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Acute ataxia

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https://www.readbyqxmd.com/read/27932510/embolization-of-intracranial-dural-arteriovenous-fistulas-using-phil-liquid-embolic-agent-in-26-patients-a-multicenter-study
#1
S Lamin, H S Chew, S Chavda, A Thomas, M Piano, L Quilici, G Pero, M Holtmannspolter, M E Cronqvist, A Casasco, L Guimaraens, L Paul, A Gil Garcia, A Aleu, R Chapot
BACKGROUND AND PURPOSE: The introduction of liquid embolic agents has revolutionized endovascular approach to cranial vascular malformations. The aim of the study was to retrospectively assess the efficacy and safety of Precipitating Hydrophobic Injectable Liquid (PHIL), a new nonadhesive liquid embolic agent, in the treatment of patients with cranial dural arteriovenous fistulas. The primary end point was the rate of complete occlusion of dural arteriovenous fistulas. Secondary end points included the incidence of adverse events and clinical status at 3-month follow-up...
December 8, 2016: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/27928389/oculomotor-nerve-palsy-presented-with-isolated-unilateral-ptosis-and-minimal-upgaze-palsy
#2
Canan Togay Işıkay, Busra S Polat
This case with unilateral complete blepharoptosis and slight limitation of upgaze as ocular findings due to midbrain infarction represents an unusual form of intra-axial oculomotor nerve involvement. A 65-year-old man was admitted with acute unilateral ptosis of the left eyelid and gait ataxia. He had chronic hypertension in the medical history. The neurologic examination revealed that he had a complete ptosis of the left eyelid, slight limitation of elevation in the left eye, bilateral dysmetria, and gait ataxia...
April 2016: Neuro-ophthalmology
https://www.readbyqxmd.com/read/27894792/pyruvate-dehydrogenase-e1%C3%AE-deficiency-presenting-as-recurrent-acute-proximal-muscle-weakness-of-upper-and-lower-extremities-in-an-8-year-old-boy
#3
Bülent Kara, Hülya Maraş Genç, Emek Uyur-Yalçın, Ayfer Sakarya-Güneş, Uğur Topçu, Serap Mülayim, Serdar Ceylaner
The mitochondrial pyruvate dehydrogenase enzyme complex (PDHC) plays an important role in aerobic energy metabolism and acid-base equilibrium. PDHC contains of 5 enzymes, 3 catalytic (E1, E2, E3) and 2 regulatory, as well as 3 cofactors and an additional protein (E3-binding protein) encoded by nuclear genes. The clinical presentation of PDHC deficiency ranges from fatal neonatal lactic acidosis to chronic neurologic dysfunction without lactic acidosis. Paroxysmal neurologic problems such as intermittent ataxia, episodic weakness, exercise-induced dystonia and recurrent demyelination may also be seen although they are rare...
November 9, 2016: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/27876395/massive-lamotrigine-poisoning-a-case-report
#4
Salvatore Grosso, Silvia Ferranti, Carla Gaggiano, Elisabetta Grande, Barbara Loi, Rosanna Di Bartolo
Lamotrigine (LTG) represents the most commonly prescribed of the so-called new generation antiepileptic drugs. We describe a child who was admitted to the emergency room because of generalized tonic-clonic status epilepticus followed by a complex neurological picture with hyperkinesia and acute ataxia as a result of a LTG intoxication. The experience on acute LTG intoxication is very limited in pediatrics. The present case provides information on the clinical picture related to LTG overdose and confirms that drug intoxications should be considered in the differential diagnosis strategy when severe and polymorphic neurological symptoms occur acutely...
November 19, 2016: Brain & Development
https://www.readbyqxmd.com/read/27862026/unusual-presentation-of-sj%C3%A3-gren-associated-neuropathy-with-plasma-cell-rich-infiltrate
#5
Elie Naddaf, Sarah E Berini, P James B Dyck, Ruple S Laughlin
INTRODUCTION: Sjögren syndrome is thought to be a lymphocyte-driven process. Peripheral nervous system involvement occurs in about 20-25% of patients. A sensory predominant, large fiber peripheral neuropathy is most common, and it is usually associated with a subacute to chronic presentation. METHODS: We report a rare case of an acute Sjögren-associated, sensory predominant, length-dependent peripheral neuropathy mimicking Guillain-Barré syndrome. The patient presented with sensory ataxia preceded by fever and polyarthralgia...
November 16, 2016: Muscle & Nerve
https://www.readbyqxmd.com/read/27855658/cytomegalovirus-associated-encephalomyelitis-in-an-immunocompetent-adult-a-two-stage-attack-of-direct-viral-and-delayed-immune-mediated-invasions-case-report
#6
Kensuke Daida, Yuta Ishiguro, Hiroto Eguchi, Yutaka Machida, Nobutaka Hattori, Hideto Miwa
BACKGROUND: It is clinically rare to find cytomegalovirus (CMV)-associated encephalomyelitis in immunocompetent adults. Here, we present the case of an adult patient who developed acute transverse myelitis that was followed by immune-mediated disseminated encephalomyelitis. CASE PRESENTATION: A 38-year-old man developed acute paraplegia with paresthesia below the level of the T7-8 dermatome. Both brain and spinal cord MRIs performed at admission appeared normal...
November 17, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27826325/enterovirus-71-infection-and-neurological-complications
#7
REVIEW
Kyung Yeon Lee
Since the outbreak of the enterovirus 71 (EV71) infection in Malaysia in 1997, large epidemics of EV71 have occurred in the Asia-Pacific region. Many children and infants have died from serious neurological complications during these epidemics, and EV71 infection has become a serious public health problem in these areas. EV71 infection causes hand, foot and mouth disease (HFMD) in children, and usually resolves spontaneously. However, EV71 occasionally involves the central nervous system (CNS), and induces diverse neurological complications such as brainstem encephalitis, aseptic meningitis, and acute flaccid paralysis...
October 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/27821089/posterior-reversible-encephalopathy-as-the-first-manifestation-of-bickerstaff-s-brainstem-encephalitis
#8
Pei-Ru Chen, Shih-Pin Chen
BACKGROUND: Posterior reversible encephalopathy syndrome (PRES) has been associated with Guillain-Barre syndrome in rare cases. Here we report a patient in whom PRES was the presenting manifestation of Bickerstaff's brainstem encephalitis. CASE PRESENTATION: A 75-year-old woman presented with acute onset of hypertension, headache, blurred vision, and left eyelid drooping. Magnetic resonance imaging of the brain showed characteristic PRES lesions involving the parietal and occipital lobes bilaterally...
November 8, 2016: BMC Neurology
https://www.readbyqxmd.com/read/27817982/from-genotype-to-phenotype-in-dravet-disease
#9
Svetlana Gataullina, Olivier Dulac
Dravet syndrome combines clonic generalized, focal or unilateral seizures, beginning within the first year of life, often triggered by hyperthermia whatever its cause, including pertussis vaccination. Long-lasting febrile seizures are frequent in infancy and repeat status epilepticus (SE) has negative prognostic value. Massive myoclonus, rare absences, complex partial seizures and generalized spikes may appear several years later. Myoclonic status may occur in childhood, but acute encephalopathy with febrile SE followed by ischemic lesions and psychomotor impairment, the most severe condition, occurs mainly within the first five years of life...
October 21, 2016: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/27802825/mog-igg-in-nmo-and-related-disorders-a-multicenter-study-of-50-patients-part-3-brainstem-involvement-frequency-presentation-and-outcome
#10
Sven Jarius, Ingo Kleiter, Klemens Ruprecht, Nasrin Asgari, Kalliopi Pitarokoili, Nadja Borisow, Martin W Hümmert, Corinna Trebst, Florence Pache, Alexander Winkelmann, Lena-Alexandra Beume, Marius Ringelstein, Oliver Stich, Orhan Aktas, Mirjam Korporal-Kuhnke, Alexander Schwarz, Carsten Lukas, Jürgen Haas, Kai Fechner, Mathias Buttmann, Judith Bellmann-Strobl, Hanna Zimmermann, Alexander U Brandt, Diego Franciotta, Kathrin Schanda, Friedemann Paul, Markus Reindl, Brigitte Wildemann
BACKGROUND: Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) are present in a subset of aquaporin-4 (AQP4)-IgG-negative patients with optic neuritis (ON) and/or myelitis. Little is known so far about brainstem involvement in MOG-IgG-positive patients. OBJECTIVE: To investigate the frequency, clinical and paraclinical features, course, outcome, and prognostic implications of brainstem involvement in MOG-IgG-positive ON and/or myelitis. METHODS: Retrospective case study...
November 1, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27796550/hodgkin-s-lymphoma-associated-with-paraneoplastic-cerebellar-degeneration-in-children-a-case-report-and-review-of-the-literature
#11
Serdal Gungor, Betul Kilic, Mujgan Arslan, Unsal Ozgen, Josep Dalmau
Paraneoplastic cerebellar degeneration (PCD) can occur severely and appear as subacute cerebellar syndrome. PCD may be associated with small cell lung cancer, adenocarcinoma, breast cancer, ovarian carcinoma, and Hodgkin's lymphoma. An 11-year-old male was admitted with acute cerebellar ataxia, dysarthria, and diplopia. Mediastinal conglomerated lymph nodes were depicted in a chest computed tomography (CT) examination, and diagnosis of stage IV Hodgkin's lymphoma was obtained after a lymph node biopsy. The antibodies against Purkinje cells (anti-Tr antibody) were positive immunohistochemically...
October 29, 2016: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/27793977/acute-stimulation-of-glucose-influx-upon-mitoenergetic-dysfunction-requires-lkb1-ampk-sirt2-and-mtor-raptor
#12
Dania C Liemburg-Apers, Jori A L Wagenaars, Jan A M Smeitink, Peter H G M Willems, Werner J H Koopman
Mitochondria play a central role in cellular energy production, and their dysfunction can trigger a compensatory increase in glycolytic flux to sustain cellular ATP levels. Here, we studied the mechanism of this homeostatic phenomenon in C2C12 myoblasts. Acute (30 min) mitoenergetic dysfunction induced by the mitochondrial inhibitors piericidin A and antimycin A stimulated Glut1-mediated glucose uptake without altering Glut1 (also known as SLC2A1) mRNA or plasma membrane levels. The serine/threonine liver kinase B1 (LKB1; also known as STK11) and AMP-activated protein kinase (AMPK) played a central role in this stimulation...
December 1, 2016: Journal of Cell Science
https://www.readbyqxmd.com/read/27793206/mog-igg-in-nmo-and-related-disorders-a-multicenter-study-of-50-patients-part-2-epidemiology-clinical-presentation-radiological-and-laboratory-features-treatment-responses-and-long-term-outcome
#13
Sven Jarius, Klemens Ruprecht, Ingo Kleiter, Nadja Borisow, Nasrin Asgari, Kalliopi Pitarokoili, Florence Pache, Oliver Stich, Lena-Alexandra Beume, Martin W Hümmert, Marius Ringelstein, Corinna Trebst, Alexander Winkelmann, Alexander Schwarz, Mathias Buttmann, Hanna Zimmermann, Joseph Kuchling, Diego Franciotta, Marco Capobianco, Eberhard Siebert, Carsten Lukas, Mirjam Korporal-Kuhnke, Jürgen Haas, Kai Fechner, Alexander U Brandt, Kathrin Schanda, Orhan Aktas, Friedemann Paul, Markus Reindl, Brigitte Wildemann
BACKGROUND: A subset of patients with neuromyelitis optica spectrum disorders (NMOSD) has been shown to be seropositive for myelin oligodendrocyte glycoprotein antibodies (MOG-IgG). OBJECTIVE: To describe the epidemiological, clinical, radiological, cerebrospinal fluid (CSF), and electrophysiological features of a large cohort of MOG-IgG-positive patients with optic neuritis (ON) and/or myelitis (n = 50) as well as attack and long-term treatment outcomes. METHODS: Retrospective multicenter study...
September 27, 2016: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/27791036/atm-g6pd-driven-redox-metabolism-promotes-flt3-inhibitor-resistance-in-acute-myeloid-leukemia
#14
Mark A Gregory, Angelo D'Alessandro, Francesca Alvarez-Calderon, Jihye Kim, Travis Nemkov, Biniam Adane, Andrii I Rozhok, Amit Kumar, Vijay Kumar, Daniel A Pollyea, Michael F Wempe, Craig T Jordan, Natalie J Serkova, Aik Choon Tan, Kirk C Hansen, James DeGregori
Activating mutations in FMS-like tyrosine kinase 3 (FLT3) are common in acute myeloid leukemia (AML) and drive leukemic cell growth and survival. Although FLT3 inhibitors have shown considerable promise for the treatment of AML, they ultimately fail to achieve long-term remissions as monotherapy. To identify genetic targets that can sensitize AML cells to killing by FLT3 inhibitors, we performed a genome-wide RNA interference (RNAi)-based screen that identified ATM (ataxia telangiectasia mutated) as being synthetic lethal with FLT3 inhibitor therapy...
October 25, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/27789117/acute-ataxia-in-children-a-review-of-the-differential-diagnosis-and-evaluation-in-the-emergency-department
#15
REVIEW
Mauro Caffarelli, Amir A Kimia, Alcy R Torres
Acute ataxia in a pediatric patient poses a diagnostic dilemma for any physician. While the most common etiologies are benign, occasional individuals require urgent intervention. Children with stroke, toxic ingestion, infection, and neuro-inflammatory disorders frequently exhibit ataxia as an essential-if not the only-presenting feature. The available retrospective research utilize inconsistent definitions of acute ataxia, precluding the ability to pool data from these studies. No prospective data exist that report on patients presenting to the emergency department with ataxia...
December 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/27777786/progressive-cerebellar-degeneration-revealing-primary-sj%C3%A3-gren-syndrome-a-case-report
#16
Emna Farhat, Mourad Zouari, Ines Ben Abdelaziz, Cyrine Drissi, Rahma Beyrouti, Mohamed Ben Hammouda, Fayçal Hentati
BACKGROUND: Cerebellar ataxia represents a rare and severe complication of Sjӧgren syndrome (SS), especially with a progressive onset and cerebellar atrophy on imaging. CASE PRESENTATION: We report the case of a 30-year-old woman, with a past history of dry eyes and mouth, who presented a severe cerebellar ataxia worsening over 4 years associated with tremor of the limbs and the head. Brain MRI showed bilateral hyperintensities on T2 and FLAIR sequences, affecting periventricular white matter, with marked cerebellar atrophy...
2016: Cerebellum & Ataxias
https://www.readbyqxmd.com/read/27770207/gait-disorders-in-adults-and-the-elderly-a%C3%A2-clinical-guide
#17
Walter Pirker, Regina Katzenschlager
Human gait depends on a complex interplay of major parts of the nervous, musculoskeletal and cardiorespiratory systems. The individual gait pattern is influenced by age, personality, mood and sociocultural factors. The preferred walking speed in older adults is a sensitive marker of general health and survival. Safe walking requires intact cognition and executive control. Gait disorders lead to a loss of personal freedom, falls and injuries and result in a marked reduction in the quality of life. Acute onset of a gait disorder may indicate a cerebrovascular or other acute lesion in the nervous system but also systemic diseases or adverse effects of medication, in particular polypharmacy including sedatives...
October 21, 2016: Wiener Klinische Wochenschrift
https://www.readbyqxmd.com/read/27761224/neuro-ophthalmological-manifestations-after-intramuscular-medroxyprogesterone-a-forme-fruste-of-idiopathic-intracranial-hypertension
#18
Mandreker Bahall, Antonio Jose Reyes, Kanterpersad Ramcharan, Nadeem Hosein, Karan Seegobin, Krishni Bahall, Hiranyadeva Sharma, Stephanie Dhansingh, Amanda Mahabir
We report a case of a 22-year-old female student nurse who presented to hospital with an acute neuro-ophthalmological syndrome characterized by papilledema, ataxia, ophthalmoplegia and headache after a single first time use of 150 mg medroxyprogesterone intramuscular injection. Clinical, laboratory, radiological and ophthalmological investigations were in keeping with the diagnosis of idiopathic intracranial hypertension but lumbar puncture did not show a raised cerebrospinal fluid pressure suggesting a forme fruste of this entity...
September 30, 2016: Neurology International
https://www.readbyqxmd.com/read/27680226/fisher-syndrome-with-delayed-facial-weakness-and-taste-impairment-a-case-report
#19
Daisuke Yamamoto, Syuuichirou Suzuki, Bungo Hirose, Minoru Yamada, Masaki Shimizu, Shun Shimohama
A 55-year-old man was admitted to our hospital because of acute onset of diplopia and gait disturbance. On admission, ophthalmoplegia, ataxia and areflexia were observed. He was diagnosed with Fisher syndrome and given intravenous immunoglobulin therapy from day 6 to day 10 after disease onset. After treatment, ophthalmoplegia and ataxia began to improve. However, he developed taste impairment on day 13 and right hemifacial weakness on day 16 after onset. A blink reflex test revealed right facial nerve impairment...
October 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/27673113/poster-360-acute-rehabilitation-of-a-patient-with-stiff-person-syndrome-sps-and-gad-antibody-cerebellar-ataxia-a-case-report
#20
Nicole A Strong, Nicholas F Love, Kristen M Brusky, Sara Salim
No abstract text is available yet for this article.
September 2016: PM & R: the Journal of Injury, Function, and Rehabilitation
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