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https://www.readbyqxmd.com/read/28612899/recurrent-miller-fisher-a-new-case-report-and-a-literature-review
#1
F Barbato, A Di Paolantonio, M Distefano, A Mastrorosa, M Sabatelli, S Servidei, M Luigetti
Miller Fisher syndrome (MFS) is considered to be an uncommon variant of Guillain-Barré Syndrome. The disease is clinically characterized by acute ataxia of limbs, areflexia and ophthalmoplegia, although the set of symptoms and signs can be quite heterogeneous, with a benign and monophasic course. We describe a case of recurrent MFS where there have been four clinical episodes occurred with complete remission after each relapse. Last recurrence was treated with oral steroids. The reported frequency of recurrent MFS in literature is variable as well as the best treatment in these cases...
May 2017: La Clinica Terapeutica
https://www.readbyqxmd.com/read/28606112/acute-cerebellitis-in-children-an-eleven-year-retrospective-multicentric-study-in-italy
#2
Laura Lancella, Susanna Esposito, Maria Luisa Galli, Elena Bozzola, Valeria Labalestra, Elena Boccuzzi, Andrzej Krzysztofiak, Laura Cursi, Guido Castelli Gattinara, Nadia Mirante, Danilo Buonsenso, Claudia Tagliabue, Luca Castellazzi, Carlotta Montagnani, Chiara Tersigni, Piero Valentini, Michele Capozza, Davide Pata, Maria Di Gangi, Piera Dones, Silvia Garazzino, Luca Baroero, Alberto Verrotti, Maria Luisa Melzi, Michele Sacco, Michele Germano, Filippo Greco, Elena Uga, Giovanni Crichiutti, Alberto Villani
BACKGROUND: Acute cerebellitis (AC) and acute cerebellar ataxia (ACA) are the principal causes of acute cerebellar dysfunction in childhood. Nevertheless. there is no accepted consensus regarding the best management of children with AC/ACA: the aim of the study is both to assess clinical, neuroimaging and electrophysiologic features of children with AC/ACA and to evaluate the correlation between clinical parameters, therapy and outcome. METHODS: A multicentric retrospective study was conducted on children ≤ 18 years old admitted to 12 Italian paediatric hospitals for AC/ACA from 01/01/2003 to 31/12/2013...
June 12, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28601293/from-dizziness-to-severe-ataxia-and-dysarthria-new-cases-of-anti-ca-arhgap26-autoantibody-associated-cerebellar-ataxia-suggest-a-broad-clinical-spectrum
#3
Ulrike Wallwitz, Sebastian Brock, Antje Schunck, Brigitte Wildemann, Sven Jarius, Frank Hoffmann
In 2010, a novel anti-neuronal autoantibody, termed anti-Ca, was described in a patient with subacute cerebellar ataxia, and Rho GTPase-activating protein 26 (ARHGAP26) was identified as the target antigen. Recently, three additional cases of anti-Ca-positive cerebellar ataxia have been published. In addition to ataxia, cognitive decline and depression have been observed in some patients. Here, we report two new cases of anti-Ca-associated autoimmune cerebellar ataxia. Patient 1 presented with dizziness and acute yet mild limb and gait ataxia...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28566787/acute-flaccid-paraparesis-cauda-equina-syndrome-in-a-patient-with-bardet-biedl-syndrome
#4
Vibhu Krishnan Viswanathan, Rishi Mugesh Kanna, Ajoy Prasad Shetty, S Rajasekaran
Bardet-Biedl syndrome (BBS) is a rare, autosomal-recessive, debilitating genetic disorder, which can present with multitudinous systemic clinical features including rod-cone dystrophy, polydactyly, Frohlich-like central obesity, mental retardation, hypogonadism, and renal anomalies. Diverse neuromuscular manifestations in patients afflicted by this heterogeneous disorder include ataxia, cervical, and thoracic canal stenoses, presenting as spastic quadriparesis and other gait disturbances. We report a young patient with BBS, who had presented with acute flaccid paraparesis due to severe primary lumbar canal stenosis...
May 2017: Indian Journal of Orthopaedics
https://www.readbyqxmd.com/read/28556429/acute-nutritional-axonal-neuropathy
#5
Johanna Hamel, Eric L Logigian
INTRODUCTION: This study describes clinical, laboratory, and electrodiagnostic features of a severe acute axonal polyneuropathy common to patients with acute nutritional deficiency in the setting of alcoholism, bariatric surgery (BS), or anorexia. METHODS: Retrospective analysis of clinical, electrodiagnostic, and laboratory data of patients with acute axonal neuropathy. RESULTS: Thirteen patients were identified with a severe, painful, sensory or sensorimotor axonal polyneuropathy that developed over 2-12 weeks with sensory ataxia, areflexia, variable muscle weakness, poor nutritional status, and weight loss, often with prolonged vomiting and normal cerebrospinal fluid protein...
May 26, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28554905/clinical-reasoning-a-27-year-old-man-with-acute-onset-ataxia
#6
Jorge Risco, Menachem Weiss
No abstract text is available yet for this article.
May 30, 2017: Neurology
https://www.readbyqxmd.com/read/28549713/childhood-opsoclonus-myoclonus-syndrome-a-case-series-from-tunisia
#7
Nedia Ben Achour, Saloua Mrabet, Ibtihel Rebai, Ines Abid, Hanene Benrhouma, Hedia Klaa, Aida Rouissi, Ichraf Kraoua, Ilhem Ben Youssef Turki
INTRODUCTION: Opsoclonus myoclonus syndrome (OMS) is a rare immune-mediated disorder characterized by opsoclonus, myoclonus, ataxia and behavioral changes. The aim of our study was to investigate the epidemiology, clinical features, etiological aspects and outcome of OMS in Tunisian children. METHODS: We conducted a retrospective study over 11years (2005-2016) including all patients aged under 18years who were managed for newly diagnosed OMS in a tertiary care research centre for children with neurological symptoms...
May 23, 2017: Brain & Development
https://www.readbyqxmd.com/read/28536821/impaired-motor-coordination-and-learning-in-mice-lacking-anoctamin-2-calcium-gated-chloride-channels
#8
Franziska Neureither, Katharina Ziegler, Claudia Pitzer, Stephan Frings, Frank Möhrlen
Neurons communicate through excitatory and inhibitory synapses. Both lines of communication are adjustable and allow the fine tuning of signal exchange required for learning processes in neural networks. Several distinct modes of plasticity modulate glutamatergic and GABAergic synapses in Purkinje cells of the cerebellar cortex to promote motor control and learning. In the present paper, we present evidence for a role of short-term ionic plasticity in the cerebellar circuit activity. This type of plasticity results from altered chloride driving forces at the synapses that molecular layer interneurons form on Purkinje cell dendrites...
May 23, 2017: Cerebellum
https://www.readbyqxmd.com/read/28515935/methotrexate-encephalopathy-two-cases-in-adult-cancer-patients-who-recovered-with-pathophysiologically-based-therapy
#9
Shodeinde A Coker, David A Pastel, Melissa C Davis, Elizabeth M Bengtson, Camilo E Fadul, Lionel D Lewis
BACKGROUND/OBJECTIVES: Neurotoxicity is a serious and sometimes fatal adverse effect that can occur following methotrexate treatment. We describe two adult patients with hematological malignancies with methotrexate encephalopathy who recovered with dextromethorphan therapy. RESULTS: Case 1: A 24-year-old male with acute lymphoblastic leukemia developed the acute onset of bilateral facial weakness and slurred speech after his first treatment with high-dose intravenous methotrexate...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28500261/osmotic-demyelination-syndrome-in-type-1-diabetes-in-the-absence-of-dyselectrolytaemia-an-overlooked-complication
#10
Chandramohan Sharma, Banshi Lal Kumawat, Maulik Panchal, Mohit Shah
Central pontine myelinolysis (CPM) is a demyelinating disorder of central nervous system which involves central portion of the pons and sometimes extrapontine areas also. It is commonly reported in settings of hyponatraemia or its rapid correction, but in the last few years it has also been reported in patients with diabetes in the absence of electrolyte disturbances or correction of serum osmolality. Here we report a case of a 20-year-old female patient, with a known history of type 1 diabetes mellitus, who presented with acute onset spastic quadriparesis with dysarthria and mild ataxia which evolved over 2 weeks...
May 12, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28484839/plasma-exchanges-for-severe-acute-neurological-deterioration-in-patients-with-igm-anti-myelin-associated-glycoprotein-anti-mag-neuropathy
#11
M Baron, P Lozeron, S Harel, D Bengoufa, M Vignon, B Asli, M Malphettes, N Parquet, A Brignier, J P Fermand, N Kubis, Bertrand Arnulf
Monoclonal IgM anti-myelin-associated glycoprotein (MAG) antibody-related peripheral neuropathy (anti-MAG neuropathy) is predominantly a demyelinating sensory neuropathy with ataxia and distal paresthesia. The clinical course of anti-MAG neuropathy is usually slowly progressive making difficult the identification of clear criteria to start a specific treatment. Although no consensus treatment is yet available, a rituximab-based regimen targeting the B-cell clone producing the monoclonal IgM may be proposed, alone or in combination with alkylating agents or purine analogs...
June 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28483396/early-diagnosis-of-capos-syndrome-before-acute-onset-ataxia-review-of-the-literature-and-a-new-family
#12
Anna Duat Rodriguez, Michaela Prochazkova, Saturnino Santos Santos, Oscar Rubio Cabezas, Veronica Cantarin Extremera, Luis Gonzalez-Gutierrez-Solana
BACKGROUND: CAPOS syndrome (cerebellar ataxia, areflexia, pes cavus, optic atrophy, and sensorineural hearing loss) is a rare disease that has been reported in 22 patients so far. In all cases, the mutation c.2452G>A (p.Glu818Lys) in the ATP1A3 gene was identified. Patients typically present at an early age with an acute-onset fever-induced episode of ataxia frequently associated with encephalopathy and weakness. They usually present one to three episodes. The acute symptoms improve within days, but most patients show slow progression afterward...
June 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28460866/an-unusual-presentation-of-varicella-zoster-virus-with-acute-cerebellitis-and-siadh-without-a-rash
#13
Michal Lubomski, Lauren Brown, Romesh Markus
We report a case of varicella-zoster virus (VZV) infection with acute cerebellitis and encephalitis with associated Syndrome of Inappropriate Antidiuretic Hormone Secretion (SIADH) in an elderly man presenting with acute cerebellar ataxia without antecedent rash. Cerebrospinal fluid examination (CSF) revealed a mononuclear pleocytosis, high protein, normal glucose, positive for VZV polymerase chain reaction (PCR). Early acyclovir treatment is beneficial for acute VZV cerebellitis. Clinicians should consider infectious Central Nervous System (CNS) causes for presentations of acute cerebellar ataxia in adult patients, particularly if there is an accompanying clouded sensorium...
July 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28460008/the-spectrum-and-burden-of-influenza-associated-neurological-disease-in-children-combined-encephalitis-and-influenza-sentinel-site-surveillance-from-australia-2013-2015
#14
P N Britton, C C Blyth, K Macartney, R C Dale, J Li-Kim-Moy, G Khandaker, N Crawford, H Marshall, J Clark, E Elliott, R Booy, A C Cheng, C A Jones
Background.: There are few longitudinal studies of seasonal influenza associated neurological disease (IAND) and none from the Southern hemisphere. Methods.: We extracted prospectively acquired Australian surveillance data from two studies nested within the Paediatric Active Enhanced Disease Surveillance (PAEDS) network: the Influenza Complications Alert Network (FluCAN) study and the Australian Childhood Encephalitis (ACE) study between 2013 and 2015. We described the clinical features and severity of IAND in children, including influenza associated encephalitis/encephalopathy (IAE)...
April 29, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28457669/%C3%AE-6%C3%AE-2-nicotinic-acetylcholine-receptors-influence-locomotor-activity-and-ethanol-consumption
#15
Helen M Kamens, Colette Peck, Caitlin Garrity, Alex Gechlik, Brenita C Jenkins, Akshat Rajan
Nicotinic acetylcholine receptors (nAChRs) in the mesolimbic dopamine system have been implicated in ethanol behaviors. In particular, work in genetically engineered mice has demonstrated that α6-containing nAChRs are involved in ethanol consumption and sedation. A limitation of these studies is that the alteration in the receptor was present throughout development. The recently described α6β2 antagonist, N,N-decane-1,10-diyl-bis-3-picolinium diiodide (bPiDI), now makes it possible to test for the involvement of these receptors using a pharmacological approach...
June 2017: Alcohol
https://www.readbyqxmd.com/read/28457486/vaccination-against-louping-ill-virus-protects-goats-from-experimental-challenge-with-spanish-goat-encephalitis-virus
#16
L M Salinas, R Casais, J F García Marín, K P Dalton, L J Royo, A Del Cerro, E Gayo, M P Dagleish, P Alberdi, R A Juste, J de la Fuente, A Balseiro
Spanish goat encephalitis virus (SGEV) is a recently described member of the genus Flavivirus belonging to the tick-borne encephalitis group of viruses, and is closely related to louping ill virus (LIV). Naturally acquired disease in goats results in severe, acute encephalitis and 100% mortality. Eighteen goats were challenged subcutaneously with SGEV; nine were vaccinated previously against LIV and nine were not. None of the vaccinated goats showed any clinical signs of disease or histological lesions, but all of the non-vaccinated goats developed pyrexia and 5/9 developed neurological clinical signs, primarily tremors in the neck and ataxia...
April 28, 2017: Journal of Comparative Pathology
https://www.readbyqxmd.com/read/28446490/complicated-acute-cerebellitis-with-obstructive-hydrocephalus-and-tonsillar-herniation-in-a-child
#17
Manchikanti Venkatesh, Sunitha Vellathussery Chakkalakkoombil, Manju Bashini Duraipandi, Reena Gulati
Acute cerebellitis (AC) is a rare inflammatory syndrome presenting as cerebellar dysfunction, seen more frequently in children. AC can have a variable course with features of cerebellar dysfunction, raised intracranial pressure and neurological deficits, and can sometimes even be potentially fatal due to complications such as obstructive hydrocephalus and brainstem compression, warranting surgical intervention. We report a case of a 12-year-old boy who presented with raised intracranial pressure and ataxia...
April 26, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28438663/acute-effects-of-amitriptyline-on-adult-zebrafish-potential-relevance-to-antidepressant-drug-screening-and-modeling-human-toxidromes
#18
Konstantin A Demin, Tatiana O Kolesnikova, Sergey L Khatsko, Darya M Meshalkina, Evgeniya V Efimova, Yuri Yu Morzherin, Allan V Kalueff
The need to develop novel antidepressants is an emerging problem in biomedicine. An aquatic vertebrate species, the zebrafish (Danio rerio) may serve as a useful in-vivo screen for CNS drugs, and displays high sensitivity to a wide range of antidepressants. Amitriptyline is a commonly used tricyclic antidepressant which acts primarily as a serotonin and noradrenaline reuptake inhibitor. Here, we characterize drug-induced behavioral and neurochemical responses in adult zebrafish following their acute exposure to amitriptyline...
April 21, 2017: Neurotoxicology and Teratology
https://www.readbyqxmd.com/read/28432174/postintervention-acute-opsoclonus-myoclonus-syndrome
#19
Annelise Aquilina, Nicola Dingli, Josanne Aquilina
Opsoclonus myoclonus syndrome (OMS) is a rare, neurological condition affecting 1 in 10 000 000 people annually. Opsoclonus, defined as involuntary rapid, multivectorial oscillations of the eyes, together with ataxia and myoclonus are usually present. OMS may be paraneoplastic: often associated with occult neuroblastoma in childhood and with breast carcinoma or small cell lung carcinoma in adults. Other aetiologies include viral or toxic agents. The pathogenesis is thought to be immune mediated. A 37-year-old woman with previous inflammatory cranial mononeuropathies was admitted for elective dilatation and curettage (D&C)...
April 21, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28431624/opsoclonus-myoclonus-ataxia-associated-with-west-nile-virus-infection-a-dramatic-presentation-with-benign-prognosis
#20
Roy Zaltzman, Colin Klein, Carlos R Gordon
Opsoclonus myoclonus and ataxia is a combination of severe neurological signs associated with several pathologic agents and conditions. Only few cases of opsoclonus have been related to West Nile virus infection. We report on a 61-year-old woman and on a 55-year-old man who had history of recent fever, who were hospitalized because of acute severe truncal ataxia, opsoclonus and tremor with minimal myoclonic jerks. A through work-up revealed the presence of both IgM and IgG antibodies against West Nile virus both in the serum and Cerebrospinal Fluid and excluded other causes known to be associated with this combination of neurological signs...
May 15, 2017: Journal of the Neurological Sciences
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