magnetic Resonance Imaging; Male; Middle Aged; Phenotype; Practice Guidelines as Topic; Pregnancy; Pregnancy Complications; Prevalence; Rare Diseases; Registries; Syringomyelia; Young Adult
PubMed: 32242535
DOI: 10.4415/ANN_20_01_08 -
The Journal of Spinal Cord Medicine Nov 2021Symptomatic post-traumatic syringomyelia can affect the quality of life in patients whose neurologic function has already been impacted by a spinal cord injury. To... (Review)
Review
Symptomatic post-traumatic syringomyelia can affect the quality of life in patients whose neurologic function has already been impacted by a spinal cord injury. To investigate the radiographic and clinical outcomes following surgery for syringomyelia, we present a literature review along with a case series from a single surgeon's experience. A retrospective review was conducted on patients with post-traumatic syringomyelia who were treated by a single surgeon. Thirty-four patients who underwent surgical treatment consisting of syrinx fenestration, lysis of adhesions, and duraplasty were identified. In addition, a narrative literature review was conducted with a primary focus on diagnosis and management of post-traumatic syringomyelia. Literature review suggests that regardless of age, sex, vertebral location, or severity of trauma, patients who experience a spinal cord injury should be closely monitored for post-traumatic syringomyelia. Retrospective review of our 34 patients revealed 24 patients for whom pre- and post- operative MRI was available. The predominant location of the injury was cervical (15). The average syrinx length, measured in spinal segments, was similar when comparing pre- and post-operative MRIs; average syrinx length was 5.5 and 5.4 spinal segments, respectively. In contrast, syrinx axial dimension was decreased in 16 of the patients post-operatively and stable or increased in the other eight. The change in syrinx size did not correlate with clinical outcomes. Current surgical treatment of post-traumatic syringomyelia involves restoration of normal CSF flow dynamics; further prospective work is needed to correlate the clinical state, radiographic measures, and efficacy of surgical intervention.
Topics: Humans; Magnetic Resonance Imaging; Quality of Life; Retrospective Studies; Spinal Cord Injuries; Syringomyelia
PubMed: 32223591
DOI: 10.1080/10790268.2020.1743086