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Ashish Chugh, Prashant Punia, Sarang Gotecha, Jayant Arun Gaud, Rajeev Reddy, Ramis Abdul Aziz
Chiari malformation (CM) is a common neurological disorder with foramen magnum decompression (FMD) as a commonly accepted treatment. The authors present a case of CM-1 wherein there was no radiological instability preoperatively and FMD was done as a treatment, after which the patient improved transiently only to deteriorate further. Atlantoaxial fixation was done as a second-stage procedure, after which the patient improved clinically and radiologically. The knowledge of this case and surgical entity should be borne in mind before the formulation of a treatment plan...
2024: Journal of Craniovertebral Junction and Spine
#2
JOURNAL ARTICLE
Yuanxuan Xia, Zach Pennington, A Karim Ahmed, Daniel Sciubba, Philippe Gailloud
BACKGROUND: Spinal hemangioblastomas are often evaluated with catheter angiography for both workup and treatment planning. We report a unique longitudinal pulse-synchronous bouncing phenomenon observed during their angiographic evaluation and consider the association of pulse-synchronous bouncing with syringomyelia, another pathologic feature associated with hemangioblastomas. METHODS: Preoperative spinal angiograms and associated MRIs obtained over a 16-year period at a single institution were retrospectively evaluated...
April 18, 2024: World Neurosurgery
#3
JOURNAL ARTICLE
Maria Pia Tropeano, Zefferino Rossini, Andrea Franzini, Ali Baram, Donato Creatura, Luca Raspagliesi, Federico Pessina, Maurizio Fornari
BACKGROUND: Intramedullary spinal cord tumors (IMSCTs) are a rare subgroup of neoplasms, encompassing both benign, slow-growing masses and malignant lesions; radical surgical excision represents the cornerstone of treatment for such pathologies regardless of histopathology, which, on the other hand, is a known predictor of survival and neurological outcome post-surgery. The present study aims to investigate the relevance of other factors in predicting survival and long-term functional outcome...
April 10, 2024: World Neurosurgery
#4
REVIEW
Alaina Dhawan, Jillian Dhawan, Ajay N Sharma, Daniel B Azzam, Ahmed Cherry, Michael G Fehlings
OBJECTIVE: Chiari malformations (CMs) are a group of congenital or acquired disorders characterized by hindbrain overcrowding into an underdeveloped posterior cranial fossa. CM is considered largely sporadic-however, there exists growing evidence of transmissible genetic underpinnings. The purpose of this systematic review of all familial studies of CM was to investigate the existence of an inherited component and provide recommendations to manage and monitor at-risk family members. METHODS: This paper includes the following: 1) a unique case report of dizygotic twins who presented at the Toronto Western Hospital Spinal Cord Clinic with symptomatic CM type 1 (CM-1) and syringomyelia; and 2) a systematic review of familial CM...
April 12, 2024: Journal of Neurosurgery. Spine
#5
JOURNAL ARTICLE
Enver I Bogdanov, John D Heiss
The diagnosis of Chiari I malformation is straightforward in patients with typical signs and symptoms of Chiari I malformation and magnetic resonance imaging (MRI) confirming ≥5 mm of cerebellar tonsillar ectopia, with or without a syrinx. However, in many cases, Chiari I malformation is discovered incidentally on MRI to evaluate global headache, cervical radiculopathy, or other conditions. In those cases, the clinician must consider if cerebellar tonsillar ectopia is related to the presenting symptoms...
2024: Advances and Technical Standards in Neurosurgery
#6
JOURNAL ARTICLE
Anand M Hatgaonkar, Sandeep M Mahajan, Kajal A Hatgoankar, Gulshan R Bandre
Chiari malformation (CM) type 1 is a complex neurological disorder characterized by the displacement of the cerebellar tonsils into the upper spinal canal. Hydrosyringomyelia (HSM), which frequently coexists with this condition, presents diagnostic and treatment problems due to its broad spectrum of symptoms. There are various forms of CMs, with CM type 1 (CM1) being the most common type. Magnetic resonance imaging (MRI) is the best imaging technique to properly identify and diagnose CM1 and HSM. Important imaging findings include downward displacement of the cerebellar tonsils across the foramen magnum, the appearance of the syrinx in the spinal cord, and the alteration of the flow dynamics of the cerebrospinal fluid...
March 2024: Curēus
#7
JOURNAL ARTICLE
Alexander T Yahanda, Joyce Koueik, Laurie L Ackerman, P David Adelson, Gregory W Albert, Philipp R Aldana, Tord D Alden, Richard C E Anderson, David F Bauer, Tammy Bethel-Anderson, Karin Bierbrauer, Douglas L Brockmeyer, Joshua J Chern, Daniel E Couture, David J Daniels, Brian J Dlouhy, Susan R Durham, Richard G Ellenbogen, Ramin Eskandari, Herbert E Fuchs, Gerald A Grant, Patrick C Graupman, Stephanie Greene, Jeffrey P Greenfield, Naina L Gross, Daniel J Guillaume, Todd C Hankinson, Gregory G Heuer, Mark Iantosca, Bermans J Iskandar, Eric M Jackson, George I Jallo, James M Johnston, Bruce A Kaufman, Robert F Keating, Nickalus R Khan, Mark D Krieger, Jeffrey R Leonard, Cormac O Maher, Francesco T Mangano, Jonathan Martin, J Gordon McComb, Sean D McEvoy, Thanda Meehan, Arnold H Menezes, Michael S Muhlbauer, Brent R O'Neill, Greg Olavarria, John Ragheb, Nathan R Selden, Manish N Shah, Chevis N Shannon, Joshua S Shimony, Matthew D Smyth, Scellig S D Stone, Jennifer M Strahle, Mandeep S Tamber, James C Torner, Gerald F Tuite, Elizabeth C Tyler-Kabara, Scott D Wait, John C Wellons, William E Whitehead, Tae Sung Park, David D Limbrick, Raheel Ahmed
OBJECTIVE: Congenital anomalies of the atlanto-occipital articulation may be present in patients with Chiari malformation type I (CM-I). However, it is unclear how these anomalies affect the biomechanical stability of the craniovertebral junction (CVJ) and whether they are associated with an increased incidence of occipitocervical fusion (OCF) following posterior fossa decompression (PFD). The objective of this study was to determine the prevalence of condylar hypoplasia and atlas anomalies in children with CM-I and syringomyelia...
April 5, 2024: Journal of Neurosurgery. Pediatrics
#8
JOURNAL ARTICLE
Mo Li, Xinyu Wang, Boling Qi, Shengyu Cui, Tianqi Zheng, Yunqian Guan, Longbing Ma, Sumei Liu, Qian Li, Zhiguo Chen, Fengzeng Jian
BACKGROUND: Syringomyelia is a progressive chronic disease that leads to nerve pain, sensory dissociation, and dyskinesia. Symptoms often do not improve after surgery. Stem cells have been widely explored for the treatment of nervous system diseases due to their immunoregulatory and neural replacement abilities. METHODS: In this study, we used a rat model of syringomyelia characterized by focal dilatation of the central canal to explore an effective transplantation scheme and evaluate the effect of mesenchymal stem cells and induced neural stem cells for the treatment of syringomyelia...
April 5, 2024: Tissue Engineering and Regenerative Medicine
#9
JOURNAL ARTICLE
Joao Miguel De Frias, Steven De Decker, Alberta De Stefani, Francisco Llabres-Diaz
The conformation of spinal arachnoid diverticula (SAD) and their clinical implications are poorly characterized in dogs. This retrospective cross-sectional study describes different SAD conformations in dogs and aims to identify if there is an association between SAD conformation and clinical features, localization, syringomyelia (SM) presence, concurrent vertebral condition, treatment option, and short as well as long-term outcome. Sixty-two dogs were included (12 cervical and 50 thoracolumbar SAD). All dogs with a cervical SAD had a cranial tethered conformation and were not included in the statistical analysis...
April 4, 2024: Veterinary Radiology & Ultrasound
#10
Nabha Mahajan, Suresh Phatak, Prashant Onkar, Ashish N Ambhore, Pranit Pantawane
A Chiari I malformation is a frequently encountered anomaly of the posterior fossa, occurring in a notable percentage of the population. It often coexists with various other craniovertebral junction abnormalities, albeit less frequently with Klippel-Feil syndrome. Interestingly, the majority of individuals with Chiari I malformation do not exhibit any symptoms. We present a rare case of a 25-year-old male with chronic neck and occipital pain, along with progressive weakness and sensory disturbances in all four limbs, urinary urgency, and elevated left shoulder...
March 2024: Curēus
#11
JOURNAL ARTICLE
Ignazio G Vetrano, Arianna Barbotti, Tommaso Francesco Galbiati, Sabrina Mariani, Alessandra Erbetta, Luisa Chiapparini, Veronica Saletti, Laura G Valentini
Background: In patients with Chiari 1.5 malformation (CM1.5), a more aggressive disease course and an increased association with craniovertebral junction (CVJ) anomalies has been suggested. The best management of this subgroup of patients is not clearly defined, also due to the lack of specific series elucidating this anomaly's peculiar characteristics. Methods: We evaluated a series of 33 patients (25 females, 8 males; mean age at surgery: 13 years) fulfilling the criteria for Chiari 1.5 diagnosis who underwent posterior fossa decompression and duraplasty (PFDD) between 2006 and 2021...
March 15, 2024: Journal of Clinical Medicine
#12
JOURNAL ARTICLE
Mehmet Yigit Akgun, Ozkan Ates, Goktug Akyoldas, Mehmet Ali Tepebasili, Mehdi Sasani, Tunc Oktenoglu, Ali Fahir Ozer
Syringomyelia associated with extensive spinal adhesive arachnoiditis (SAA) can be defined as a rare but progressive disease with potentially devastating clinical consequences. Diagnosis can be challenging due to the absence of specific clinical findings, confusion with other pathologies, and late imaging modalities. The treatment strategy for syringomyelia associated with extensive SAA should include direct drainage of the syringomyelia, and dissection of the adhesive arachnoid with expansive duraplasty. Hence, several approaches have been reported for arachnoid dissection and decompression of the subarachnoid space...
March 22, 2024: Neuro-Chirurgie
#13
JOURNAL ARTICLE
Vikas N Vattipally, Sina Ahmadi, Carly Weber-Levine, Ann Liu, Nancy Abu-Bonsrah, Jawad Khalifeh, Jordina Rincon-Torroella, Ali Bydon
BACKGROUND: Spinal arachnoid webs (SAWs) are rare pathologies of the spinal meninges often associated with syringomyelia and the radiographic "scalpel sign." Patients can experience pain, numbness, gait disturbances, or no symptoms at all. They are typically diagnosed via magnetic resonance imaging and treated with laminectomy and excision. OBSERVATIONS: A 61-year-old male presented after a mechanical fall and had an incidentally discovered SAW on imaging. He was initially asymptomatic and was therefore conservatively managed...
March 11, 2024: J Neurosurg Case Lessons
#14
JOURNAL ARTICLE
Donald E Ogolo, E C Ajare, C A Ndubuisi, Okwunodulu Okwuoma, Sunday Nnama, S C Ohaegbulam
BACKGROUND AND OBJECTIVES: Posterior fossa pathologies can have potentially devastating outcomes. The volumetric capacity of this fossa, known to have ethnoregional variations, can thus be critical in determining outcomes and intervention measures and approaches to pathologies involving this region. This study aimed to evaluate the normal posterior fossa volumes within the West-African subpopulation. METHODS: This was a descriptive study of all patients presenting for a cranial imaging study at the study location within a two-year period using a 1...
April 2024: World neurosurgery: X
#15
JOURNAL ARTICLE
Göksal Günerhan, Emin Çağıl, Zeynep Dağlar, Uğur Kemal Gündüz, Ali Dalgıç, Ahmet Deniz Belen
Objective The aim this study is to present the results of the minimal invasive neuroendoscopic-assisted system application as an alternative to traditional surgery in patients with Chiari malformation type 1 (CM type 1) with/without syringomyelia. Design, Setting, and Participants In the study, data of 22 symptomatic patients were prospectively collected. Before and after the operation, patient characteristics, computed tomography, magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) flow dynamics MRI, and outcome scales scores were recorded...
April 2024: Journal of Neurological Surgery. Part B, Skull Base
#16
JOURNAL ARTICLE
Daniel Sherlock, Nolan J Brown, Alvin Y Chan, Jessica K Campos, Joffre Olaya
BACKGROUND: Unlike syringomyelia, syringobulbia is not commonly observed in pediatric patients with Chiari malformation type I (CMI). Previous series have reported the incidence of syringobulbia as between 3% and 4% in these patients. Presentation is typically chronic, with the slow onset of neurological symptoms and cranial nerve (CN) palsies resulting from lower brainstem involvement. The authors report the first case of a pediatric patient with simultaneous CMI, syringobulbia, and unilateral CN VII palsy...
March 4, 2024: J Neurosurg Case Lessons
#17
REVIEW
Grant Alexander Bateman, Alexander Robert Bateman
Spaceflight associated neuro-ocular syndrome (SANS) alters the vision of astronauts during long-duration spaceflights. Previously, the current authors have discussed the similarities and differences between SANS and idiopathic intracranial hypertension to try to elucidate a possible pathophysiology. Recently, a theory has been advanced that SANS may occur secondary to failure of the glymphatic system caused by venous dilatation within the brain and optic nerves. There is recent evidence to suggest glymphatic obstruction occurs in childhood hydrocephalus, multiple sclerosis and syringomyelia due to venous outflow dilatation similar to that proposed in SANS...
February 28, 2024: NPJ Microgravity
#18
JOURNAL ARTICLE
Turkka Kirjavainen, Päriä Miraftabi, Laura Martelius, Atte Karppinen
OBJECTIVES: Chiari type 1 malformation (CM1) may occasionally lead to central sleep apnea (CSA). We studied, in a large clinical cohort of pediatric CM1 patients, the effect of CM1 on breathing during sleep. METHODS: This is a retrospective single pediatric pulmonology center study with a systematic evaluation of pediatric CM1 patients under age 18 with polysomnography (PSG) during 2008-2020. Children with syndromes were excluded. All patients had undergone head and spine magnetic resonance imaging...
February 16, 2024: Sleep Medicine
#19
JOURNAL ARTICLE
Samir Moniruzzaman, Aku Kaipainen, Joona Tervonen, Jukka Huttunen, Henna-Kaisa Jyrkkänen, Terhi J Huuskonen, Susanna Rantala
PURPOSE: The purpose of our study was to examine the long-term outcomes of operated Chiari malformation type 1 (CM1) patients and evaluate whether different duraplasty techniques affected outcome after surgery in Kuopio University Hospital catchment area. METHODS: In this retrospective study, a total of 93 patients were diagnosed with CM1 and underwent posterior fossa decompression surgery with or without duraplasty between 2005 and 2020. All patients' medical records were examined for baseline characteristics, surgical details, and long-term follow-up data after operation...
February 28, 2024: Acta Neurochirurgica
#20
JOURNAL ARTICLE
Dipak D Pukale, Kayla Adkins-Travis, Siddhartha R Aryal, Leah P Shriver, Gary J Patti, Nic D Leipzig
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...
February 26, 2024: Fluids and Barriers of the CNS
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