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brainstem syndromes

Chen Cheng, Ying Jiang, Xiaodong Lu, Fu Gu, Zhuang Kang, Yongqiang Dai, Zhengqi Lu, Xueqiang Hu
BACKGROUND: Acute brainstem syndrome (ABS) may herald multiple sclerosis (MS), neuromyelitis optica (NMO), or occur as an isolated syndrome. The aquaporin 4 (AQP4)-specific serum autoantibody, NMO-IgG, is a biomarker for NMO. However, the role of anti-AQP4 antibody in the conversion of ABS to NMO is unclear. METHODS: Thirty-one patients with first-event ABS were divided into two groups according to the presence of anti-AQP4 antibodies, their clinical features and outcomes were retrospectively analyzed...
October 21, 2016: BMC Neurology
Scott Seaman, Paul Nelson, Jacob Alexander, Andrew Swift, James Fick
The authors present the case of a 53-year-old man who was referred with disabling retching provoked by left arm abduction. At the time of his initial evaluation, a cervical MRI study was available for review and revealed an anatomical variation of the ipsilateral juxtamedullary vertebrobasilar junction. After brain imaging revealed contact of the medulla by a dolichoectatic vertebral artery at the dorsal root entry zone of the glossopharyngeal and vagus nerves, the patient was successfully treated by microvascular decompression of the brainstem and cranial nerves...
October 21, 2016: Journal of Neurosurgery
Giuseppe Lanza, Cornelius G Bachmann, Imad Ghorayeb, Yuping Wang, Raffale Ferri, Walter Paulus
Neurophysiological techniques have been applied in restless legs syndrome (RLS) to obtain direct and indirect measures of central and peripheral nervous system excitability, as well as to probe different neurotransmission pathways. Data converge on the hypothesis that, from a pure electrophysiological perspective, RLS should be regarded as a complex sensorimotor disorder in which cortical, subcortical, spinal cord, and peripheral nerve generators are all involved in a network disorder, resulting in an enhanced excitability and/or decreased inhibition...
August 27, 2016: Sleep Medicine
Shubir Dutt, Richard J Binney, Hilary W Heuer, Phi Luong, Suneth Attygalle, Priyanka Bhatt, Gabe A Marx, Jonathan Elofson, Maria C Tartaglia, Irene Litvan, Scott M McGinnis, Bradford C Dickerson, John Kornak, Dana Waltzman, Lisa Voltarelli, Norbert Schuff, Gil D Rabinovici, Joel H Kramer, Clifford R Jack, Bruce L Miller, Howard J Rosen, Adam L Boxer
OBJECTIVE: To examine the utility and reliability of volumetric MRI in measuring disease progression in the 4 repeat tauopathies, progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS), to support clinical development of new tau-directed therapeutic agents. METHODS: Six- and 12-month changes in regional MRI volumes and PSP Rating Scale scores were examined in 55 patients with PSP and 33 patients with CBS (78% amyloid PET negative) compared to 30 normal controls from a multicenter natural history study...
October 14, 2016: Neurology
Christos Panayi, Nagui Antoun, Richard Sandford
A 44-year-old woman with a history of von Hippel-Lindau (VHL) disease, a rare inherited neoplasia syndrome, presented acutely to hospital with a productive cough, symptoms of respiratory tract infection and odynophagia (painful swallowing). A chest X-ray confirmed right-sided pneumonia. Investigation of the persistent odynophagia using barium swallow revealed aspiration of the contrast into the lungs and suggested a neurological cause for her chest infection. Clinical assessment and speech and language therapy confirmed a pseudobulbar palsy...
October 13, 2016: BMJ Case Reports
Sung-Hee Kim, David S Zee, Sascha du Lac, Hyo Jung Kim, Ji-Soo Kim
OBJECTIVE: To describe the ocular motor abnormalities in 9 patients with a lesion involving the nucleus prepositus hypoglossi (NPH), a key constituent of a vestibular-cerebellar-brainstem neural network that ensures that the eyes are held steady in all positions of gaze. METHODS: We recorded eye movements, including the vestibulo-ocular reflex during head impulses, in patients with vertigo and a lesion involving the NPH. RESULTS: Our patients showed an ipsilesional-beating spontaneous nystagmus, horizontal gaze-evoked nystagmus more intense on looking toward the ipsilesional side, impaired pursuit more to the ipsilesional side, central patterns of head-shaking nystagmus, contralateral eye deviation, and decreased vestibulo-ocular reflex gain during contralesionally directed head impulses...
October 12, 2016: Neurology
Emmanuel Lilitsis, Alexia Papaioannou, Aikaterini Hatzimichali, Konstantinos Spyridakis, Sofia Xenaki, George Chalkiadakis, Emmanuel Chrysos
BACKGROUND: We present a case of a patient with multinodular goiter disease who suffered asystole during head hyperextension for surgical positioning on the operational table. CASE PRESENTATION: Manipulation of carotid sinus may trigger bradycardia or even asystole even in patients without prior history of carotid sinus hypersensitivity. The time proximity between patient positioning and asystole, the late responsiveness to atropine, the immediate increase of heart rate after head elevation and the lack of any other trigger factor or prior history support the hypothesis of carotid sinus syndrome...
October 6, 2016: BMC Anesthesiology
Mohammad Zare Mehrjardi, Elham Keshavarz, Andrea Poretti, Adriano N Hazin
Zika virus (ZIKV) is an arbovirus from the Flaviviridae family. It is usually transmitted by mosquito bite. There have been no reports of severe symptoms caused by ZIKV infection up until the last few years. In October 2013 an outbreak was reported in French Polynesia with severe neurological complications in some affected cases. In November 2015, the Ministry of Health of Brazil attributed the increased number of neonatal microcephaly cases in northeastern Brazil to congenital ZIKV infection. The rapid spread of the virus convinced the World Health Organization to announce ZIKV infection as a "Public Health Emergency of International Concern" in February 2016...
October 6, 2016: Japanese Journal of Radiology
Giulia Ottaviani
Crib death, or sudden infant death syndrome (SIDS), is the most frequent form of death in the first year of life, striking one baby in every 1,700-2,000. Yet, despite advances in maternal-infant care, sudden intrauterine unexplained/unexpected death syndrome (SIUDS) has a sixfold to eightfold greater incidence than that of SIDS. Frequent congenital abnormalities, likely morphological substrates for SIDS-SIUDS, were detected, mainly represented by alterations of the cardiac conduction system, such as accessory pathways and abnormal resorptive degeneration, and hypoplasia/agenesis of the vital brainstem structures...
2016: Frontiers in Pediatrics
W Teng, H Chen, F Guo, X Du, X Fu, Y Fang, H Zhang, M Fang, M Ding
Neurokinin-1 receptor (NK1R) is a high affinity Substance P (SP) receptor and plays a key role in visceral hypersensitivity in irritable bowel syndrome (IBS). Early life stress is a significant risk factor in IBS. The aim of the present study was to investigate the influence of neonatal maternal separation on the expression and distribution of SP and its receptor along the brain-gut axis in a neonatal maternally separated rat model with visceral hypersensitivity. Male neonatal Sprague-Dawley rats, 2-21-day old, were randomly distributed into maternal separation groups of 3 h daily maternal separation (MS) or non-handling (NH)...
August 30, 2016: Genetics and Molecular Research: GMR
Perrine Séguin, Annie Moulin, Lesly Fornoni, Jérémie Mattout, Emmanuel Maby, Brice Lapin, Véronique Blandin, Pascal Giraux
OBJECTIVE: Brainstem lesions can cause a locked-in syndrome (LIS). At the initial state, the LIS can be complete (CLIS), without any possibility for the patient to communicate. The main issues are to do the right diagnosis and the recovery of a communication. The delay to the diagnosis of LIS is of about 78 days after the initial injury (León-Carrión, 2002). The aim of this study is to identify possibilities to improve the care of persons with LIS at the initial state. MATERIAL/PATIENTS AND METHODS: An original questionnaire of 33 items, concerning patient's feelings and communication possibilities at the initial state of LIS, was sent to the 274 LIS members of the French association of LIS (ALIS) in November, 2015...
September 2016: Annals of Physical and Rehabilitation Medicine
Swen Hülsmann, Guillaume Mesuret, Julia Dannenberg, Mauricio Arnoldt, Marcus Niebert
Mutations in methyl-CpG-binding protein 2 (MECP2) gene have been shown to manifest in a neurodevelopmental disorder that is called Rett syndrome. A typical problem that occurs during development is a disturbance of breathing. To address the role of inhibitory neurons, we generated a mouse line that restores MECP2 in inhibitory neurons in the brainstem by crossbreeding a mouse line that expresses the Cre-recombinase (Cre) in inhibitory neurons under the control of the glycine transporter 2 (GlyT2, slc6a5) promotor (GlyT2-Cre) with a mouse line that has a floxed-stop mutation of the Mecp2 gene (Mecp2 (stop/y))...
2016: Frontiers in Physiology
Chiara Cerami, Alessandra Dodich, Lucia Greco, Sandro Iannaccone, Giuseppe Magnani, Alessandra Marcone, Elisabetta Pelagallo, Roberto Santangelo, Stefano F Cappa, Daniela Perani
BACKGROUND AND OBJECTIVE: Primary progressive aphasia (PPA) is a clinical syndrome due to different neurodegenerative conditions in which an accurate early diagnosis needs to be supported by a reliable diagnostic tool at the individual level. In this study, we investigated in PPA the FDG-PET brain metabolic patterns at the single-subject level, in order to assess the case-to-case variability and its relationship with clinical-neuropsychological findings. MATERIAL AND METHODS: 55 patients (i...
September 20, 2016: Journal of Alzheimer's Disease: JAD
A Caignard, S Leruez, D Milea
Neuro-ophthalmic emergencies can cause life-threatening or sight-threatening complications. Various conditions may have acute neuro-ophthalmic manifestations, including inflammatory or ischemic processes, as well as tumoral, aneurysmal compression or metabolic and systemic diseases. Diplopia related to a partial third nerve palsy with pupillary involvement may reveal an intracranial aneurysm. Abnormalities of conjugate gaze may reveal an inflammatory or ischemic lesion, most often of the brainstem. An intracranial tumor may also manifest itself as a single or multiple oculomotor palsy, or causing various visual field defects, due to optic nerve, chiasm or retrochiasmal involvement...
October 2016: Journal Français D'ophtalmologie
K-D Choi, H Lee, J-S Kim
Dizziness/vertigo and imbalance are the most common symptoms of vertebrobasilar ischemia. Even though dizziness/vertigo usually accompanies other neurologic symptoms and signs in cerebrovascular disorders, a diagnosis of isolated vascular vertigo is increasing markedly by virtue of recent developments in clinical neurotology and neuroimaging. It is important to differentiate isolated vertigo of a vascular cause from more benign disorders involving the inner ear, since therapeutic strategies and prognosis differ between these two conditions...
2016: Handbook of Clinical Neurology
Xiaoran Ma, Bradley McPherson, Lian Ma
OBJECTIVES: Cleft lip and/or palate is a common congenital craniofacial malformation found worldwide. A frequently associated disorder is conductive hearing loss, and this disorder has been thoroughly investigated in children with non-syndromic cleft lip and/or palate (NSCL/P). However, analysis of auditory processing function is rarely reported for this population, although this issue should not be ignored since abnormal auditory cortical structures have been found in populations with cleft disorders...
2016: PeerJ
Robin L Haynes, Rebecca D Folkerth, David S Paterson, Kevin G Broadbelt, S Dan Zaharie, Richard H Hewlett, Johan J Dempers, Elsie Burger, Shabbir Wadee, Pawel Schubert, Colleen Wright, Mary Ann Sens, Laura Nelsen, Bradley B Randall, Hoa Tran, Elaine Geldenhuys, Amy J Elliott, Hein J Odendaal, Hannah C Kinney
The Safe Passage Study is an international, prospective study of approximately 12 000 pregnancies to determine the effects of prenatal alcohol exposure (PAE) upon stillbirth and the sudden infant death syndrome (SIDS). A key objective of the study is to elucidate adverse effects of PAE upon binding to serotonin (5-HT) 1A receptors in brainstem homeostatic networks postulated to be abnormal in unexplained stillbirth and/or SIDS. We undertook a feasibility assessment of 5-HT1A receptor binding using autoradiography in the medulla oblongata (6 nuclei in 27 cases)...
September 15, 2016: Journal of Neuropathology and Experimental Neurology
Zlatko Kolić, Melita Kukuljan, Duje Vukas, David Bonifačić, Kristina Vrbanec, Ivana Karla Franić
We describe a case of acute obstructive hydrocephalus as a consequence of compression of the brainstem by a large aneurysm of the basilar artery (BA) in a 62-year-old male. After the insertion of the ventriculoperitoneal shunt (VPS), we encountered the "locked-in syndrome" clinical condition. "Locked-in syndrome" is a clinical state characterized by quadriplegia and anarthria with preserved consciousness, most commonly caused by ischemia in the ventral part of pons.
September 15, 2016: British Journal of Neurosurgery
Suna Tokgoz-Yilmaz, Meral Didem Turkyilmaz, Filiz Basak Cengiz, Alev Pektas Sjöstrand, Serdal Kenan Kose, Mustafa Tekin
OBJECTIVES: The aim of this study was to evaluate audiologic properties of patients with Noonan syndrome and compare these findings with those of unaffected peers. METHODS: The study included 17 children with Noonan syndrome and 20 typically developing children without Noonan syndrome. Pure tone and speech audiometry, immitancemetric measurement, otoacoustic emissions measurement and auditory brainstem response tests were applied to all (n = 37) children. RESULTS: Hearing thresholds of children with Noonan syndrome were higher (poorer) than those observed unaffected peers, while the hearing sensitivity of the both groups were normal limits (p = 0...
October 2016: International Journal of Pediatric Otorhinolaryngology
Gabriela Timarova, Iveta Lisa, Peter Kukumberg
Rasmussen's encephalitis is a rare autoimmune encephalitis usually involving one brain hemisphere, presenting with refractory epileptic seizures, and neurological and cognitive decline. Only 10% of cases start later in adolescence/adulthood. The only effective treatment for refractory seizures in childhood is hemispherectomy. For late-onset cases with mild neurological deficit the hemispherectomy is usually postponed because of its severe consequences. Immunotherapy shows some temporal effect for seizure control and slowing the brain atrophy, mainly in late onset Rasmussen's encephalitis...
July 16, 2016: Neuro Endocrinology Letters
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