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https://www.readbyqxmd.com/read/28342959/early-lance-adams-syndrome-after-cardiac-arrest-prevalence-time-to-return-to-awareness-and-outcome-in-a-large-cohort
#1
Irene Aicua Rapun, Jan Novy, Daria Solari, Mauro Oddo, Andrea O Rossetti
INTRODUCTION: Early myoclonus after Cardiac Arrest (CA) is traditionally viewed as a poor prognostic sign (status myoclonus). However, some patients may present early Lance-Adams syndrome (LAS): under appropriate treatment, they can reach a satisfactory functional outcome. Our aim was to describe their profile, focusing on pharmacologic management in the ICU, time to return of awareness, and long-term prognosis. METHODS: Adults with early LAS (defined as generalized myoclonus within 96hours, with epileptiform EEG within 48hours after CA) were retrospectively identified in our CA registry between 2006 and 2016...
March 22, 2017: Resuscitation
https://www.readbyqxmd.com/read/28341199/quantitative-magnetic-resonance-diffusion-weighted-imaging-evaluation-of-the-supratentorial-brain-regions-in-patients-diagnosed-with-brainstem-variant-of-posterior-reversible-encephalopathy-syndrome-a-preliminary-study
#2
Tai-Yuan Chen, Te-Chang Wu, Ching-Chung Ko, I-Jung Feng, Yu-Kun Tsui, Chien-Jen Lin, Jeon-Hor Chen, Ching-Po Lin
BACKGROUND AND PURPOSE: Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic entity with several causes, characterized by rapid onset of symptoms and typical neuroimaging features, which usually resolve if promptly recognized and treated. Brainstem variant of PRES presents with vasogenic edema in brainstem regions on magnetic resonance (MR) images and there is sparing of the supratentorial regions. Because PRES is usually caused by a hypertensive crisis, which would likely have a systemic effect and global manifestations on the brain tissue, we thus proposed that some microscopic abnormalities of the supratentorial regions could be detected with diffusion-weighted imaging (DWI) using apparent diffusion coefficient (ADC) analysis in brainstem variant of PRES and hypothesized that "normal-looking" supratentorial regions will increase water diffusion...
March 21, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28337694/the-posterior-fossa-and-foreign-accent-syndrome-report-of-two-new-cases-and-review-of-the-literature
#3
Stefanie Keulen, Peter Mariën, Kim van Dun, Roelien Bastiaanse, Mario Manto, Jo Verhoeven
Foreign accent syndrome is a rare motor speech disorder that causes patients to speak their language with a non-native accent. In the neurogenic condition, the disorder develops after lesions in the language dominant hemisphere, often affecting Broca's area, the insula, the supplementary motor area and the primary motor cortex. Here, we present two new cases of FAS after posterior fossa lesions. The first case is a 44-year-old, right-handed, Dutch-speaking man who suffered motor speech disturbances and a left hemiplegia after a pontine infarction...
March 23, 2017: Cerebellum
https://www.readbyqxmd.com/read/28337123/analysis-of-the-serotonergic-system-in-a-mouse-model-of-rett-syndrome-reveals-unusual-upregulation-of-serotonin-receptor-5b
#4
Steffen Vogelgesang, Sabine Niebert, Ute Renner, Wiebke Möbius, Swen Hülsmann, Till Manzke, Marcus Niebert
Mutations in the transcription factor methyl-CpG-binding-protein 2 (MeCP2) cause a delayed-onset neurodevelopmental disorder known as Rett syndrome (RTT). Although alteration in serotonin levels have been reported in RTT patients, the molecular mechanisms underlying these defects are not well understood. Therefore, we chose to investigate the serotonergic system in hippocampus and brainstem of male Mecp2(-/y) knock-out mice in the B6.129P2(C)-Mecp2(tm1.1Bird) mouse model of RTT. The serotonergic system in mouse is comprised of 16 genes, whose mRNA expression profile was analyzed by quantitative RT-PCR...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28334875/white-matter-changes-in-paediatric-multiple-sclerosis-and-monophasic-demyelinating-disorders
#5
Giulia Longoni, Robert A Brown, Parya MomayyezSiahkal, Colm Elliott, Sridar Narayanan, Amit Bar-Or, Ruth Ann Marrie, E Ann Yeh, Massimo Filippi, Brenda Banwell, Douglas L Arnold
Most children who experience an acquired demyelinating syndrome of the central nervous system will have a monophasic disease course, with no further clinical or radiological symptoms. A subset will be diagnosed with multiple sclerosis, a life-long disorder. Using linear mixed effects models we examined longitudinal diffusion properties of normal-appearing white matter in 505 serial scans of 132 paediatric participants with acquired demyelinating syndromes followed for a median of 4.4 years, many from first clinical presentation, and 106 scans of 80 healthy paediatric participants...
March 14, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28330972/fatal-cerebral-edema-with-status-epilepticus-in-children-with-dravet-syndrome-report-of-5-cases
#6
Kenneth A Myers, Jacinta M McMahon, Simone A Mandelstam, Mark T Mackay, Renate M Kalnins, Richard J Leventer, Ingrid E Scheffer
Dravet syndrome (DS) is a well-recognized developmental and epileptic encephalopathy associated with SCN1A mutations and 15% mortality by 20 years. Although over half of cases succumb to sudden unexpected death in epilepsy, the cause of death in the remainder is poorly defined. We describe the clinical, radiologic, and pathologic characteristics of a cohort of children with DS and SCN1A mutations who developed fatal cerebral edema causing mass effect after fever-associated status epilepticus. Cases were identified from a review of children with DS enrolled in the Epilepsy Genetics Research Program at The University of Melbourne, Austin Health, who died after fever-associated status epilepticus...
March 22, 2017: Pediatrics
https://www.readbyqxmd.com/read/28324264/reverse-locked-in-syndrome
#7
Pooja Raibagkar, Ram V Chavali, Tamara B Kaplan, Jennifer A Kim, Meaghan V Nitka, Sherry H-Y Chou, Brian L Edlow
BACKGROUND: Basilar artery occlusion can cause locked-in syndrome, which is characterized by quadriplegia, anarthria, and limited communication via eye movements. Here, we describe an uncommon stroke syndrome associated with endovascular recanalization of the top of the basilar artery: "reverse locked-in syndrome." METHODS: We report the case of a patient with atypical neurological deficits caused by acute ischemic stroke of the midbrain tegmentum. We perform neuroanatomic localization of the patient's infarcts by mapping the magnetic resonance imaging (MRI) data onto a brainstem atlas...
March 21, 2017: Neurocritical Care
https://www.readbyqxmd.com/read/28318543/obesity-induced-neuroinflammation-beyond-the-hypothalamus
#8
REVIEW
Owein Guillemot-Legris, Giulio G Muccioli
Obesity is now a worldwide health issue. Far from being limited to weight gain, obesity is generally associated with low-grade inflammation and with a cluster of disorders collectively known as the 'metabolic syndrome'. When considering obesity and the subsequent neuroinflammation, the focus was long set on the hypothalamus. More recently, obesity-derived neuroinflammation has been shown to affect other brain structures such as the hippocampus, cortex, brainstem, or amygdala. Furthermore, obesity has been associated with increased occurrence of central disorders such as depression and impaired cognitive function...
March 16, 2017: Trends in Neurosciences
https://www.readbyqxmd.com/read/28318060/integrating-postural-and-vestibular-dimensions-to-depict-impairment-in-moderate-to-severe-obstructive-sleep-apnea-syndrome-patients
#9
Alessandro Micarelli, Claudio Liguori, Andrea Viziano, Francesca Izzi, Fabio Placidi, Marco Alessandrini
Vestibular dysfunction was linked to moderate-to-severe obstructive sleep apnea syndrome (OSAS) patients in literature. However, due to a lack of knowledge among valid and recent implementations conceived to study postural control on static posturography (SP) and vestibulo-ocular reflex (VOR) gain under physiological conditions (video Head Impulse Test; vHIT), the aim of this work was to integrate (i) VOR changes via vHIT implementation, (ii) postural arrangement by studying both classical parameters and frequency spectra (PS) and (iii) correlation between these findings, polygraphic (PG) and subjective scores along Dizziness Handicap Inventory (DHI) and Epworth Sleepiness Scale (ESS)...
March 20, 2017: Journal of Sleep Research
https://www.readbyqxmd.com/read/28314977/isolated-vestibular-syndromes-due-to-brainstem-and-cerebellar-lesions
#10
Sung-Hee Kim, Hyo Jung Kim, Ji-Soo Kim
Dizziness/vertigo is the most common symptoms of posterior circulation strokes. Isolated vestibular symptoms and signs without other neurologic deficits have been found in infarctions involving the brainstem and cerebellum. In the brainstem, infarctions responsible for isolated vestibular syndrome are usually restricted to the dorsal portion that contains the vestibular nucleus and the nucleus prepositus hypoglossi. Cerebellar lesions confined to the flocculus, tonsil, and nodulus also produce isolated vertigo and imbalance...
March 17, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28283819/neurological-complications-of-beh%C3%A3-et-s-syndrome
#11
D P Kidd
In this review of the neurological complications of Behçet's syndrome, the clinical features and epidemiology of the systemic disease are summarised before a discussion of the neurological syndromes which may develop is made. Neurological involvement occurs in 9% of cases, and is equally prevalent in each geographical area. Vascular complications occur in 14%, in whom thrombosis of the venous sinuses or cerebral veins occurs, and intracranial hypertension, venous infarction and parenchymal haemorrhage may develop...
March 10, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28275351/botulinum-toxin-is-effective-in-the-management-of-neurogenic-dysphagia-clinical-electrophysiological-findings-and-tips-on-safety-in-different-neurological-disorders
#12
Enrico Alfonsi, Domenico A Restivo, Giuseppe Cosentino, Roberto De Icco, Giulia Bertino, Antonio Schindler, Massimiliano Todisco, Mauro Fresia, Andrea Cortese, Paolo Prunetti, Matteo C Ramusino, Arrigo Moglia, Giorgio Sandrini, Cristina Tassorelli
Background and Aims: Neurogenic dysphagia linked to failed relaxation of the upper esophageal sphincter (UES) can be treated by injecting botulinum toxin (BTX) into the cricopharyngeal (CP) muscle. We compared the effects of this treatment in different neurological disorders with dysphagia, to evaluate its efficacy over time including the response to a second injection. Materials and Methods: Sixty-seven patients with neurogenic dysphagia associated with incomplete or absent opening of the UES (24 with brainstem or hemispheric stroke, 21 with parkinsonian syndromes, 12 with multiple sclerosis, and 10 with spastic-dystonic syndromes secondary to post-traumatic encephalopathy) were treated with the injection of IncobotulinumtoxinA (dose 15-20 U) into the CP muscle under electromyographic guidance...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28273704/-clinical-and-genetic-characteristics-of-children-with-leigh-syndrome
#13
F Fang, Y Shen, D M Shen, Z M Liu, C H Ding, W C Zhang, S Z Sun, J L Lyu, T L Han, X H Wang, W H Zhang, X Y Yang, J W Li, H S Wu
Objective: To investigate the clinically and genetic characteristics of children with Leigh syndrome. Method: Patients with clinically diagnosed Leigh syndrome(LS)in the department of Neurology, Beijing Children's Hospital from January 2013 to February 2016 underwent the mitochondrial DNA (mtDNA) and nuclear DNA (nDNA) detecting with next generation sequencing (NGS) technology. The clinical data of gene confirmed cases were retrospectively collected and analyzed. The differences in the onset age, clinical manifestations, lactic acid level and MRI results between the mtDNA variation and nDNA variation were compared and analyzed...
March 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28272727/-an-unusual-presentation-of-painful-tic-convulsive
#14
S Romano-Feinholz, M P Medina, F E Nares-Lopez, J L Soto-Hernandez, R Revuelta-Gutierrez
INTRODUCTION: The term 'painful tic convulsive' is used to describe the syndrome involving concomitant hemifacial spasm and ipsilateral trigeminal neuralgia. Vascular compression of the fifth and seventh cranial nerves is the most common cause, involving the entry and exit zone of rootlets coming from the brainstem; nevertheless, different etiologies of this syndrome has been previously reported. Treatment for this disease is based on surgical microvascular decompression of the nerve rootlets, but still a topic of debate...
March 16, 2017: Revista de Neurologia
https://www.readbyqxmd.com/read/28270749/identifying-candidate-genes-that-underlie-cellular-ph-sensitivity-in-serotonin-neurons-using-transcriptomics-a-potential-role-for-kir5-1-channels
#15
Madeleine M Puissant, Gary C Mouradian, Pengyuan Liu, Matthew R Hodges
Ventilation is continuously adjusted by a neural network to maintain blood gases and pH. Acute CO2 and/or pH regulation requires neural feedback from brainstem cells that encode CO2/pH to modulate ventilation, including but not limited to brainstem serotonin (5-HT) neurons. Brainstem 5-HT neurons modulate ventilation and are stimulated by hypercapnic acidosis, the sensitivity of which increases with increasing postnatal age. The proper function of brainstem 5-HT neurons, particularly during post-natal development is critical given that multiple abnormalities in the 5-HT system have been identified in victims of Sudden Infant Death Syndrome...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28258082/dppx-antibody-associated-encephalitis-main-syndrome-and-antibody-effects
#16
Makoto Hara, Helena Ariño, Mar Petit-Pedrol, Lidia Sabater, Maarten J Titulaer, Eugenia Martinez-Hernandez, Marco W J Schreurs, Myrna R Rosenfeld, Francesc Graus, Josep Dalmau
OBJECTIVE: To report the main syndrome of dipeptidyl-peptidase-like protein 6 (DPPX) antibody-associated encephalitis, immunoglobulin G (IgG) subclass, and the antibody effects on DPPX/Kv4.2 potassium channels. METHODS: A retrospective analysis of new patients and cases reported since 2013 was performed. IgG subclass and effects of antibodies on cultured neurons were determined with described techniques. RESULTS: Nine new patients were identified (median age 57 years, range 36-69 years)...
March 3, 2017: Neurology
https://www.readbyqxmd.com/read/28256369/anti-hu-paraneoplastic-brainstem-encephalitis-caused-by-a-pancreatic-neuroendocrine-tumor-presenting-with-central-hypoventilation
#17
Marc Najjar, Andrew Taylor, Surbhi Agrawal, Tito Fojo, Alexander E Merkler, Marc K Rosenblum, Laura Lennihan, Michael D Kluger
Paraneoplastic neurological syndromes are rare autoimmune manifestations of malignancies associated with specific antibodies. Anti-Hu associated brainstem encephalitis, a well-described syndrome, usually presents subacutely with preferential involvement of the medulla. Anti-Hu antibodies target intraneuronal antigens and are therefore highly correlated with neurological syndromes when present concomitantly with a neoplasm. Reported is a case of anti-Hu brainstem encephalitis associated with a pancreatic neuroendocrine tumor (PNET) presenting with central hypoventilation...
February 27, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28247337/widening-the-heterogeneity-of-leigh-syndrome-clinical-biochemical-and-neuroradiologic-features-in-a-patient-harboring-a-ndufa10-mutation
#18
Francesca Minoia, Marta Bertamino, Paolo Picco, Mariasavina Severino, Andrea Rossi, Chiara Fiorillo, Carlo Minetti, Claudia Nesti, Filippo Maria Santorelli, Maja Di Rocco
Leigh syndrome (LS) is an early-onset progressive neurodegenerative disorder, characterized by a wide clinical and genetic heterogeneity, and is the most frequent disorder of mitochondrial energy production in children. Beside its great variability in clinical, biochemical, and genetic features, LS is pathologically uniformly characterized by multifocal bilateral and symmetric spongiform degeneration of the basal ganglia, brainstem, thalamus, cerebellum, spinal cord, and optic nerves. Isolated complex I deficiency is the most common defect identified in Leigh syndrome...
March 1, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28243339/various-imaging-manifestations-of-posterior-reversible-encephalopathy-syndrome-pres-on-magnetic-resonance-imaging-mri
#19
Rajesh Raman, Radhika Devaramane, Geetha Mukunda Jagadish, Sanjana Chowdaiah
BACKGROUND: Posterior reversible encephalopathy syndrome (PRES), also called the acute hypertensive encephalopathy and reversible posterior leukoencephalopathy syndrome (RPLS), is a neurotoxic syndrome of cerebral vasoregulation classically characterized by bilaterally symmetrical parieto-occipital edema. However, the imaging findings are variable and may occur in other locations such as the frontal lobes, thalami, basal ganglia and brainstem. Most commonly, PRES presents with hyperintense signals on T2 and FLAIR sequences...
2017: Polish Journal of Radiology
https://www.readbyqxmd.com/read/28229064/abnormal-auditory-brainstem-response-abr-findings-in-a-near-normal-hearing-child-with-noonan-syndrome
#20
Bahram Jalaei, Mohd Normani Zakaria, Dinsuhaimi Sidek
INTRODUCTION: Noonan syndrome (NS) is a heterogeneous genetic disease that affects many parts of the body. It was named after Dr. Jacqueline Anne Noonan, a paediatric cardiologist. CASE REPORT: We report audiological tests and auditory brainstem response (ABR) findings in a 5-year old Malay boy with NS. Despite showing the marked signs of NS, the child could only produce a few meaningful words. Audiological tests found him to have bilateral mild conductive hearing loss at low frequencies...
January 2017: Iranian Journal of Otorhinolaryngology
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