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https://www.readbyqxmd.com/read/29197651/yawning-its-anatomy-chemistry-role-and-pathological-considerations
#1
REVIEW
Heinz Krestel, Claudio L Bassetti, Olivier Walusinski
Yawning is a clinical sign of the activity of various supra- and infratentorial brain regions including the putative brainstem motor pattern, hypothalamic paraventricular nucleus, probably the insula and limbic structures that are interconnected via a fiber network. This interaction can be seen in analogy to other cerebral functions arising from a network or zone such as language. Within this network, yawning fulfills its function in a stereotype, reflex-like manner; a phylogenetically old function, preserved across species barriers, with the purpose of arousal, communication, and maybe other functions including respiration...
November 29, 2017: Progress in Neurobiology
https://www.readbyqxmd.com/read/29189299/global-orientation-in-space-and-the-lateralization-of-brain-functions
#2
Marianne Dieterich, Thomas Brandt
PURPOSE OF REVIEW: The functional role of the vestibular system for multisensory orientation and sensorimotor control is reviewed with a special focus on hemispherical lateralization and its consequences for neurological disorders of higher cortical function. RECENT FINDINGS: The peripheral and central vestibular systems are bilaterally organized with ipsilateral and contralateral ascending pathways and two multisensory cortical networks in the right and left hemisphere...
November 20, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/29180620/quantitative-measurement-of-post-concussion-syndrome-using-electrovestibulography
#3
Abdelbaset Suleiman, Brian Lithgow, Zeinab Dastghieb, Behzad Mansouri, Zahra Moussavi
In this study, a noninvasive quantitative measure was used to identify short and long term post-concussion syndrome (PCS) both from each other and from healthy control populations. We used Electrovestibulography (EVestG) for detecting neurophysiological PCS consequent to a mild traumatic brain injury (mTBI) in both short-term (N = 8) and long-term (N = 30) (beyond the normal recovery period) symptomatic individuals. Peripheral, spontaneously evoked vestibuloacoustic signals incorporating - and modulated by - brainstem responses were recorded using EVestG, while individuals were stationary (no movement stimulus)...
November 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29168586/regional-oligodendrocytopathy-and-astrocytopathy-precede-myelin-loss-and-blood-brain-barrier-disruption-in-a-murine-model-of-osmotic-demyelination-syndrome
#4
Joanna Bouchat, Bruno Couturier, Catherine Marneffe, Fabrice Gankam-Kengne, Benoît Balau, Kathleen De Swert, Jean-Pierre Brion, Luc Poncelet, Jacques Gilloteaux, Charles Nicaise
The osmotic demyelination syndrome (ODS) is a non-primary inflammatory disorder of the central nervous system myelin that is often associated with a precipitous rise of serum sodium concentration. To investigate the physiopathology of ODS in vivo, we generated a novel murine model based on the abrupt correction of chronic hyponatremia. Accordingly, ODS mice developed impairments in brainstem auditory evoked potentials and in grip strength. At 24 hr post-correction, oligodendrocyte markers (APC and Cx47) were downregulated, prior to any detectable demyelination...
November 23, 2017: Glia
https://www.readbyqxmd.com/read/29146727/primary-intraventricular-haemorrhage-due-to-rupture-of-giant-varix-of-the-basal-vein-of-rosenthal-in-a-patient-with-long-standing-direct-ccf-angiographic-features-and-treatment-considerations
#5
Chinmay P Nagesh, Aneesh Mohimen, Santhosh K Kannath, Jayadevan E Rajan
Direct carotid cavernous fistulae (CCF) are often detected early and treated promptly resulting in a paucity of literature regarding its long-term evolution. We present a case of high flow post-traumatic direct CCF that was neglected for over 6 years and presented with a rare manifestation of primary intraventricular haemorrhage. Occlusions of the primary venous outlets likely resulted in engorgement of the deep venous system. The segmental anatomy of the shunting basal vein is critical to the clinical presentation and may range from basal ganglia or brainstem oedema/infarctions to uniquely, as in our case, isolated intraventricular haemorrhage secondary to variceal rupture...
November 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29137102/lateral-medullary-infarction-with-similar-features-of-brown-sequard-syndrome-caused-by-vertebrobasilar-dysplasia-and-klippel-feil-syndrome-a-case-report
#6
Jingzhe Han, Duanhua Cao, Guomei Ma, Tingting Wang, Ye Ji, Zhilei Kang
RATIONALE: Patients with Klippel-Feil syndrome (KFS) are always anomaly associated with vertebrobasilar dysplasia. That may present commonly as infarction of brainstem, medulla, and cerebellum. In this article, we reported a rare case of lateral medullary infarction (LMI) with similar features of Brown Sequard syndrome caused by vertebrobasilar dysplasia and KFS, and the 2 rare conditions that are causally related. The case is being reported because of its unusual and rare presentation...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29133062/anatomical-and-physiological-basis-of-continuous-spike-wave-of-sleep-syndrome-after-early-thalamic-lesions
#7
Alberto Leal, Eulália Calado, José P Vieira, Carla Mendonça, José C Ferreira, Hugo Ferreira, Daniel Carvalho, Fátima Furtado, Roseli Gomes, José P Monteiro
OBJECTIVE: Early neonatal thalamic lesions account for about 14% of continuous spike-wave of sleep (CSWS) syndrome, representing the most common etiology in this epileptic encephalopathy in children, and promise useful insights into the pathophysiology of the disease. METHODS: We describe nine patients with unilateral neonatal thalamic lesions which progressed to CSWS. Longitudinal whole-night and high-density electroencephalograms (EEGs) were performed, as well as detailed imaging and clinical evaluation...
November 10, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29124438/reorganization-of-sensory-input-at-brainstem-in-hemifacial-spasm-and-postparalytic-facial-syndrome
#8
Meral E Kızıltan, Ayşegul Gunduz
We hypothesized the filtering of sensory input from face and hand at brainstem may reorganize in hemifacial spasm (HFS) and postparalytic facial syndrome (PFS). Thus, we examined the prepulse inhibition of blink reflex (BR-PPI) in HFS and PFS. We included 12 healthy subjects, 13 patients with HFS, and 11 patients with PFS. Baseline BR, BR recovery at interstimulus interval (ISI) of 300 ms and BR-PPI at ISI of 100 ms were performed on the right sides of healthy subjects and on both sides of patients. Within-subject analysis showed baseline BR and BR-PPI were similar between asymptomatic and symptomatic sides of patients with HFS whereas BR recovery was higher on the symptomatic side...
November 9, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/29112083/characteristics-of-liver-disease-in-100-individuals-with-joubert-syndrome-prospectively-evaluated-at-a-single-center
#9
Anna Strongin, Theo Heller, Dan Doherty, Ian A Glass, Melissa A Parisi, Joy Bryant, Peter Choyke, Baris Turkbey, Kailash Daryanani, Deniz Yildirimli, Meghana Vemulapalli, Jim C Mullikin, May C Malicdan, Thierry Vilboux, William A Gahl, Meral Gunay-Aygun
BACKGROUND & AIMS: Joubert Syndrome (JS) is a rare, inherited, ciliopathy defined by cerebellar and brainstem malformations and is variably associated with liver, kidney, and ocular dysfunction. This study characterizes the hepatic findings in JS and identifies factors associated with probable portal hypertension. METHODS: 100 individuals with JS were prospectively evaluated at the National Institutes of Health Clinical Center. Laboratory tests, imaging, and DNA sequencing were performed...
November 3, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29110767/cerebrospinal-fluid-findings-in-guillain-barr%C3%A3-syndrome-and-chronic-inflammatory-demyelinating-polyneuropathies
#10
Zsolt Illes, Morten Blaabjerg
The classic immunologic alteration of the cerebrospinal fluid (CSF) in Guillain-Barré syndrome (GBS), albuminocytologic dissociation, has been known since the original paper by Guillain, Barré, and Strohl. Albuminocytologic dissociation has been also described in other forms of the GBS spectrum, such as axonal motor or motor-sensory forms (AMAN, AMSAN), the anti-GQ1b spectrum of Miller Fisher syndrome, and Bickerstaff brainstem encephalitis. Cytokines, chemokines, antibodies, complement components, and molecules with a putative neuroprotective role or indicating axonal damage have also been examined using different methods...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29110334/frontotemporal-dementia-with-trans-activation-response-dna-binding-protein-43-presenting-with-catatonic-syndrome
#11
Ryohei Watanabe, Ito Kawakami, Mitsumoto Onaya, Shinji Higashi, Nobutaka Arai, Haruhiko Akiyama, Masato Hasegawa, Tetsuaki Arai
Catatonia is a clinical syndrome characterized by symptoms such as immobility, mutism, stupor, stereotypy, echophenomena, catalepsy, automatic obedience, posturing, negativism, gegenhalten and ambitendency. This syndrome occurs mostly in mood disorder and schizophrenic patients, and is related to neuronal dysfunction involving the frontal lobe. Some cases of frontotemporal dementia (FTD) with catatonia have been reported, but these cases were not examined by autopsy. Here, we report on a FTD case which showed catatonia after the first episode of brief psychotic disorder...
November 7, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29102776/assessing-disease-modifying-effects-of-norepinephrine-in-down-syndrome-and-alzheimer-s-disease
#12
Ravikumar Ponnusamy, M Windy McNerney, Shahrzad Moghadam, Ahmad Salehi
Building upon the knowledge that a number of important brain circuits undergo significant degeneration in Alzheimer's disease, numerous recent studies suggest that the norepinephrine-ergic system in the brainstem undergoes significant alterations early in the course of both Alzheimer's disease and Down syndrome. Massive projections from locus coeruleus neurons to almost the entire brain, extensive innervation of brain capillaries, and widespread distribution of noradrenergic receptors enable the norepinephrine-ergic system to play a crucial role in neural processes, including cognitive function...
November 8, 2017: Brain Research
https://www.readbyqxmd.com/read/29098103/cochlear-implantation-in-patients-with-keratitis-ichthyosis-deafness-syndrome-a-report-of-two-cases
#13
Birgul Gumus, Armagan Incesulu, Mehmet Ozgur Pinarbasli
Background: Keratitis-ichthyosis-deafness (KID) syndrome is a syndrome which presents with hearing loss and visual and keratinization disorders. In such patients, hearing aids cannot be effectively used in the rehabilitation of hearing loss because of the frequent blockage of the external ear canal with epithelial debris and due to dry and tense skin of the external ear canal. Moreover, severe or profound hearing loss also limits the benefits gained from the conventional hearing aids...
2017: Case Reports in Otolaryngology
https://www.readbyqxmd.com/read/29089158/cerebellar-ataxia-with-neuropathy-and-vestibular-areflexia-syndrome-canvas
#14
Masakatsu Taki, Takashi Nakamura, Hiraku Matsuura, Tatsuhisa Hasegawa, Hirofumi Sakaguchi, Kanako Morita, Ryotaro Ishii, Ikuko Mizuta, Takashi Kasai, Toshiki Mizuno, Shigeru Hirano
Cerebellar ataxia with neuropathy and bilateral vestibular areflexia syndrome (CANVAS) is a novel ataxic disorder consisting of the triad of cerebellar impairment, bilateral vestibular hypofunction, and a somatosensory deficit. We report the first Japanese case of CANVAS. The patient is a 68-year-old Japanese male. He was referred to our university for further evaluation of progressive gait disturbance and ataxia. He exhibited horizontal gaze-evoked nystagmus and sensory deficit. Nerve conduction studies showed sensory neuronopathy...
October 28, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/29081427/clinical-and-video-head-impulse-test-in-the-diagnosis-of-posterior-circulation-stroke-presenting-as-acute-vestibular-syndrome-in%C3%A2-the-emergency-department
#15
Ayse Guler, Funda Karbek Akarca, Cenk Eraslan, Ceyda Tarhan, Cem Bilgen, Tayfun Kirazli, Nese Celebisoy
INTRODUCTION: Head impulse test (HIT) is the critical bedside examination which differentiates vestibular neuritis (VN) from posterior circulation stroke (PCS) in acute vestibular syndrome (AVS). Video-oculography based HIT (vHIT) may have aadditional strength in making the differentiation. METHODS: Patients admitted to the emergency department of a tertiary-care medical center with AVS were studied. An emergency specialist and a neurologist performed HIT. vHIT was conducted by an neuro-otology research fellow...
2017: Journal of Vestibular Research: Equilibrium & Orientation
https://www.readbyqxmd.com/read/29080228/morphology-of-subcortical-brain-nuclei-is-associated-with-autonomic-function-in-healthy-humans
#16
James K Ruffle, Steven J Coen, Vincent Giampietro, Steven C R Williams, A Vania Apkarian, Adam D Farmer, Qasim Aziz
The autonomic nervous system (ANS) is a brain body interface which serves to maintain homeostasis by influencing a plethora of physiological processes, including metabolism, cardiorespiratory regulation and nociception. Accumulating evidence suggests that ANS function is disturbed in numerous prevalent clinical disorders, including irritable bowel syndrome and fibromyalgia. While the brain is a central hub for regulating autonomic function, the association between resting autonomic activity and subcortical morphology has not been comprehensively studied and thus was our aim...
October 28, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/29065229/prediction-of-disease-progression-in-miller-fisher-and-overlap-syndromes
#17
Christine Verboon, Heleen van Berghem, Pieter A van Doorn, Liselotte Ruts, Bart C Jacobs
BACKGROUND: Patients with Miller Fisher syndrome (MFS) may have a relatively mild clinical course or progress to Guillain-Barré syndrome with limb weakness (MFS-GBS overlap syndrome). Other variants in this spectrum are GBS with ophthalmoparesis and Bickerstaff's Brainstem encephalitis (BBE). AIM: To compare the clinical course of MFS and overlap syndromes and to identify predictors of disease progression. METHODS: In a prospective study of 170 patients with GBS and variant forms, 37 (22%) had a MFS, MFS-GBS overlap syndrome, ophthalmoplegic GBS or BBE...
October 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/29064441/pattern-recognition-of-the-multiple-sclerosis-syndrome
#18
REVIEW
Rana K Zabad, Renee Stewart, Kathleen M Healey
During recent decades, the autoimmune disease neuromyelitis optica spectrum disorder (NMOSD), once broadly classified under the umbrella of multiple sclerosis (MS), has been extended to include autoimmune inflammatory conditions of the central nervous system (CNS), which are now diagnosable with serum serological tests. These antibody-mediated inflammatory diseases of the CNS share a clinical presentation to MS. A number of practical learning points emerge in this review, which is geared toward the pattern recognition of optic neuritis, transverse myelitis, brainstem/cerebellar and hemispheric tumefactive demyelinating lesion (TDL)-associated MS, aquaporin-4-antibody and myelin oligodendrocyte glycoprotein (MOG)-antibody NMOSD, overlap syndrome, and some yet-to-be-defined/classified demyelinating disease, all unspecifically labeled under MSsyndrome...
October 24, 2017: Brain Sciences
https://www.readbyqxmd.com/read/29055484/chronic-lymphocytic-inflammation-with-pontine-perivascular-enhancement-responsive-to-steroids-clippers-a-pediatric-case-report-with-six-year-follow-up
#19
Aravindhan Veerapandiyan, Amit Chaudhari, Prabhav Deo, Xue Ming
BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder involving brainstem, pons in particular, characterized by a predominant T-cell pathology and responsiveness to glucocorticosteroids. We describe a difficult 6 year course of this rare syndrome in a 10-year-old girl. CASE REPORT: Our patient presented with diplopia, spastic paraparesis, and unsteady gait. MRI showed characteristic punctate hyper-intensities with enhancement in the brain stem, cerebellar peduncles, and optic chiasm and diffuse nodular enhancement throughout the cervical and thoracic spinal cord...
October 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/29053833/clinical-pathological-and-functional-characterization-of-riboflavin-responsive-neuropathy
#20
Andreea Manole, Zane Jaunmuktane, Iain Hargreaves, Marthe H R Ludtmann, Vincenzo Salpietro, Oscar D Bello, Simon Pope, Amelie Pandraud, Alejandro Horga, Renata S Scalco, Abi Li, Balasubramaniem Ashokkumar, Charles M Lourenço, Simon Heales, Rita Horvath, Patrick F Chinnery, Camilo Toro, Andrew B Singleton, Thomas S Jacques, Andrey Y Abramov, Francesco Muntoni, Michael G Hanna, Mary M Reilly, Tamas Revesz, Dimitri M Kullmann, James E C Jepson, Henry Houlden
Brown-Vialetto-Van Laere syndrome represents a phenotypic spectrum of motor, sensory, and cranial nerve neuropathy, often with ataxia, optic atrophy and respiratory problems leading to ventilator-dependence. Loss-of-function mutations in two riboflavin transporter genes, SLC52A2 and SLC52A3, have recently been linked to Brown-Vialetto-Van Laere syndrome. However, the genetic frequency, neuropathology and downstream consequences of riboflavin transporter mutations are unclear. By screening a large cohort of 132 patients with early-onset severe sensory, motor and cranial nerve neuropathy we confirmed the strong genetic link between riboflavin transporter mutations and Brown-Vialetto-Van Laere syndrome, identifying 22 pathogenic mutations in SLC52A2 and SLC52A3, 14 of which were novel...
November 1, 2017: Brain: a Journal of Neurology
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