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https://www.readbyqxmd.com/read/28103199/diagnostic-criteria-for-susac-syndrome
#1
Ilka Kleffner, Jan Dörr, Marius Ringelstein, Catharina C Gross, Yvonne Böckenfeld, Wolfram Schwindt, Benedikt Sundermann, Hubertus Lohmann, Heike Wersching, Julia Promesberger, Natascha von Königsmarck, Anne Alex, Rainer Guthoff, Catharina J M Frijns, L Jaap Kappelle, Sven Jarius, Brigitte Wildemann, Orhan Aktas, Friedemann Paul, Heinz Wiendl, Thomas Duning
BACKGROUND: Susac syndrome is characterised by the triad of encephalopathy with or without focal neurological signs, branch retinal artery occlusions and hearing loss. Establishment of the diagnosis is often delayed because the triad is complete only in a minority of patients at disease onset. This leads to a critical delay in the initiation of appropriate treatment. Our objective was to establish criteria for diagnosis of either definite or probable Susac syndrome. METHOD: The establishment of diagnostic criteria was based on the following three steps: (1) Definition of a reference group of 32 patients with an unambiguous diagnosis of Susac syndrome as assessed by all interdisciplinary experts of the European Susac Consortium (EuSaC) team (EuSaC cohort); (2) selection of diagnostic criteria, based on common clinical and paraclinical findings in the EuSaC cohort and on a review of the literature; and (3) validation of the proposed criteria in the previously published cohort of all Susac cases reported until 2012...
December 2016: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28102813/intramedullary-amputation-neuroma-a-case-report-and-review-of-the-literature
#2
Laura Stone McGuire, Mandana Behbahini, Sumit Das, David Loeffler, Peter Burger, Herbert Engelhard, Tibor Valyi-Nagy, Ankit Mehta
BACKGROUND AND IMPORTANCE: Amputation neuromas consist of non-neoplastic collections of myelinated axons and Schwann cells and typically arise in injured peripheral nerves. Rarely, however, neuromas occur within the spinal cord. Intramedullary amputation neuromas have been described both with and without a history of trauma within the peripheral nervous system. We report a rare case of an isolated intramedullary spinal cord amputation neuroma. CLINICAL PRESENTATION: This 43-year-old man presented with progressive and severe gait deterioration for ~ 7 years...
January 19, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28102623/ischemic-stroke-in-kawasaki-disease
#3
Wasana Prangwatanagul, Alisa Limsuwan
Pediatric stroke is considered to be rare. Stroke resulting from cerebral vasculitis is also uncommon in young children. With the increasing prevalence of Kawasaki disease (KD) diagnosis, this acquired vasculitis has been reported with various clinical presentations including neurological symptoms. Herein we describe the case of a KD patient presenting with stroke. A 15-month-old boy was referred due to stroke that occurred on the fifth day of febrile illness. He was initially admitted to another hospital due to fever and diarrhea...
January 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28102512/anti-phospholipid-syndrome-associated-with-schizophrenia-description-of-five-patients-and-review-of-the-literature
#4
Pikman Regina, Rotman Pnina, Aiman Natur, Levy Yair
Anti-phospholipid syndrome is an autoimmune disorder characterized by anti-phospholipid antibodies, arterial and venous thrombosis, pregnancy morbidity, and various neurological manifestations including psychiatric disorders. Higher incidence of various autoimmune disorders was found in schizophrenia. In addition, an association between the presence of anti-phospholipid antibodies and schizophrenia or psychosis was previously described, mainly as case reports. Although initially believed to be a result of neuroleptic treatment, the reasons for this association remain obscure...
January 19, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28102171/lumbar-disc-herniation-causing-cauda-equina-syndrome-in-a-paediatric-patient-a-case-report
#5
Mohamad Irshad, Khurshid Ahmad, Hilal Ahmad Malla
Lumbar disc disease occurs mainly in the adult population. A disc prolapse in the paediatric population is very rare. Cauda equine syndrome resulting from compression of the cauda equina is a rare syndrome and is one of the few spinal surgical emergencies. Here we present a 13-year-old boy with pain in the lumbar region radiating bilaterally to the lower limbs, with asymmetrical weakness of lower the limbs, perianal hypoaesthesia and urinary retention. MRI of lumbar spine confirmed disc protrusion at the L3-L4 level with severe spinal canal stenosis...
August 30, 2016: Ortopedia, Traumatologia, Rehabilitacja
https://www.readbyqxmd.com/read/28101360/successful-treatment-of-anti-nmda-receptor-encephalitis-with-a-prompt-ovarian-tumour-removal-and-prolonged-course-of-plasmapheresis-a-case-report
#6
Elzbieta Rypulak, Michal Borys, Pawel Piwowarczyk, Magdalena Fijalkowska, Beata Potrec, Justyna Sysiak, Janusz Spustek, Alicja Bartkowska-Sniatkowska, Jan Kotarski, Waldemar A Turski, Konrad Rejdak, Miroslaw Czuczwar
Anti-N-methyl-d-aspartate-receptor (NMDAR) encephalitis is an uncommon autoimmune disorder with a wide spectrum of neuropsychiatric symptoms. There is a great requirement to emphasize the importance of a multidisciplinary team approach in the process of diagnosis and treatment of the potentially fatal condition, including psychiatrists, neurologists, gynaecologists and intensivists. Physicians must be aware that psychiatric and neurological disorders, which are typical features for NMDAR encephalitis in young women with ovarian tumours, may progress into status epilepticus and respiratory insufficiency...
December 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28100097/spina-bifida-and-lower-limb-amputation-in-northern-ireland-a-retrospective-study-of-demographics-and-outcome
#7
Lorraine Graham
BACKGROUND: Spina bifida is an uncommon cause for lower limb amputation. The causes and level of amputation and mobility outcome for these patients have not been reported previously. CASE DESCRIPTION: To identify the causes and level of amputation and the mobility outcome for amputee patients with spina bifida. STUDY DESIGN: Retrospective case series. METHODS: Chart review of patients identified by computer as having an amputation secondary to neurological or congenital cause...
January 1, 2017: Prosthetics and Orthotics International
https://www.readbyqxmd.com/read/28099107/case-238-spontaneous-pneumothorax-secondary-to-intrapulmonary-necrobiotic-rheumatoid-nodule
#8
Ammar A Chaudhry, Maryam Gul, Abbas A Chaudhry, William Moore
History A 54-year-old white woman with a history of rheumatoid arthritis who was taking glucocorticoids and methotrexate presented to the emergency department in December with worsening shortness of breath and chest heaviness for 1 week. She reported additional symptoms of weakness, headache, and arthralgia primarily involving her bilateral hands, wrist, ankles, and feet. She denied experiencing fevers, syncope or presyncope, focal neurologic deficits, chest pain, nausea, vomiting, unintentional weight loss, or recent trauma...
February 2017: Radiology
https://www.readbyqxmd.com/read/28097854/-citrullinemia-type-i-with-recurrent-liver-failure-in-a-child
#9
Verónica Bindi, Hernán Eiroa
Citrullinemia type I is an autosomal recessive disorder caused by mutation of the gene expressing ASS1 argininosuccinate synthetase, limiting enzyme of the urea cycle. The classic variants are associated with neonatal/infantile forms that cause hyperammonemia leading to death if treatment is not established. Initial symptoms of disorders of the urea cycle include neurological impairment with mild or moderate liver damage. We report a case of recurrent liver failure in an infant diagnosed with type I citrullinemia without severe neurological involvement that was referred to our center for liver transplantation...
1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28097252/enterococcus-faecalis-causing-delayed-spondylodiscitis-in-a-case-with-retained-intraspinal-bullet
#10
Siddharth N Aiyer, Ajoy Prasad Shetty, Rishi Kanna, Srikanth Reddy, Shanmuganathan Rajasekaran
Delayed presentations have been reported following gunshot wounds (GSW) with retained intraspinal bullets due to migration of projectile or lead intoxication. We report on the rare occurrence of delayed pyogenic spondylodiscitis and neurological dysfunction following injury from low velocity GSW to the spine with a retained projectile. A 55-year-old male presented 4 months following GSW to the abdomen which resulted in colonic injury and L5 fracture. The patient was treated initially with ileo-transverse anastomosis, antibiotics, without retrieval of the bullet...
December 2016: Journal of Spine Surgery (Hong Kong)
https://www.readbyqxmd.com/read/28097250/spinal-cord-compression-from-wegener-s-granulomatosis-an-unusual-presentation
#11
Deb Roy, Prashanth J Rao, Kevin Phan, Ralph J Mobbs, Michael Selby
Wegener's granulomatosis (WG) causing spinal cord compression is very rare with only few cases reported in literature. We present a case report with review of literature. A 55-year-old lady with known WG presented with acute on chronic spinal cord compression. MRI scan revealed spinal cord compression anteriorly and posteriorly at T2-T5 level. Patient underwent urgent surgical decompression with excision of the posterior dural lesion with synthetic duraplasty. Patient made good neurological recovery. Histopathology revealed features consistent with WG...
December 2016: Journal of Spine Surgery (Hong Kong)
https://www.readbyqxmd.com/read/28095665/a-case-of-cerebral-cysticercosis-in-thailand
#12
Samasuk Thammachantha, Ratana Kunnatiranont, Pongwat Polpong
Cysticercosis and sparganosis are not uncommon parasitic infections in the developing world. Central nervous system infection by both cestodes can present with neurological signs and symptoms, such as seizure and mass effect, including brain hernia. Early detection and accurate diagnosis can prevent a fatal outcome. Histological examinations of brain tissues can confirm the diagnosis of cerebral cysticercosis, which differs from sparganosis by the presence of a cavitated body. We report here a case of cerebral cysticercosis which has the similar clinical and imaging findings as sparganosis...
December 2016: Korean Journal of Parasitology
https://www.readbyqxmd.com/read/28095468/evaluating-hospital-based-surveillance-for-outbreak-detection-in-bangladesh-analysis-of-healthcare-utilization-data
#13
Birgit Nikolay, Henrik Salje, Katharine Sturm-Ramirez, Eduardo Azziz-Baumgartner, Nusrat Homaira, Makhdum Ahmed, A Danielle Iuliano, Repon C Paul, Mahmudur Rahman, M Jahangir Hossain, Stephen P Luby, Simon Cauchemez, Emily S Gurley
BACKGROUND: The International Health Regulations outline core requirements to ensure the detection of public health threats of international concern. Assessing the capacity of surveillance systems to detect these threats is crucial for evaluating a country's ability to meet these requirements. METHODS AND FINDINGS: We propose a framework to evaluate the sensitivity and representativeness of hospital-based surveillance and apply it to severe neurological infectious diseases and fatal respiratory infectious diseases in Bangladesh...
January 2017: PLoS Medicine
https://www.readbyqxmd.com/read/28095134/physical-and-occupational-therapy-for-a-teenager-with-acute-flaccid-myelitis-a-case-report
#14
Tracy Kornafel, Elaine Y Tsao, Emily Sabelhaus, Lori Surges, Susan D Apkon
AIMS: The purpose of this case report is to describe the inpatient rehabilitation program of a 13-year-old boy with acute flaccid myelitis (AFM), specific to physical and occupational therapy examination, treatment, and outcomes. CASE DESCRIPTION: AFM is a rare, acute neurologic illness in children and young adults who present with weakness and/or paralysis of unknown etiology. The teenager was admitted to the program, dependent for all mobility and self-care. Interventions focused on range of motion, transfer training, self-care, power wheelchair mobility, and environmental adaptations...
January 17, 2017: Physical & Occupational Therapy in Pediatrics
https://www.readbyqxmd.com/read/28094021/neuropsychological-characteristics-of-encephalopathy-in-susac-s-syndrome-case-report
#15
Magdalena Roessler-Górecka, Tadeusz Mendel, Justyna Wiśniowska, Joanna Seniów
Susac's Syndrome (SS) is a rare, autoimmune angiopathy characterized by hearing loss, retinal artery occlusions and encephalopathy, which is usually expressed in multifocal neurological signs and symptoms, confusion state and cognitive impairment. There have been few descriptions of neuropsychological assessment of SS. We present a case study of 29-year-old woman who developed full SS. During the post-acute stage of disease, she was admitted to neurorehabilitation ward to improve her cognitive-behavioral and motor functioning...
January 10, 2017: Neurologia i Neurochirurgia Polska
https://www.readbyqxmd.com/read/28092986/a-review-of-the-treatment-of-primary-orthostatic-hypotension
#16
Genevieve M Hale, Jose Valdes, Michael Brenner
OBJECTIVE: To review the efficacy and safety of pharmacological and nonpharmacological strategies used to treat primary orthostatic hypotension (OH). DATA SOURCES: A literature review using PubMed and MEDLINE databases searching hypotension, non-pharmacological therapy, midodrine, droxidopa, pyridostigmine, fludrocortisone, atomoxetine, pseudoephedrine, and octreotide was performed. STUDY SELECTION AND DATA EXTRACTION: Randomized or observational studies, cohorts, case series, or case reports written in English between January 1970 and November 2016 that assessed primary OH treatment in adult patients were evaluated...
January 1, 2017: Annals of Pharmacotherapy
https://www.readbyqxmd.com/read/28092914/paraneoplastic-cerebellar-degeneration-associated-with-pelvic-liposarcoma-a-rare-case-report
#17
Liang Zhou, Xin Wei, Nian-Wei Wu, Han-Mei Zhang, Bang-Hua Liao, Shu-Lian Chen, Hong Li, Yu-Chun Zhu, Kun-Jie Wang
Paraneoplastic cerebellar degeneration (PCD) is one of the most common paraneoplastic neurological syndromes characterized by the rapid development of severe cerebellar ataxia. In this report, a 23-year-old female with noticeable dizziness and gait instability was described. The enhanced CT scanning suggested the presence of a pelvic tumor. Then, PCD was established. Postoperative pathological result defined it as a liposarcoma (LS) with dedifferentiation. Interestingly, clinical symptoms disappeared after the surgical removal of the pelvic tumor...
January 17, 2017: Urologia Internationalis
https://www.readbyqxmd.com/read/28089837/direct-midline-posterior-corpectomy-and-fusion-of-a-lumbar-burst-fracture-with-retrospondyloptosis-a-case-report
#18
Arthur Carminucci, Rachid Assina, R Nick Hernandez, Ira M Goldstein
BACKGROUND: Traumatic burst fractures of the lumbar spine can result in significant neurological injury and mechanical instability. The ideal surgical approach for the treatment of unstable lumbar spine burst fractures remains debatable. CASE DESCRIPTION: A 37 year-old male presented with severe neurological injury including loss of motor function below the level of the iliopsoas muscles bilaterally, saddle anesthesia, and absent rectal tone, following a fall from 60 ft...
January 9, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28089323/neurophysiological-monitoring-of-lumbar-spinal-nerve-roots-a-case-report-of-postoperative-deficit-and-literature-review
#19
Yuguang Chen, Baoqing P Wang, Junlin Yang, Yaolong Deng
INTRODUCTION: Intraoperative neurophysiological monitoring (IONM) has proven to help reduce the probability of postoperative neurological deficit for spinal deformity correctional surgeries. However, in rare cases new deficits may still happen. We report a surgical case in which the patient had postoperative paralysis. We would like to call for more case reports with postoperative neurological deficits as they present difficult clinical cases. PRESENTATION OF CASE: A 61-year-old male patient with severe thoracolumbar kyphoscoliosis underwent posterior spinal correction and fusion with segmental T10-L5 pedicle screws and rods instrumentation with IONM...
November 19, 2016: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28088217/surgical-treatment-of-upper-cervical-spine-metastases-a-retrospective-study-of-39-cases
#20
Jian Yang, Qi Jia, Dongyu Peng, Wei Wan, Nanzhe Zhong, Yan Lou, Xiaopan Cai, Zhipeng Wu, Chenglong Zhao, Xinghai Yang, Jianru Xiao
BACKGROUND: The surgical treatment of upper cervical spine metastases are controversial up to now. By summarizing and analyzing the clinical data of the upper cervical spine involved metastases treated surgically in our center, we mainly aimed to investigate the surgical decisions and outcomes so as to provide more references for the clinical treatment of this special and complex spine metastasis. METHODS: We evaluated the patients' pre- and post-operative neck pain and neurologic function with paired t test, followed by the statistics of the selection of surgical approaches, ways of reconstruction, and related complications...
January 14, 2017: World Journal of Surgical Oncology
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