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https://www.readbyqxmd.com/read/28340257/cd8-positive-t-cell-leukoencephalitis-with-astrocytopathy-clinically-presenting-as-neuromyelitis-optica
#1
Diana L Thomas, Jody Manners, Daniel Marker, Joseph Mettenburg, Geoffrey Murdoch, Bryan Stevens, Guoji Wang, Clayton Wiley
We describe a novel disease entity with the clinical and radiologic presentation of neuromyelitis optica (NMO) and widespread CD8-positive T-cell leukoencephalitis and astrocytopathy. The 59-year-old female patient had a complex 2-year neurological history that included early changes in cognition and memory, progressive lower extremity motor dysfunction, and multimodal sensory involvement. MRI of the spinal cord showed increased T2 signal in the central cord extending from C2 through T4. MRI of the brain showed symmetric radial enhancement in periventricular deep white matter without evidence of demyelinating lesions...
March 16, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28338441/radiosurgery-in-the-management-of-brain-metastasis-a-retrospective-single-center-study-comparing-gamma-knife-and-linac-treatment
#2
Constantin Tuleasca, Laura Negretti, Mohamed Faouzi, Vera Magaddino, Thierry Gevaert, Erik von Elm, Marc Levivier
OBJECTIVE The authors present a retrospective analysis of a single-center experience with treatment of brain metastases using Gamma Knife (GK) and linear accelerator (LINAC)-based radiosurgery and compare the results. METHODS From July 2010 to July 2012, 63 patients with brain metastases were treated with radiosurgery. Among them, 28 (with 83 lesions) were treated with a GK unit and 35 (with 47 lesions) with a LINAC. The primary outcome was local progression-free survival (LPFS), evaluated on a per-lesion basis...
March 24, 2017: Journal of Neurosurgery
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/28334875/white-matter-changes-in-paediatric-multiple-sclerosis-and-monophasic-demyelinating-disorders
#4
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/28330960/a-multi-institutional-study-of-brainstem-gliomas-in-children-with-neurofibromatosis-type-1
#5
Jasia Mahdi, Amish C Shah, Aimee Sato, Stephanie M Morris, Robert C McKinstry, Robert Listernick, Roger J Packer, Michael J Fisher, David H Gutmann
OBJECTIVE: To define the clinical and radiologic features of brainstem gliomas (BSGs) in children with neurofibromatosis type 1 (NF1). METHODS: We performed a retrospective cross-sectional study of 133 children with NF1 and concurrent BSGs cared for at 4 NF1 referral centers. BSG was determined using radiographic criteria. Age at diagnosis, tumor location and appearance, clinical symptoms, treatment, and presence of a concurrent optic pathway glioma were assessed...
March 22, 2017: Neurology
https://www.readbyqxmd.com/read/28325621/-diplopia-an-important-symptom-in-internal-medicine
#6
S Bidot, V Biousse
Diplopia is defined as "double vision" when looking at a single object. Monocular diplopia is related to an ocular disorder and must be differentiated from binocular diplopia which is secondary to ocular misalignment. The examination of the patient with binocular diplopia is often challenging for non-specialists. However, a careful and systematic clinical examination followed by targeted ancillary testing allows the clinician to localize the lesion along the oculomotor pathways. The lesion may involve the brainstem, the ocular motor nerves III, IV or VI, the neuromuscular junction, the extraocular ocular muscles, or the orbit...
March 18, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28314977/isolated-vestibular-syndromes-due-to-brainstem-and-cerebellar-lesions
#7
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/28298049/the-interpeduncular-fossa-approach-for-resection-of-ventromedial-midbrain-lesions
#8
M Yashar S Kalani, Kaan Yağmurlu, Robert F Spetzler
The authors describe the interpeduncular fossa safe entry zone as a route for resection of ventromedial midbrain lesions. To illustrate the utility of this novel safe entry zone, the authors provide clinical data from 2 patients who underwent contralateral orbitozygomatic transinterpeduncular fossa approaches to deep cavernous malformations located medial to the oculomotor nerve (cranial nerve [CN] III). These cases are supplemented by anatomical information from 6 formalin-fixed adult human brainstems and 4 silicone-injected adult human cadaveric heads on which the fiber dissection technique was used...
March 10, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28298018/brainstem-arteriovenous-malformations-lesion-characteristics-and-treatment-outcomes
#9
Venkatesh S Madhugiri, Mario K C Teo, Joli Vavao, Teresa Bell-Stephens, Gary K Steinberg
OBJECTIVE Brainstem arteriovenous malformations (AVMs) are rare lesions that are difficult to diagnose and treat. They are often more aggressive in their behavior when compared with their supratentorial counterparts. The consequence of a brainstem hemorrhage is often devastating, and many patients are in poor neurological status at presentation. The authors examine the factors associated with angiographically confirmed cure and those affecting management outcomes for these complex lesions. METHODS This was a retrospective analysis of data gathered from the prospectively maintained Stanford AVM database...
February 17, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28298014/effect-of-granulocyte-colony-stimulating-factor-on-the-cochlear-nuclei-after-creation-of-a-partial-nerve-lesion-an-experimental-study-in-rats
#10
Guilherme Ramina Montibeller, Benjamin Schackmann, Steffi Urbschat, Joachim M K Oertel
OBJECTIVE The risk of injury of the cochlear nerve during angle (CPA) surgery is high. Granulocyte colony-stimulating factor (G-CSF) has been found in various experimental models of peripheral and CNS injury to have a neuroprotective effect by inhibiting apoptosis and inflammation. However, to the authors' knowledge, the influence of G-CSF on cochlear nerve regeneration has not been reported. This study investigated the neuroprotective effect of G-CSF after a partial cochlear nerve lesion in rats. METHODS A lesion of the right cochlear nerve in adult male Sprague-Dawley rats was created using a water-jet dissector with a pressure of 8 bar...
February 17, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28297672/identification-of-intrinsic-axon-growth-modulators-for-intact-cns-neurons-after-injury
#11
Kathren L Fink, Francesc López-Giráldez, In-Jung Kim, Stephen M Strittmatter, William B J Cafferty
Functional deficits persist after spinal cord injury (SCI) because axons in the adult mammalian central nervous system (CNS) fail to regenerate. However, modest levels of spontaneous functional recovery are typically observed after trauma and are thought to be mediated by the plasticity of intact circuitry. The mechanisms underlying intact circuit plasticity are not delineated. Here, we characterize the in vivo transcriptome of sprouting intact neurons from Ngr1 null mice after partial SCI. We identify the lysophosphatidic acid signaling modulators LPPR1 and LPAR1 as intrinsic axon growth modulators for intact corticospinal motor neurons after adjacent injury...
March 14, 2017: Cell Reports
https://www.readbyqxmd.com/read/28283819/neurological-complications-of-beh%C3%A3-et-s-syndrome
#12
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/28281033/a-treatable-cause-of-myelopathy-and-vision-loss-mimicking-neuromyelitis-optica-spectrum-disorder-late-onset-biotinidase-deficiency
#13
Sanem Yilmaz, Mine Serin, Ebru Canda, Cenk Eraslan, Hande Tekin, Sema Kalkan Ucar, Sarenur Gokben, Hasan Tekgul, Gul Serdaroglu
Biotinidase deficiency is characterized by severe neurological manifestations as hypotonia, lethargy, ataxia, hearing loss, seizures and developmental retardation in its classical form. Late-onset biotinidase deficiency presents distinctly from the classical form such as limb weakness and vision problems. A 14-year-old boy presented with progressive vision loss and upper limb weakness. The patient was initiated steroid therapy with a preliminary diagnosis of neuromyelitis optica spectrum disorder due to the craniospinal imaging findings demonstrating optic nerve, brainstem and longitudinally extensive spinal cord involvement...
March 9, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28278447/depressive-disorder-may-be-associated-with-raphe-nuclei-lesions-in-patients-with-brainstem-infarction
#14
Yoshiyuki Numasawa, Takaaki Hattori, Sumio Ishiai, Zen Kobayashi, Tomoyuki Kamata, Minoru Kotera, Satoru Ishibashi, Nobuo Sanjo, Hidehiro Mizusawa, Takanori Yokota
BACKGROUND: Depression is a common symptom after stroke, but its neural substrates remain unclear. The ascending serotonergic system originates from the raphe nuclei in the brainstem. We hypothesized that depressive disorder due to brainstem infarction is associated with damage to the raphe nuclei. METHODS: We prospectively enrolled 19 patients who had the first-ever acute isolated brainstem infarction in an observational cross-sectional study. All patients were evaluated by using the Montgomery Åsberg Depression Rating Scale (MADRS), the clinician-rated version of Apathy Evaluation Scale (AES-C) and Mini-Mental State Examination (MMSE)...
February 7, 2017: Journal of Affective Disorders
https://www.readbyqxmd.com/read/28277560/acute-necrotizing-encephalopathy-of-childhood-anec-a-case-report
#15
Afagh Hassanzadeh Rad, Vahid Aminzadeh
Acute Necrotizing Encephalopathy of childhood (ANEC) is a specific type of encephalopathy. After viral infection, it can be diagnosed by bilateral symmetrical lesions predominantly observed in thalami & brainstem of infants & children. Although, it is commonly occurred in Japanese and Taiwanese population. The goal of this article is to report a rare case of ANEC in a 15 months old girl infant from Thaleghani Hospital, Ramian, Gorgan, northern Iran.
2017: Iranian Journal of Child Neurology
https://www.readbyqxmd.com/read/28268687/impact-of-mild-traumatic-brain-injury-on-auditory-brain-stem-dysfunction-in-mouse-model
#16
Reza M Amanipour, Robert D Frisina, Samantha A Cresoe, Teresa J Parsons, Xiaoxia Zhu, Cesario V Borlongan, Joseph P Walton
The auditory brainstem response (ABR) is an electrophysiological test that examines the functionality of the auditory nerve and brainstem. Traumatic brain injury (TBI) can be detected if prolonged peak latency is observed in ABR measurements, since latency measures the neural conduction time in the brainstem, and an increase in latency can be a sign of pathological lesion at the auditory brainstem level. The ABR is elicited by brief sounds that can be used to measure hearing sensitivity as well as temporal processing...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28253561/the-neurology-of-acutely-failing-respiratory-mechanics
#17
Eelco F M Wijdicks
Forces involved in breathing-which effectively pull in air-are the diaphragmatic, intercostal, spine, and neck muscles. Equally important are the bulbar musculature maintaining the architecture of a patent airway conduit and abdominal wall and internal intercostal muscles providing cough. Acute injury along a neural trajectory from brainstem to muscle will impair the coordinated interaction between these muscle groups. Acutely failing respiratory mechanics can be caused by central and peripheral lesions. In central lesions, the key lesion is in the nucleus ambiguus innervating the dilator muscles of the soft palate, pharynx, and larynx, but abnormal respiratory mechanics rarely coincide with abnormalities of the respiratory pattern generator...
March 2, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28236065/glioblastoma-spheroids-produce-infiltrative-gliomas-in-the-rat-brainstem
#18
Zhiping Zhou, Neal Luther, Ranjodh Singh, John A Boockvar, Mark M Souweidane, Jeffrey P Greenfield
PURPOSE: Diffuse intrinsic pontine glioma (DIPG) is universally fatal without proven therapy other than radiation therapy for palliation. Representative animal models will play an essential role in the preclinical stage of future therapy development. To address the shortage of representative models, we created a novel infiltrative brainstem glioma model in rats based on glioblastoma spheroids. METHODS: Cells dissociated from glioblastoma spheroids grown from surgical specimens were implanted into the brainstem of NIH nude rats...
February 24, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28235898/gabaergic-neurons-of-the-central-amygdala-promote-cataplexy
#19
Carrie E Mahoney, Lindsay J Agostinelli, Jessica N K Brooks, Bradford B Lowell, Thomas E Scammell
Narcolepsy is characterized by chronic sleepiness and cataplexy - sudden muscle paralysis triggered by strong, positive emotions. Narcolepsy is caused by a lack of orexin (hypocretin) signaling, but little is known about the neural mechanisms that mediate cataplexy. The amygdala regulates responses to rewarding stimuli and contains neurons active during cataplexy. In addition, lesions of the amygdala reduce cataplexy. As GABAergic neurons of the central nucleus of the amygdala (CeA) target brainstem regions known to regulate muscle tone, we hypothesized that these cells promote emotion-triggered cataplexy...
February 24, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28226874/impact-of-mild-traumatic-brain-injury-on-auditory-brain-stem-dysfunction-in-mouse-model
#20
Reza M Amanipour, Robert D Frisina, Samantha A Cresoe, Teresa J Parsons, Xiaoxia Zhu, Cesario V Borlongan, Joseph P Walton, Reza M Amanipour, Robert D Frisina, Samantha A Cresoe, Teresa J Parsons, Xiaoxia Zhu, Cesario V Borlongan, Joseph P Walton, Robert D Frisina, Cesario V Borlongan, Reza M Amanipour, Xiaoxia Zhu, Samantha A Cresoe, Joseph P Walton, Teresa J Parsons
The auditory brainstem response (ABR) is an electrophysiological test that examines the functionality of the auditory nerve and brainstem. Traumatic brain injury (TBI) can be detected if prolonged peak latency is observed in ABR measurements, since latency measures the neural conduction time in the brainstem, and an increase in latency can be a sign of pathological lesion at the auditory brainstem level. The ABR is elicited by brief sounds that can be used to measure hearing sensitivity as well as temporal processing...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
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