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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 -
Acta Neuropathologica Communications Aug 2014We explored the prevalence of syringomyelia in a series of 113 cases of fetal dysraphism and hindbrain crowding, of gestational age ranging from 17.5 to 34 weeks with...
We explored the prevalence of syringomyelia in a series of 113 cases of fetal dysraphism and hindbrain crowding, of gestational age ranging from 17.5 to 34 weeks with the vast majority less than 26 weeks gestational age. We found syringomyelia in 13 cases of Chiari II malformations, 5 cases of Omphalocele/Exostrophy/Imperforate anus/Spinal abnormality (OEIS), 2 cases of Meckel Gruber syndrome and in a single pair of pyopagus conjoined twins. Secondary injury was not uncommon, with vernicomyelia in Chiari malformations, infarct like histology, or old hemorrhage in 8 cases of syringomyelia. Vernicomyelia did not occur in the absence of syrinx formation. The syringes extended from the sites of dysraphism, in ascending or descending patterns. The syringes were usually in a major proportion anatomically distinct from a dilated or denuded central canal and tended to be dorsal and paramedian or median. We suggest that fetal syringomyelia in Chiari II malformation and other dysraphic states is often established prior to midgestation, has contributions from the primary malformation as well as from secondary in utero injury and is anatomically and pathophysiologically distinct from post natal syringomyelia secondary to hindbrain crowding.
Topics: Anus, Imperforate; Arnold-Chiari Malformation; Ciliary Motility Disorders; Encephalocele; Fetus; Gestational Age; Hernia, Umbilical; Humans; Polycystic Kidney Diseases; Retinitis Pigmentosa; Scoliosis; Syringomyelia; Urogenital Abnormalities
PubMed: 25092126
DOI: 10.1186/s40478-014-0091-0 -
Journal of Spine Surgery (Hong Kong) Jun 2018The spinal arachnoid web is an abnormal formation of an arachnoid membrane in the subarachnoid space. It is a rare entity with some degree of uncertainty surrounding its... (Review)
Review
The spinal arachnoid web is an abnormal formation of an arachnoid membrane in the subarachnoid space. It is a rare entity with some degree of uncertainty surrounding its etiology. It can result in a displacement of the spinal cord causing pain and neurological symptoms as well as blockage of cerebrospinal fluid (CSF) flow and subsequent syringomyelia. The syrinx resulting from the altered CSF flow dynamics has been described to assume variable positions relative to the web itself. The "scalpel sign" is regarded as a pathognomonic feature of a spinal arachnoid web. The arachnoid web, however, is relatively thin and may be elusive of routine radiological investigations. As such, a myriad of preoperative and intraoperative investigations have been postulated to improve the sensitivity of detecting this abnormality. Management of spinal arachnoid webs ranges from conservative management to surgical excision where in the latter, the extent of excision remains the subject of debate. The authors herein present a review of the available information on this rare topic.
PubMed: 30069540
DOI: 10.21037/jss.2018.05.08 -
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