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Chiari I

Wouter I Schievink, M Marcel Maya, Zachary R Barnard, Franklin G Moser, Stacey Jean-Pierre, Alan D Waxman, Miriam Nuño
BACKGROUND: Behavioral variant frontotemporal dementia (bvFTD) is a devastating early onset dementia. Symptoms of bvFTD may be caused by spontaneous intracranial hypotension (SIH), a treatable disorder, but no comprehensive study of such patients has been reported. OBJECTIVE: To describe detailed characteristics of a large cohort of patients with SIH and symptoms of bvFTD. METHODS: We identified patients with SIH who met clinical criteria for bvFTD...
March 8, 2018: Operative Neurosurgery (Hagerstown, Md.)
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
Hasan Önal, Atilla Ersen, Hakan Gemici, Erdal Adal, Serhat Güler, Serdar Sander, Sait Albayram
Spontaneous intracranial hypotension (SIH) is a rare and potentially serious condition in childhood. Cerebrospinal fluid (CSF) volume depletion is thought to be the main causative feature for intracranial hypotension, results from spontaneous CSF leak, often at the spine level. SIH is increasingly diagnosed in clinical practice, although it manifests into a variegated symptomatology. Indeed, downward displacement of the brain, sometimes mimicking a Chiari I malformation, but concomitant presentation of these syndromes has rarely been reported...
February 27, 2018: Journal of Clinical Research in Pediatric Endocrinology
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
Surget V Beatrous, Ryan R Riahi, Stratton B Grisoli, Philip R Cohen
Dermatofibromas are benign, fibrohistiocytic, dermal tumors. Solitary dermatofibromas may be incidental findings, whereas multiple dermatofibromas may be associated with systemic conditions or previous therapies. Two women and one man with multiple dermatofibromas and an associated systemic condition, immunosuppression, or both, are described. Nine dermatofibromas developed in a woman with hypothyroidism, optic neuritis, and Arnold Chiari I malformation. Five dermatofibromas developed in a woman with breast cancer who had received several systemic antineoplastic therapies...
September 22, 2017: Dermatology Online Journal
Jeffrey M Rogers, Greg Savage, Marcus A Stoodley
Displacement of the cerebellar tonsils in Chiari type I malformation (CMI) can affect functions controlled by the cerebellum and brainstem. While playing an integral role in the control of movement, the cerebellum also has widespread cortical connections, influencing a range of cognitive process. A systematic literature review was conducted to examine the relationship between cognition and CMI, assessing evidence for general or domain-specific cognitive change. The search protocol examined the AMED, CINAHL, Cochrane Library, EMBASE, MEDLINE, PsycINFO, and Scopus databases...
February 21, 2018: Neuropsychology Review
I Swarup, P Derman, E Sheha, J Nguyen, J Blanco, R Widmann
Purpose: Previous studies have suggested an association between increased thoracic kyphosis and neural axis abnormalities in patients with adolescent idiopathic scoliosis (AIS). However, the basis for this finding is unclear, and this association has been mainly noted in retrospective studies on a non-consecutive series of patients. The purpose of this study was to assess the relationship between thoracic kyphosis and neural axis abnormalities in patients with AIS. Methods: We studied a consecutive series of AIS patients treated with spinal fusion...
February 1, 2018: Journal of Children's Orthopaedics
Shuai Guo, Xue-Feng Fan, Jie-Yuan Jin, Liang-Liang Fan, Lei Zeng, Zheng-Bing Zhou, Rong Xiang, Ju-Yu Tang
Background: Chiari malformation type II (CM-II) is mainly characterized by elongation and descent of the cerebellum through the foramen magnum into the spinal canal. Moreover, CM-II is uniquely associated with myelomeningocele. Sprengel's deformity refers to the malposition of the scapula, i.e. scapular elevation which is sometimes accompanied with scapula dysplasia. Although few familial cases of CM-II and Sprengel's deformity have been previously reported, both of these defects are considered to be sporadic, thus the exact etiology and causative genes have largely remained unknown...
2018: Molecular Cytogenetics
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
Gordan Grahovac, Tatiana Pundy, Tadanori Tomita
OBJECTIVES: Chiari I malformation has been a well-recognized clinical entity; however, its occurrence among infants and toddlers is unusual. Their clinical presentations may be different from other age groups due to their lack of effective verbal communication. The authors analyze their personal series of patients focusing on symptomatology and MRI characteristics. Treatment methods, results, and outcome are analyzed in order to identify appropriate surgical management among infants and toddlers with Chiari I malformation...
February 2, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Gavin J Andrews
Human geography's varied engagement with the brain has involved considerations of the way people know and respond to their environments, and their place-based experiences with emotions, mental illnesses and disorders, intellectual disabilities and particular neurological conditions. This paper argues however that this scholarship could be augmented by, and existing expertise be directed towards, considering physical brain abnormalities and injuries. As a case in point it considers the spatial experience of living with Type 1 Chiari Malformation...
January 27, 2018: Social Science & Medicine
Giuseppe Brancatelli, Alessandro Furlan, Alberto Calandra, Marco Dioguardi Burgio
Hepatic sinusoidal dilatation refers to the enlargement of the hepatic capillaries. Most of the time this condition is caused by hepatic venous outflow obstruction, which results in vascular stasis and congestion of hepatic parenchyma. In this setting, hepatic sinusoidal dilatation can be related to pericardial disease, heart failure, compression or thrombosis of the hepatic veins or inferior vena cava (i.e., Budd-Chiari syndrome) or central veins/sinusoids involvement (i.e., sinusoidal obstruction syndrome)...
February 1, 2018: Abdominal Radiology
Giacomo Tondo, Fabiola De Marchi, Daniela Mittino, Roberto Cantello
Occipital neuralgia (ON) is characterized by severe pain in the occipital region due to an irritation of the occipital nerves. Traumatic injuries, mass or vascular compression, and infective and inflammatory processes could cause ON. The dislocation of a nerve/muscle/tendon, as can happen in malformations such as the Chiari I malformation (CIM), also can be responsible. Usually, headaches associated with CIM and ON are distinguishable based on specific features of pain. However, the diagnosis is not easy in some cases, especially if a clear medical history cannot be accurately collected...
November 29, 2017: Curēus
James R Houston, Michelle L Hughes, Mei-Ching Lien, Bryn A Martin, Francis Loth, Mark G Luciano, Sarel Vorster, Philip A Allen
Type I Chiari malformation (CMI) is a neurological condition in which the cerebellar tonsils descend into the cervical spinal subarachnoid space resulting in cervico-medullary compression. Early case-control investigations have indicated cognitive deficits in the areas of attention, memory, processing speed, and visuospatial function. The present study further examined cognitive and emotional processing deficits associated with CMI using a dual-task paradigm. Nineteen CMI patients were recruited during pre-surgical consultation and 19 matched control participants identified emotional expressions in separate single and asynchronous dual-task designs...
January 30, 2018: Cerebellum
Mohammadali M Shoja, Rebecca Ramdhan, Chad J Jensen, Joshua Chern, W Jerry Oakes, R Shane Tubbs
Although the embryology of the posterior cranial fossa can have life altering effects on a patient, a comprehensive review on this topic is difficult to find in the peer-reviewed medical literature. Therefore, this review paper, using standard search engines, seemed timely. The embryology of the posterior cranial fossa is complex and relies on a unique timing of various neurovascular and bony elements. Derailment of these developmental processes can lead to a wide range of malformations such as the Chiari malformations...
January 18, 2018: Clinical Anatomy
Fatima Azahraa Haddad, Ibraheem Qaisi, Nagham Joudeh, Hamada Dajani, Fareed Jumah, Amjad Elmashala, Nimer Adeeb, Joshua J Chern, R Shane Tubbs
In 1891, Hans Chiari described a group of congenital hindbrain anomalies, which were eventually named after him. He classified these malformations into three types (Chiari malformations I, II, and III), and four years later added the Chiari IV malformation. However, numerous reports across the literature do not seem to fit Chiari's original descriptions of these malformations, so researchers have been encouraged to propose new classifications to encompass these variants (e.g., Chiari 0, Chiari1.5, and Chiari 3...
April 2018: Clinical Anatomy
Tatsuya Ohtonari, Nobuharu Nishihara, Shinzo Ota, Akio Tanaka
OBJECTIVE: We investigated CSF dynamics at CCJ using Time-SLIP MRI to demonstrate the significance of ventral and dorsal combined CSF dynamics in assessing CSF flow disturbance in patients with CM-I. METHODS: Fifteen examinations were performed in nine cases of CM-I (three females patients; mean age, 24.7 years; age range, 11-46 years) before or after craniocervical decompression. The longitudinal maximum movement of the caudal edge of tagged mid-sagittal CSF at CCJ was measured as a LOM, and total on the ventral and dorsal sides was defined as total LOM...
January 8, 2018: World Neurosurgery
Matthias Buechter, Paul Manka, Guido Gerken, Ali Canbay, Sandra Blomeyer, Axel Wetter, Jens Altenbernd, Alisan Kahraman, Jens M Theysohn
BACKGROUND AND AIMS: Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment of choice in decompensated portal hypertension. TIPS revision due to thrombosis or stenosis increases morbidity and mortality. Our aim was to investigate patient- and procedure-associated risk factors for TIPS-revision. PATIENTS AND METHODS: We retrospectively evaluated 189 patients who underwent the TIPS procedure. Only patients who required TIPS revision within 1 year (Group I, 34 patients) and patients who did not require re-intervention within the first year (Group II [control group], 54 patients) were included...
January 9, 2018: Digestive Diseases
Tatsushi Inoue, Natsuki Hattori, Tsukasa Ganaha, Tadashi Kumai, Shinichiro Tateyama, Yuichi Hirose
Chiari malformation type I (CM-I) is typically treated with foramen magnum decompression. However, a recent study proposed a new technique for patients with CM-I, wherein only short atlantoaxial joint fusion and distraction is applied. Posterior fusion with or without atlantoaxial distraction is a potential option for patients with CM-I associated with basilar invagination or complex anomalous bony craniovertebral junction pathology, since this procedure allows clinicians to avoid using the technically demanding transoral approach in which some or all of the odontoid tip is invisible...
January 5, 2018: Journal of Neurosurgery. Spine
Silky Chotai, Jade Basem, Stephen Gannon, Michael Dewan, Chevis N Shannon, John C Wellons, Christopher M Bonfield
BACKGROUND/AIMS: Scoliosis is common in patients with Chiari malformation-I (CM-I). This study examined the change in scoliosis severity after posterior fossa decompression (PFD) for CM-I. METHODS: We conducted a retrospective review at a single tertiary center for children undergoing PFD with untreated scoliosis, and identified 17 patients with complete follow-up data and imaging. RESULTS: Overall, scoliosis improved in 7 (41.2%) patients, worsened in 9 (52...
January 4, 2018: Pediatric Neurosurgery
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