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arnold chiari

Sepideh Sefidbakht, Sakineh Dehghani, Maryam Safari, Homeira Vafaei, Maryam Kasraeian
BACKGROUND: Magnetic resonance imaging (MRI) is gradually becoming more common for thorough visualization of the fetus than ultrasound (US), especially for neurological anomalies, which are the most common indications for fetal MRI and are a matter of concern for both families and society. OBJECTIVES: We investigated fetal MRIs carried out in our center for frequency of central nervous system anomalies. This is the first such report in southern Iran. MATERIALS AND METHODS: One hundred and seven (107) pregnant women with suspicious fetal anomalies in prenatal ultrasound entered a cross-sectional retrospective study from 2011 to 2013...
August 2016: Iranian Journal of Pediatrics
Zachary C Fausel, Dale C Strasser
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
Ruth Mc Donagh, David Bradley, Joseph Augustine Harbison
A previously well 30-year-old woman presented at 17:30 with a sudden onset of dizziness, ataxia and headache. She was initially investigated with a CT scan of the brain and lumbar puncture, which yielded no diagnosis. Subsequent MR scan revealed multiple posterior circulation infarcts, along with a previously undiagnosed Arnold-Chiari 2 malformation with an associated syrinx of her cervical and thoracic spine. The infarct involved one of the herniated cerebellar tonsils. Oedema of an infarct in the herniated tonsils caused compression of the medulla at the foramen magnum, with associated neurological symptoms including Lhermitte's phenomenon and headache on valsalva manoeuvre...
2016: BMJ Case Reports
Keely Smith, Ana M Gomez-Rubio, Tomika S Harris, Lauren E Brooks, Ricardo A Mosquera
We present a case of a 17-year-old Hispanic male with Arnold-Chiari Type 1 [AC-Type 1] with syringomyelia, status post decompression, who complains of exercise intolerance, headaches, and fatigue with exertion. The patient was found to have diurnal hypercapnia and nocturnal alveolar hypoventilation. Cardiopulmonary testing revealed blunting of the ventilatory response to the rise in carbon dioxide (CO2) resulting in failure of the parallel correlation between increased CO2 levels and ventilation; the expected vertical relationship between PETCO2 and minute ventilation during exercise was replaced with an almost horizontal relationship...
2016: Case Reports in Pediatrics
Pavaman Pandit Sindgikar, Kuntal Kanti Das, Awadesh K Jaiswal, Rabi Narayan Sahu, Arun K Srivastava, Anant Mehrotra, Jayesh Chunilal Sardhara, Kamlesh Singh Bhaisora, Sanjay Behari
INTRODUCTION: Surgery for craniovertebral junction (CVJ) abnormalities like atlantoaxial dislocation (AAD) with or without basilar invagination (BI) and/or with or without associated Arnold-Chiari malformation (ACM) cause high cervical myelopathy. Occasionally, mechanical factors such as inadequate canal decompression, torticollis, and/or scoliosis may lead to lack of improvement following the primary surgery. Also, implant-related factors requiring its revision/removal or surgical site infections may cause patient to undergo resurgery...
August 2016: Neurosurgery
Saif Yousif, Mark Walsh, Hannah Burns
INTRODUCTION: Bilateral vocal cord palsy is a condition which has many causes (Gupta et al., 2012) [1]. Syringomyelia is an uncommon condition which describes the formation of fluid filled cavity, occupying the spinal cord (Chang, 2003) [2]. It rarely manifests itself as subacute onset of stridor. PRESENTATION OF CASE: We present the case of a three year old female who presented for evaluation of her speech and language delay, when incidentally it was made note of her loud breathing which had previously been managed as bronchiolitis by her general practitioner...
2016: International Journal of Surgery Case Reports
N Sharma, E Bache, T Clare
INTRODUCTION: We report a case of an adolescent sustaining bilateral femoral neck fractures due to a first time epileptic seizure, as a result of expansion of his known syrinx. CASE REPORT: A 19-year-old patient suffering from hypophosphatasia (HPP), Arnold-Chiari malformation, and a ventriculoperitoneal shunt sustained a trivial fall with profound pain and an inability to mobilize. Radiographs demonstrated a right-sided Garden-4 femoral neck and left-sided multi-fragmentary intracapsular/extracapsular fractures...
July 2015: Journal of Orthopaedic Case Reports
Gaston O Camino Willhuber, Santiago T Bosio, Miguel H Puigdevall, Carolina Halliburton, Carlos A Sola, Ruben A Maenza
To present and describe an unusual case of spinal instability after craniocervical spinal decompression for a type-1 Chiari malformation. Type-1 Chiari malformation is a craniocervical disorder characterized by tonsillar displacement greater than 5 mm into the vertebral canal; posterior fossa decompression is the most common surgical treatment for this condition. Postoperative complications have been described: cerebrospinal fluid leak, pseudomeningocele, aseptic meningitis, wound infection, and neurological deficit...
June 2, 2016: Journal of Pediatric Orthopedics. Part B
Je Hoon Jeong, A Leum Lee, Sung Yoon Cho, Dong Kyu Jin, Soo-Bin Im
SPOndylar and NAsal changes, with STRIations of the Metaphyses (SPONASTRIME) dysplasia (SD) is a dwarfing autosomal recessive syndrome, characterized by a variety of clinical and radiographic features, which form the basis for diagnosis. We describe the presentation of an Arnold Chiari malformation in a patient with a clinical diagnosis of SD. The malformation was successfully treated by decompression of the foramen magnum and elevation of the cerebellum, with complete resolution of pain.We report a rare case of Arnold Chiari malformation in a patient presenting with clinical and radiographic features strongly suggestive of SD and be successfully treated...
May 2016: Medicine (Baltimore)
L Ouhadi, M Gaudreault, S Mottard, Ph Gillet
Charcot arthropathy is a progressive, chronic and degenerative destruction of one or several joints caused by a central or peripheral neurological disorder. Approximately 25 % of the patients with syringomyelia develop this arthropathy located in the upper limb in 80 % of the cases. An early etiological diagnosis is essential to begin the treatment of the underlying neurological disorder. Afterwards, a conservative treatment of the arthropathy is preferred. We report the story of a patient with an arthropathy of the left shoulder due to Arnold-Chiari's malformation of type I with syringomyelia...
February 2016: Revue Médicale de Liège
Wen Zhang, Shifu Sha, Leilei Xu, Zhen Liu, Yong Qiu, Zezhang Zhu
BACKGROUND: Though several studies have reported the incidence of intraspinal neural axis abnormalities in infantile and juvenile "presumed idiopathic" scoliosis, there has been a varying prevalence ranging from 11.1 to 26.0% based on a limited sample size. Therefore, such inconclusive findings have resulted in some questions on the MRI-associated role in the management of these patients. We aimed to investigate the prevalence and distribution of intraspinal anomalies in the infantile and juvenile patients with "presumed idiopathic" scoliosis and to explore the radiographic and clinical indicators with large sample size...
2016: BMC Musculoskeletal Disorders
E Miraglia, G Fabbrini, C Di Biasi, C Iacovino, G Ferrazzano, G Gualdi, S Calvieri, S Giustini
OBJECTIVE: To evaluate the possible correlation and the true incidence between Neurofibromatosis type 1 and Arnold-Chiari malformation type I. MATERIALS AND METHODS: We reviewed all clinical charts, neurological consultations and MRI scans of 428 NF1 patients followed by 1994 to 2014 in our Department. NF1 patients in our clinic are seen usually every year by both the dermatologist and the neurologist. All patients also undergo a brain and spinal cord with the same 1...
2016: La Clinica Terapeutica
Jonathan Esteban-Sánchez, Almudena Rueda-Marcos, Ricardo Sanz-Fernández, Eduardo Martín-Sanz
INTRODUCTION: The presentation of a down-beating nystagmus force to discard vascular pathology of brain and cervical joint with magnetic resonance imagine (MRI). Recent studies support the low profitability of this study and is subjected that this oculomotor sign has a peripheral origin especially when the patient has a benign paroxysmal positional vertigo (BPPV) with affection of the superior semicircular canal. AIM: To evidence the profitability of MRI in a population of patients with positional down-beating nystagmus...
February 1, 2016: Revista de Neurologia
P Mace, E Quarello
The diagnosis of an abnormal fetal posterior fossa is usually done during the second trimester scan. However, some forms of open spina bifida with Arnold-Chiari malformation can be detected from the first trimester ultrasound with a precise examination of the posterior fossa and intracranial translucency. Furthermore, other abnormalities of the posterior fossa such as cystic malformations also seem to be accessible to early detection. This work detailed the possible usual and unusual aspects of the posterior fossa individualized during the first trimester ultrasound scan...
January 2016: Gynécologie, Obstétrique & Fertilité
Ryan M Smith, Ivan Garza, Carrie E Robertson
No abstract text is available yet for this article.
December 1, 2015: Neurology
M Ono, A Kojima, M Shirahashi, S Noda, Y Akiba, S Nishijima, T Amagata, T Ikeda, K Yakubo, T Fukuiya
No abstract text is available yet for this article.
May 2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
Nihat Demir, Erdal Peker, İsmail Gülşen, Kemal Ağengin, Sultan Kaba, Oğuz Tuncer
Jarcho-Levin syndrome (JLS) is a genetic disorder characterized by distinct malformations of the ribs and vertebrae, and/or other associated abnormalities such as neural tube defect, Arnold-Chiari malformation, renal and urinary abnormalities, hydrocephalus, congenital cardiac abnormalities, and extremity malformations. The study included 12 cases at 37-42 weeks of gestation and diagnosed to have had Jarcho-Levin syndrome, Arnold-Chiari malformation, and meningmyelocele. All cases of Jarcho-Levin syndrome had Arnold-Chiari type 2 malformation; there was corpus callosum dysgenesis in 6, lumbosacral meningmyelocele in 6, lumbal meningmyelocele in 3, thoracal meningmyelocele in 3, and holoprosencephaly in 1 of the cases...
March 2016: Journal of Child Neurology
Nicole Silva Bevilacqua, Denise Araujo Lapa Pedreira
Meningomyelocele is a malformation with high prevalence, and one of its main comorbidities is Arnold-Chiari malformation type II. The intrauterine repair of this defect has been studied to reduce the progressive spinal cord damage during gestation. The purpose of the present review was to describe the evolution of fetal surgery for meningomyelocele repair. Searches on PubMed database were conducted including articles published in the last 10 years. Twenty-seven articles were selected, 16 experimental studies and 11 studies in humans...
April 2015: Einstein
Ismael Ejarque, José M Millán-Salvador, Silvestre Oltra, José V Pesudo-Martínez, Magdalena Beneyto, Antonio Pérez-Aytés
INTRODUCTION: Noonan syndrome (NS) and other syndromes with a similar phenotype, such as LEOPARD, cardiofaciocutaneous, Costello and Legius, are associated to mutations in genes included in the RAS/MAPK pathway (RASopathies), which is an important signalling pathway related to cell proliferation. Tonsillar descent into the upper cervical spinal canal, known as Arnold-Chiari malformation (ACM), has been reported in patients with NS and this has led some researchers to suggest that ACM could be part of the phenotypic spectrum of NS...
May 1, 2015: Revista de Neurologia
Jean-François Lecompte, Geraldine Hery, Jean-Michel Guys, Claude Louis-Borrione
OBJECTIVE: To examine the effectiveness of posterior tibial nerve stimulation (PTNS) for the treatment of fecal and urinary incontinence in children with malformations of the bowel or neurological pathologies. INTRODUCTION: Treatment of fecal and urinary leaks, in cases of congenital malformations remains a challenge. Recent studies in adults have shown the effectiveness of PTNS. METHOD: Eight children: 4 with anorectal malformations, 3 with neurological causes (1 medullary lipoma, 1 Arnold Chiari malformation, 1 sacrococcygeal teratoma) and 1 with Hirschsprung's disease presenting with serious anal incontinence, despite extensive bowel management during at least 2 years, were treated with PTNS...
April 2015: Journal of Pediatric Surgery
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