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Hsin-Chin Lin, Yu-Lin Tsai, Wen-Chung Tsai
Posttraumatic syringomyelia with an initial presentation of involuntary movement is rare. We describe a 25-year-old patient who sustained complete traumatic spinal cord injury at the thoracic level and presented with rhythmic neck muscle spasms and upper limb muscle myoclonic jerks 1 month after trauma. Magnetic resonance imaging revealed syrinx formation between C3 and T1. Lumbar-peritoneal shunt and decompression were performed. The symptoms completely disappeared after surgery. This report highlights that rhythmic neck muscle spasms and upper limb muscle myoclonic jerks can be the initial and only manifestations of syringomyelia...
March 14, 2018: PM & R: the Journal of Injury, Function, and Rehabilitation
Valentina Baro, Renzo Manara, Luca Denaro, Domenico d'Avella
PURPOSE: Dandy-Walker malformation is a rare condition due to imperforation of the Blake's pouch during intrauterine brain development, usually leading to early severe hydrocephalus. The association with holocord syringomyelia is rare, and from the Gardner's first report in 1957, only 23 cases have been described, mostly from autopsy series and pre-MRI period. Besides a worsening of clinical picture, its occurrence generates some concern about the best surgical treatment that varies widely among the literature reports...
March 12, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Ash Singhal, Alexander Cheong, Paul Steinbok
INTRODUCTION: In 2003, pediatric neurosurgeons were surveyed under the auspices of the education committee of the International Society for Pediatric Neurosurgery (ISPN) to determine prevailing opinions regarding the management of Chiari I malformation (C1M) with and without associated syringomyelia. In the ensuing years, there has been further information from multiple C1M studies, with regards to indications, success rates of different surgical interventions, and complications. The purpose of this study was to re-evaluate current opinions and practices in pediatric C1M...
March 12, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Srdjan Cirovic, Robert Lloyd, Jelena Jovanovik, Holger A Volk, Clare Rusbridge
BACKGROUND: Syringomyelia is a pathological condition in which fluid-filled cavities (syringes) form and expand in the spinal cord. Syringomyelia is often linked with obstruction of the craniocervical junction and a Chiari malformation, which is similar in both humans and animals. Some brachycephalic toy breed dogs such as Cavalier King Charles Spaniels (CKCS) are particularly predisposed. The exact mechanism of the formation of syringomyelia is undetermined and consequently with the lack of clinical explanation, engineers and mathematicians have resorted to computer models to identify possible physical mechanisms that can lead to syringes...
March 9, 2018: BMC Veterinary Research
Christina Mousele, Miltiadis Georgiopoulos, Constantine Constantoyannis
CONTEXT: Syringobulbia is a very rare progressive disorder of central nervous system, with several possible underlying conditions. Rarely, it is also encountered as a late complication of syringomyelia. FINDINGS: In the present manuscript, a case of a paraplegic patient, due to traumatic spinal cord injury (thoracolumbar fracture), presenting after years progressively developing symptoms of the lower cranial nerves and upper extremities, owed to syringomyelia and syringobulbia, the surgical treatment applied and its outcomes are described...
February 27, 2018: Journal of Spinal Cord Medicine
Zhi Chai, Xiaoming Xue, Huijie Fan, Lin Sun, Hongyu Cai, Yanmiao Ma, Cungen Ma, Ran Zhou
This study aimed to quantitatively assess and compare the effect and safety of posterior fossa decompression with duraplasty (PFDD) and posterior fossa decompression (PFD) in treating patients with Chiari malformation type I (CM1). PubMed, Embase, and Cochrane Library were searched through May 2017. Fourteen cohort studies, involving a total of 3666 patients with CM1, were included. Studies were pooled, and the relative risk (RR) and its corresponding 95% confidence interval (CI) were calculated. The decrease in syringomyelia was better for patients in the PFDD group than for patients in the PFD group (RR=1...
February 23, 2018: World Neurosurgery
Michael Faloon, Nikhil Sahai, Todd P Pierce, Conor J Dunn, Kumar Sinha, Ki Soo Hwang, Arash Emami
BACKGROUND: Several studies have sought to address the role of routine preoperative MRI in patients with adolescent idiopathic scoliosis (AIS) undergoing deformity correction. Despite similar results regarding the prevalence of neuraxial anomalies detected on MRI, published conclusions conflict and give opposing recommendations. Lack of consensus has led to important variations in use of MRI before spinal surgery for patients with AIS. QUESTIONS/PURPOSES: This systematic review and meta-analysis of studies about patients with AIS evaluated (1) the overall proportion of neuraxial abnormalities; (2) the patient factors and curve characteristics that may be associated with abnormalities; and (3) the proportion of patients who underwent neurosurgical intervention before scoliosis surgery and the kinds of neuraxial lesions that were identified...
February 21, 2018: Clinical Orthopaedics and related Research
Marcelo Ferreira Sabba, Beatriz Souza Renor, Enrico Ghizoni, Helder Tedeschi, Andrei Fernandes Joaquim
Chiari malformation (CM) is the most common and prevalent symptomatic congenital craniocervical malformation. Radiological diagnosis is established when the cerebellar tonsils are located 5 mm or more below the level of the foramen magnum on magnetic resonance imaging (MRI). Surgical treatment is indicated whenever there is symptomatic tonsillar herniation or syringomyelia/hydrocephalus. The main surgical treatment for CM without craniocervical instability (such as atlantoaxial luxation) is posterior fossa decompression, with or without duraplasty...
November 2017: Revista da Associação Médica Brasileira
Yoshiaki Takamura, Yasuhiro Takeshima, Ryuta Matsuoka, Young Soo Park, Hiroyuki Nakase
It has been reported that syringomyelia is rarely associated with degenerative spinal disorders, but the case of holocord syringomyelia is never reported. We here present a case of a 59-year-old woman with right shoulder pain, dysesthesia of the right hand, and gait disturbance. Radiographically, examinations of the spine demonstrated holocord syringomyelia with ossification of ligamentum flavum at T2/3 level. Holocord syringomyelia was reduced remarkably after posterior decompression at the T2/3 level, and her symptoms also improved...
February 14, 2018: Acta Neurochirurgica
Ying Zhang, Jingming Xie, Yingsong Wang, Ni Bi, Tao Li, Jie Zhang, Zhi Zhao, Hua Ou, Siyuan Liu
PURPOSE: Documents indicated that the average prevalence of intraspinal neural axis abnormalities (INAAs) in presumed idiopathic scoliosis (PIS) patients was about 17.7%. However, paucity study focuses on the incidence of INAAs in severe spinal deformity (SSDs). In this study, we investigate the incidence of intraspinal neural axis abnormalities (IINAAs) and the clinical relevance in SSD at a single center. METHODS: All the patients with SSDs admitted for spinal surgery were evaluated from 2003 to 2014...
February 14, 2018: European Spine Journal
Shanyong Zhang, Lili Yang, Chuangang Peng, Minfei Wu
The aim of the present study was to investigate the risk factors for postoperative recurrence of spinal tumors by logistic regression analysis and analysis of prognostic factors. In total, 77 male and 48 female patients with spinal tumor were selected in our hospital from January, 2010 to December, 2015 and divided into the benign (n=76) and malignant groups (n=49). All the patients underwent microsurgical resection of spinal tumors and were reviewed regularly 3 months after operation. The McCormick grading system was used to evaluate the postoperative spinal cord function...
February 2018: Oncology Letters
Atul Goel, Sonal Jain, Abhidha Shah
OBJECTIVE: To evaluate the musculoskeletal and soft tissue neural alterations in cases with group A basilar invagination. METHODS: Between January 2007 and August 2016, 510 patients with group A basilar invagination were surgically treated. The radiologic images of these patients were reviewed retrospectively. The patients were divided into 4 groups: group A1, comprising 60 patients with syringomyelia; group A2, comprising 354 patients with "external syrinx," marked by excessive cerebrospinal fluid (CSF) in the extramedullary space; group A3, comprising 51 patients with both syringomyelia and external syrinx; and group A4, comprising 45 patients with no abnormality of CSF cavitation in the spinal canal...
February 2018: World Neurosurgery
Christina Jackson, Brian W Yang, Wenya Linda Bi, E Antonio Chiocca, Michael W Groff
BACKGROUND: Adult tethered cord syndrome is a rare neurological disorder that classically presents with back or leg pain, weakness, and urinary dysfunction. Spinal cord tethering has been associated with acquired Chiari malformations. While the effects of tethered cord release on Chiari malformation symptoms have been previously described, we report an unusual case of acquired tethered cord syndrome following Chiari decompression. CASE DESCRIPTION: We report a 68-year-old man with a history of distant T12-level spinal cord injury who presented with two weeks of progressive bilateral lower extremity weakness...
January 31, 2018: World Neurosurgery
Guihong Li, Yaonan Ding, Chaochao Zhang, Haiyan Huang
We describe a rare case of syringomyelia with left knee Charcot arthropathy in a 35-year-old male, who presented with dissociative sensory disorder, muscle atrophy, painless knee joint swelling and limited joint mobility. Early diagnosis and appropriate treatment are essential in avoiding disease progression.
February 6, 2018: British Journal of Neurosurgery
Katrien Wijnrocx, Liesbeth François, Peter Goos, Nadine Buys, Steven Janssens
Background: The selection of a future breeding dog is a complicated task, in which disease characteristics and different traits have to be combined and weighed against one another. Truncation selection, that is the exclusion of affected animals, may be very inefficient when selecting on a large number of traits, and may result in a reduction of the genetic diversity in a population or breed. Selection could be facilitated by the use of a selection index that combines multiple traits or breeding values into one score...
2018: Canine Genetics and Epidemiology
Maggie S Eppelheimer, James R Houston, Jayapalli R Bapuraj, Richard Labuda, Dorothy M Loth, Audrey M Braun, Natalie J Allen, Soroush Heidari Pahlavian, Dipankar Biswas, Aintzane Urbizu, Bryn A Martin, Cormac O Maher, Philip A Allen, Francis Loth
Purpose: Researchers have sought to better understand Chiari type I malformation (CMI) through morphometric measurements beyond tonsillar position (TP). Soft tissue and bone structures within the brain and craniocervical junction have been shown to be different for CMI patients compared to healthy controls. Yet, several morphological characteristics have not been consistently associated with CMI. CMI is also associated with different prevalent conditions (PCs) such as syringomyelia, pseudotumor, Ehlers-Danlos syndrome (EDS), scoliosis, and craniocervical instability...
2018: Frontiers in Neuroanatomy
Ce Zhu, Shishu Huang, Chunguang Zhou, Xi Yang, Lei Wang, Tingxian Ling, Limin Liu, Yueming Song
RATIONALE: Situs inversus totalis is a rare anomaly disease in which the organs in the chest and abdomen are positioned in a mirror image reversal of normal positions. Although this has been confirmed to be associated with spinal abnormalities, reports about situs inversus totalis with congenital scoliosis remain limited. PATIENT CONCERNS: We present a 9-year-old girl having congenital scoliosis associated with situs inversus totalis. DIAGNOSES: She also had other associated anomalies: ventricular septal defect, mild restrictive ventilatory dysfunction, hydronephrosis, and syringomyelia...
December 2017: Medicine (Baltimore)
Muhammad Uzair Lodhi, Aaron R Kuzel, Intekhab Askari Syed, Mustafa Rahim
Syringomyelia classically presents as a bilateral sensory loss of the dissociated type which includes the loss of pain and temperature with the preservation of fine touch, vibratory sensation, and proprioception in the shoulder, arm, and hand. Eventually, weakness of the legs, muscle wasting, and ataxia can also be seen due to the involvement of the corticospinal tracts and the posterior columns of the spinal cord. We present the case of a 64-year-old patient with an atypical presentation of post-traumatic syringomyelia...
November 16, 2017: Curēus
Masakazu Sano, Junichi Yoshimura, Yukihiko Fujii
Craniosynostosis associated with Chiari malformation (CM) is usually found in infants with an underdeveloped posterior fossa. We here present a case of adult craniosynostosis, CM, and symptomatic syringomyelia caused by the protrusion of the posterior rim of the foramen magnum without a tight posterior fossa. A 22-year-old woman with an abnormal head shape and forearm hypesthesia was given a diagnosis of sagittal suture synostosis with CM and syringomyelia caused by foramen magnum stenosis. She underwent foramen magnum decompression with a C1 laminectomy without cranial vault expansion or duraplasty...
January 2018: NMC Case Report Journal
Aimee Verner, Swati Agarwal-Sinha, Frank Yanfeng Han
BACKGROUND: Möbius syndrome is a rare congenital condition which presents not merely with 6th and 7th nerve palsies, but involves gaze paresis associated with craniofacial, limb, and other abnormalities. Heterogeneity is well known in patients with Möbius syndrome and rather than being of familial inheritance based on rare cases, it is much more recognized as a sporadic syndrome. We report an infant with features of congenital Möbius syndrome associated with cardiac rhabdomyomas in the absence of tuberous sclerosis...
January 16, 2018: Ophthalmic Genetics
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