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Neurologic Clinics May 2022Chiari Malformation Type I (CMI) is a congenital malformation diagnosed by MRI findings of at least 5 mm of cerebellar ectopy below the foramen magnum. CM1 is... (Review)
Review
Chiari Malformation Type I (CMI) is a congenital malformation diagnosed by MRI findings of at least 5 mm of cerebellar ectopy below the foramen magnum. CM1 is frequently associated with syringomyelia. Herein, we discuss the history of CMI and syringomyelia, including early pathological and surgical studies. We also describe recent investigations into the pathogenesis and pathophysiology of CMI and their practical implications on management and surgical intervention. We also highlight the recent development of the Common Data Elements for CMI, providing a framework for ongoing investigations. Finally, we discuss current controversies of surgical management in CMI.
Topics: Arnold-Chiari Malformation; Foramen Magnum; Humans; Magnetic Resonance Imaging; Syringomyelia
PubMed: 35465876
DOI: 10.1016/j.ncl.2021.11.007 -
Neurologia Jun 2019Much has been published on syringomyelia related to Chiari malformation. In contrast, little is known about the condition when it is not associated with this... (Review)
Review
INTRODUCTION
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. We conducted a literature review to summarise the most accepted and widespread ideas about the pathophysiology, management and other aspects of syringomyelia unrelated to Chiari malformation.
DEVELOPMENT
We reviewed the most relevant literature on this condition, focusing on the pathophysiology, clinical presentation, diagnosis, and treatment.
CONCLUSIONS
Syringomyelia unrelated to Chiari malformation is a distinct entity that must be well understood to guarantee correct diagnosis, monitoring, and management. When the disease is suspected, a thorough study should be conducted to identify its aetiology. Treatment must aim to eliminate the cause of the disease; symptomatic treatment should remain a second-line option.
Topics: Arnold-Chiari Malformation; Female; Humans; Magnetic Resonance Imaging; Male; Postoperative Complications; Syringomyelia; Treatment Outcome
PubMed: 27939111
DOI: 10.1016/j.nrl.2016.09.010 -
The Journal of Spinal Cord Medicine Nov 2017Spinal cord injury (SCI) is a devastating condition that can lead to significant neurological impairment and reduced quality of life. Despite advancements in our... (Review)
Review
CONTEXT
Spinal cord injury (SCI) is a devastating condition that can lead to significant neurological impairment and reduced quality of life. Despite advancements in our understanding of the pathophysiology and secondary injury mechanisms involved in SCI, there are currently very few effective treatments for this condition. The field, however, is rapidly changing as new treatments are developed and key discoveries are made.
METHODS
In this review, we outline the pathophysiology, management, and long-term rehabilitation of individuals with traumatic SCI. We also provide an in-depth overview of emerging therapies along the spectrum of the translational pipeline.
EVIDENCE SYNTHESIS
The concept of "time is spine" refers to the concept which emphasizes the importance of early transfer to specialized centers, early decompressive surgery, and early delivery of other treatments (e.g. blood pressure augmentation, methylprednisolone) to affect long-term outcomes. Another important evolution in management has been the recognition and prevention of the chronic complications of SCI including respiratory compromise, bladder dysfunction, Charcot joints, and pressure sores through directed interventions along with early integration of physical rehabilitation and mobilization. There have also been significant advances in neuroprotective and neuroregenerative strategies for SCI, many of which are actively in clinical trial including riluzole, Cethrin, stem cell transplantation, and the use of functional electrical stimulation.
CONCLUSION
Pharmacologic treatments, cell-based therapies, and other technology-driven interventions will likely play a combinatorial role in the evolving management of SCI as the field continues to evolve.
Topics: Adult; Humans; Male; Neuralgia; Neurological Rehabilitation; Postoperative Complications; Spinal Cord Injuries; Spinal Fusion; Syringomyelia; Urinary Bladder, Neurogenic
PubMed: 28571527
DOI: 10.1080/10790268.2017.1329076 -
Neurology India 2022
Topics: Humans; Syringomyelia; Scoliosis; Arnold-Chiari Malformation; Magnetic Resonance Imaging
PubMed: 36352639
DOI: 10.4103/0028-3886.359263 -
The Pan African Medical Journal 2022
Topics: Anal Canal; Digestive System Abnormalities; Humans; Magnetic Resonance Imaging; Meningocele; Rectum; Sacrum; Syringomyelia
PubMed: 35519164
DOI: 10.11604/pamj.2022.41.143.33419 -
Arquivos de Neuro-psiquiatria Dec 2017Many myths and legends have had a deep influence on modern language, and on modern medical vernacular. The terms "syrinx" and "panic"are two of the most characteristic...
Many myths and legends have had a deep influence on modern language, and on modern medical vernacular. The terms "syrinx" and "panic"are two of the most characteristic examples and their use in neurology and other specialties is well known. This article reviews the history of these words in Greek mythology and their use in modern medicine. It is known by very few that clinical symptoms or conditions, such as syringomyelia and panic attacks, have a mythological origin in their definition and naming.
Topics: Greece; Humans; Mythology; Panic; Syringomyelia; Terminology as Topic
PubMed: 29236893
DOI: 10.1590/0004-282X20170140 -
Asian Journal of Surgery Feb 2023The prognosis of syringomyelia is not yet established. Syringomyelia derived from different etiologies contributes to similar symptoms.
BACKGROUND
The prognosis of syringomyelia is not yet established. Syringomyelia derived from different etiologies contributes to similar symptoms.
OBJECTIVE
Assess the syringomyelia in our medical institutes and describe the etiologies and clinical appearance of the disorder. And identify the predictors of a good outcome and to find the most suitable timing of surgical intervention according to our results.
METHODS
This retrospective cohort study used databases in our hospitals to analyze 70 cases of syringomyelia between 1997 and 2014. All available information was obtained from medical records and radiological reports. We used American Spinal Injuries Association disability scores (ASIA scores), the modified Nurick classification system, and recorded the number of days the patient was hospitalized, for neurological and functional assessment. Univariate and multivariate analyses were used to evaluate the relationship between clinical factors and outcomes.
RESULTS
Non-communicating syringomyelia was the most common type of syringomyelia. In univariate analysis, autonomic dysfunction and motor impairment were strong predictors of poor neurological and functional outcomes. In addition to the above factors, syrinxes at the cervical level predicted better functional outcomes than at any spinal level in multivariate analysis.
CONCLUSIONS
Motor impairment, which is commonly seen in patients with syringomyelia in Taiwan, is a strong predictor to poor neurological and functional outcomes. Our study indicates that patients without autonomic dysfunction or motor impairment should receive timely surgical intervention to prevent symptomatic deterioration. We also found that cervical syringomyelia in particular has the potential for good functional recovery after adequate intervention.
Topics: Humans; Syringomyelia; Retrospective Studies; Prognosis; Postoperative Complications; Magnetic Resonance Imaging; Treatment Outcome
PubMed: 35868963
DOI: 10.1016/j.asjsur.2022.06.150 -
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 -
Journal of Neurology, Neurosurgery, and... Sep 1985A woman with syringobulbia and syringomyelia and her sister with syringomyelia are described. The diagnosis was confirmed radiologically and surgically in the first...
A woman with syringobulbia and syringomyelia and her sister with syringomyelia are described. The diagnosis was confirmed radiologically and surgically in the first sister and no other definite abnormalities of her craniocervical junction were found.
Topics: Adult; Female; Humans; Middle Aged; Syringomyelia
PubMed: 4045487
DOI: 10.1136/jnnp.48.9.936 -
Medicine Dec 2022The current surgical management of adult Chiari malformation type I (CM-I) with associated syringomyelia remains controversial. The objective of this study was to... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
The current surgical management of adult Chiari malformation type I (CM-I) with associated syringomyelia remains controversial. The objective of this study was to explore posterior fossa decompression and duraplasty (PFDD) with and without tonsillar resection in adult patients with CM-I and syringomyelia.
METHODS
A total of 116 adult patients suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression at our institution between 2012 and 2020 were randomly divided into 2 groups: the PFDD group (n = 64) underwent PFDD without tonsillar resection, while the PFDD-T group (n = 52) underwent PFDD with tonsillar resection. The primary outcome was improvement or resolution of the syrinx. The secondary outcome was an improvement in clinical outcome based on Chicago Chiari Outcome Scale (CCOS) scores. All participants were followed-up to 1-year postoperatively.
RESULTS
The proportions of patients who had >20% improvement in syrinx size were 60.9% and 78.8% in the PFDD and PFDD-T groups, respectively (P = .038). The improvement in clinical outcome based on CCOS scores was significantly different in the 2 groups (P = .004). The functionality sub-score was significantly different between the 2 groups (P = .027), but there were no significant differences in the pain symptoms, non-pain symptoms, and complications sub-scores. The total CCOS scores were higher in the PFDD-T group than in the PFDD group (P = .037).
CONCLUSION
This study determined the role of tonsillar resection in achieving obvious syrinx improvement following PFDD-T. PFDD with tonsillar resection seems to be a safe and effective surgical option to treat adult CM-I patients with syringomyelia.
Topics: Humans; Adult; Arnold-Chiari Malformation; Syringomyelia; Dura Mater; Decompression, Surgical; Treatment Outcome; Retrospective Studies
PubMed: 36550873
DOI: 10.1097/MD.0000000000031394