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https://www.readbyqxmd.com/read/28533776/reactivity-to-novel-autoantigens-in-patients-with-coexisting-central-nervous-system-demyelinating-disease-and-autoimmune-thyroid-disease
#1
Judith M Greer, Simon Broadley, Michael P Pender
Several lines of evidence suggest a definite and unique link between CNS demyelinating diseases and autoimmune thyroid disease (AITD). The aim of the current study was to systematically compare the clinical and laboratory features of patients with coexistent AITD and CNS demyelinating disease with those of patients with just CNS demyelinating disease. Forty-four patients with coexisting CNS demyelinating disease and AITD were identified and their clinical and radiological features were recorded. Blood and DNA were collected and tested for HLA type and for the response of T cells and antibodies to a variety of antigens...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28529053/unusual-intra-parenchymal-pontomedullary-epidermoid-cyst-in-a-2-year-old-case-report-and-literature-review
#2
Anan Shtaya, Bassam Dabbous, Evgenia Fanou, Leslie Bridges, Samantha Hettige
BACKGROUND: Intrinsic brainstem epidermoid cysts are rare, benign, slow growing lesions. Their eloquence preclude complete excision, however subtotal resection will often result in prolonged or sometimes permanent relief of presenting symptoms and signs. We describe an unusual case and review the literature of this pathology in the paediatric population. CASE DESCRIPTION: We report an intra-axial pontine epidermoid cyst in a 2-year-old girl who presented with developmental delay, multiple cranial nerve palsies and pneumonia...
May 18, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28488367/chemosensitive-phox2b-expressing-neurons-are-crucial-for-hypercapnic-ventilatory-response-in-the-nucleus-tractus-solitarius
#3
Congrui Fu, Jinyu Xue, Ri Wang, Jinting Chen, Lan Ma, Yixian Liu, Xuejiao Wang, Fang Guo, Yi Zhang, Xiangjian Zhang, Sheng Wang
The nucleus tractus solitarius (NTS) neurons have been thought to function as central respiratory chemoreceptors. However, it remains unknown regarding the common molecular marker defined for these neurons. Here we ask whether paired-like homeobox 2b (Phox2b)-expressing NTS neurons are recruited in hypercapnic ventilatory response (HCVR) and whether these neurons exhibit intrinsic chemosensitivity. HCVR was assessed using whole body plethysmography and neuronal chemosensitivity was examined by patch clamp recordings in brainstem slices or dissociated neurons from Phox2b-EGFP transgenic mice...
May 10, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28484537/dorsally-exophytic-glioblastoma-arising-from-the-medulla-oblongata-in-an-adult-presenting-as-4-th-ventricular-mass
#4
Kuntal Kanti Das, Guru Prasad Bettaswamy, Anant Mehrotra, Sushila Jaiswal, Awadhesh Kumar Jaiswal, Sanjay Behari
Brainstem gliomas are relatively rare in adults (<2% of all gliomas). Exophytic gliomas are focal brainstem lesions, which project into the 4(th) ventricle or cerebellopontine angles. These exophytic lesions are usually of low-grade histology (pilocytic astrocytoma or ganglioglioma) and have a relatively better outcome compared with brainstem gliomas as a whole. Glioblastoma is the commonest primary glial cell neoplasm and mostly occurs in the supratentorial compartment. It is rather uncommon in the brainstem and seldom has been described as having an exophytic growth pattern...
April 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28483068/factors-associated-with-multidimensional-aspect-of-post-stroke-fatigue-in-acute-stroke-period
#5
Hitoshi Mutai, Tomomi Furukawa, Ayumi Houri, Akihito Suzuki, Tokiji Hanihara
BACKGROUND: Post-stroke fatigue (PSF) is a frequent and distressing consequence of stroke, and can be both acute and long lasting. We aimed to investigate multidimensional aspects of acute PSF and to determine the clinical factors relevant to acute PSF. METHODS: We collected data of 101 patients admitted to the hospital for acute stroke. PSF was assessed using the Multidimensional Fatigue Inventory within 2 weeks of stroke. Measures included Mini-Mental State Examination, Hospital Anxiety and Depression Scale, and Functional Independence Measure...
April 2017: Asian Journal of Psychiatry
https://www.readbyqxmd.com/read/28482247/a-possible-biomarker-for-methadone-related-deaths
#6
Antonina Argo, Gianfranco Francesco Spatola, Stefania Zerbo, Cettina Sortino, Antonietta Lanzarone, Maria Laura Uzzo, Alessandro Pitruzzella, Fiorenza Farè, Gabriella Roda, Veniero Gambaro, Paolo Procaccianti, Steven B Karch
Methadone (MTH) concentrations in those dying of MTH toxicity totally overlap concentrations where the presence of MTH is only an incidental finding, making it very difficult to make distinctions in actual cases. A biomarker, be it anatomical or biochemical for MTH toxicity is badly needed, particularly if that markers were known to disrupt effective ventilation. Because the brainstem houses the regulatory centers for cardiorespiratory-control enters, it would seem to be the most likely anatomical site to seek abnormalities in cardiorespiratory control...
May 3, 2017: Journal of Forensic and Legal Medicine
https://www.readbyqxmd.com/read/28479813/dilated-virchow-robin-spaces-mimicking-a-brainstem-arteriovenous-malformation
#7
Thomas J Buell, Arjun Ramesh, Dale Ding, Daniel M S Raper, Ching-Jen Chen, Robert M Starke, Sugoto Mukherjee, R Webster Crowley
Virchow-Robin spaces (VRS) are ubiquitous and commonly observed as the resolution of magnetic resonance imaging (MRI) continues to improve. The function of VRS and the etiology of their dilation is still a subject of research. Diagnosing dilated VRS (dVRS) can be challenging because they may appear similar to other pathologies such as cystic neoplasms, infectious cysts, and even arteriovenous malformations (AVMs) on certain MRI pulse sequences. We reported a unique case of brainstem dVRS mimicking an AVM. Furthermore, the extensive pontine involvement of our patient's lesion is rarely described in neurosurgical literature...
April 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28477152/revisiting-grade-3-diffuse-axonal-injury-not-all-brainstem-microbleeds-are-prognostically-equal
#8
Saef Izzy, Nicole L Mazwi, Sergi Martinez, Camille A Spencer, Joshua P Klein, Gunjan Parikh, Mel B Glenn, Steven M Greenberg, David M Greer, Ona Wu, Brian L Edlow
BACKGROUND: Recovery of functional independence is possible in patients with brainstem traumatic axonal injury (TAI), also referred to as "grade 3 diffuse axonal injury," but acute prognostic biomarkers are lacking. We hypothesized that the extent of dorsal brainstem TAI measured by burden of traumatic microbleeds (TMBs) correlates with 1-year functional outcome more strongly than does ventral brainstem, corpus callosal, or global brain TMB burden. Further, we hypothesized that TMBs within brainstem nuclei of the ascending arousal network (AAN) correlate with 1-year outcome...
May 5, 2017: Neurocritical Care
https://www.readbyqxmd.com/read/28473800/central-positional-nystagmus-a-systematic-literature-review
#9
REVIEW
Nora K Macdonald, Diego Kaski, Yougan Saman, Amal Al-Shaikh Sulaiman, Amal Anwer, Doris-Eva Bamiou
OBJECTIVE: To provide a systematic review of the clinical and radiological features of lesion-induced central positional nystagmus (CPN) and identify salient characteristics that differentiate central from peripheral positional nystagmus (PN). METHODS: Systematic literature search according to the preferred reporting items for systematic reviews and meta-analysis. RESULTS: A total of 82 patients from 28 studies met the participants intervention, comparison, outcomes, and study designs criteria for inclusion...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28465700/neuromyelitis-optica-spectrum-disease-characteristics-in-isfahan-iran-a-cross-sectional-study
#10
Fereshte Ashtari, Ali Safaei, Vahid Shaygannejad, Mohammad Amin Najafi, Sahar Vesal
BACKGROUND: Neuromyelitis optica spectrum disease (NMOSD) is a severe autoimmune demyelinating disorder of the central nervous system that throughout epidemiological data, it has not been completely determined. The aim of this study was to assess characteristics of NMOSD patients in Isfahan as one of the most prevalent cities for multiple sclerosis in Iran. MATERIALS AND METHODS: Forty-five patients diagnosed as neuromyelitis optica (NMO) disease through 5 years enrolled in this study...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/28454455/brainstem-tuberculous-abscesses-successfully-treated-by-microsurgical-excision-a-case-report-and-review-of-the-literature
#11
Baofeng Xu, Yandong Zhang, Jinlu Yu
In patients without systemic manifestations of tuberculosis, isolated brainstem tuberculous abscesses are infrequently observed. The present study reports a rare case of two cystic tuberculous abscesses involving the dorsal aspect of the medulla oblongata in an otherwise healthy 14-year-old Chinese girl, whose primary complaint was respiratory distress. These lesions were successfully treated using a microsurgical excision approach and anti-tuberculosis therapy. Although rare, tuberculous abscesses should be considered in the differential diagnosis of isolated cystic brainstem lesions...
April 2017: Oncology Letters
https://www.readbyqxmd.com/read/28443270/brain-lesions-among-orally-fed-and-gastrostomy-fed-dysphagic-preterm-infants-can-routine-qualitative-or-volumetric-quantitative-magnetic-resonance-imaging-predict-feeding-outcomes
#12
Nasser H Kashou, Irfaan A Dar, Mohamed A El-Mahdy, Charles Pluto, Mark Smith, Ish K Gulati, Warren Lo, Sudarshan R Jadcherla
INTRODUCTION: The usefulness of qualitative or quantitative volumetric magnetic resonance imaging (MRI) in early detection of brain structural changes and prediction of adverse outcomes in neonatal illnesses warrants further investigation. Our aim was to correlate certain brain injuries and the brain volume of feeding-related cortical and subcortical regions with feeding method at discharge among preterm dysphagic infants. MATERIALS AND METHODS: Using a retrospective observational study design, we examined MRI data among 43 (22 male; born at 31...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28440867/the-epileptology-of-koolen-de-vries-syndrome-electro-clinico-radiologic-findings-in-31-patients
#13
Kenneth A Myers, Simone A Mandelstam, Georgia Ramantani, Elisabeth J Rushing, Bert B de Vries, David A Koolen, Ingrid E Scheffer
OBJECTIVE: This study was designed to describe the spectrum of epilepsy phenotypes in Koolen-de Vries syndrome (KdVS), a genetic syndrome involving dysmorphic features, intellectual disability, hypotonia, and congenital malformations, that occurs secondary to 17q21.31 microdeletions and heterozygous mutations in KANSL1. METHODS: We were invited to attend a large gathering of individuals with KdVS and their families. While there, we recruited individuals with KdVS and seizures, and performed thorough phenotyping...
April 25, 2017: Epilepsia
https://www.readbyqxmd.com/read/28437859/multiple-sclerosis-broke-my-heart
#14
Géraldine Androdias, Emilien Bernard, Damien Biotti, Nicolas Collongues, Françoise Durand-Dubief, Julie Pique, Ingrid Sanchez, Clément Delmas, Jacques Ninet, Romain Marignier, Sandra Vukusic
We report 5 cases of acute heart failure (AHF) related to multiple sclerosis (MS) relapses. AHF was inaugural in 3 patients, always preceded or accompanied by signs of brainstem dysfunction; it was severe, requiring intensive care management. Echocardiography showed left ventricular hypokinesis. No other cause of AHF has been found. All patients showed a new medullary lesion on brain magnetic resonance imaging. All had rapid and complete recovery of ventricular function after intravenous corticosteroids. We concluded that the cases represent a takotsubo phenomenon...
April 24, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28433109/pathogenesis-of-wilson-disease
#15
Ivo Florin Scheiber, Radan Brůha, Petr Dušek
Wilson disease is an autosomal-recessive disorder originating from a genetic defect in the copper-transporting ATPase ATP7B that is required for biliary copper secretion and loading of ceruloplasmin with copper. Impaired ATP7B function in Wilson disease results in excessive accumulation of copper in liver, brain, and other tissues. Toxic copper deposits may induce oxidative stress, modify expression of genes, directly inhibit proteins, and impair mitochondrial function, leading to hepatic, neuropsychiatric, renal, musculoskeletal, and other symptoms...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28431905/dynamic-interhemispheric-competition-and-vestibulo-cortical-control-in-humans-a-theoretical-proposition
#16
REVIEW
Qadeer Arshad
Neuroscientific research has made a concerted effort to determine cortical localization using various functional imaging techniques. This approach has undoubtedly yielded important novel anatomical knowledge, albeit at times contradictory, regarding the structural organization of the vestibular cortex. Unfortunately however, this knowledge has not translated to our understanding regarding how neural mechanisms control vestibular function. Based upon recent functional imaging, lesion and neuro-physiological data that have demonstrated (i) the close behavioral and neuro-anatomical relationship between cortical processing of vestibular and spatial attention signals, and (ii) that inducing interhemispheric competition can in-turn strongly modulate vestibular function akin to that observed in cortical lesion patients, I herewith propose the hypothesis that vestibular cortical processing is controlled, as per for spatial attention, via dynamic interhemispheric competition...
April 19, 2017: Neuroscience
https://www.readbyqxmd.com/read/28415891/influence-of-two-electrode-montages-on-the-level-specific-ls-ce-chirp-auditory-brainstem-response-abr-at-multiple-intensity-levels
#17
Ahmad Aidil Arafat Dzulkarnain, Siti Hajra Mu'minah Noor Ibrahim, Nur Farah Aida Anuar, Siti Aisyah Abdullah, Tengku Zulaila Hasma Tengku Zam Zam, Sarah Rahmat, Muhammad Amar Mohd Ruzai
OBJECTIVE: To investigate the influence of two different electrode montages (ipsilateral: reference to mastoid and vertical: reference to nape of neck) to the ABR results recorded using a level-specific (LS)-CE-Chirp® in normally hearing subjects at multiple intensities levels. DESIGN: Quasi-experimental and repeated measure study designs were applied in this study. Two different stopping criteria were used, (1) a fixed-signal averaging 4000 sweeps and, (2) a minimum quality indicator of Fmp = 3...
April 18, 2017: International Journal of Audiology
https://www.readbyqxmd.com/read/28413710/combination-of-standard-axial-and-thin-section-coronal-diffusion-weighted-imaging-facilitates-the-diagnosis-of-brainstem-infarction
#18
Philippe Felfeli, Holger Wenz, Mansour Al-Zghloul, Christoph Groden, Alex Förster
BACKGROUND AND PURPOSE: Although diffusion-weighted imaging (DWI) is a very sensitive technique for the detection of small ischemic lesions in the human brain, in particular in the brainstem it may fail to demonstrate acute ischemic infarction. In this study, we sought to evaluate the value of additional thin-section coronal DWI for the detection of brainstem infarction. METHODS: In 155 consecutive patients (median age 69 [interquartile range, IQR 57-78] years, 95 [61...
April 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28410279/ocular-motor-manifestations-of-multiple-sclerosis
#19
Elodie Nerrant, Caroline Tilikete
BACKGROUND: Multiple sclerosis (MS) is a demyelinating disease of the central nervous system leading to disability, especially in young patients. Acute or chronic lesions of MS within the brainstem and the cerebellum frequently result in ocular motor disorders. EVIDENCE ACQUISITION: This review encompasses the spectrum of ocular motor disorders in patients with MS emphasizing prevalence, examination findings, diagnostic features, functional consequences, classification of MS course, and management of these disturbances of ocular motility...
April 13, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28395088/neuropathological-hallmarks-of-brain-malformations-in-extreme-phenotypes-related-to-dync1h1-mutations
#20
Annie Laquerriere, Camille Maillard, Mara Cavallin, Françoise Chapon, Florent Marguet, Arnaud Molin, Sabine Sigaudy, Marie Blouet, Guillaume Benoist, Carla Fernandez, Karine Poirier, Jamel Chelly, Sophie Thomas, Nadia Bahi-Buisson
Dyneins play a critical role in a wide variety of cellular functions such as the movement of organelles and numerous aspects of mitosis, making it central player in neocortical neurogenesis and migration. Recently, cytoplasmic dynein-1, heavy chain-1 (DYNC1H1) mutations have been found to cause a wide spectrum of brain cortical malformations. We report on the detailed neuropathological features of brain lesions from 2 fetuses aged 36 and 22 weeks of gestation (WG), respectively, carrying de novo DYNC1H1 mutations, p...
March 1, 2017: Journal of Neuropathology and Experimental Neurology
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