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Pyramidal tract

Didem Yücel-Yılmaz, Emrah Yücesan, Dilek Yalnızoğlu, Kader Karlı Oğuz, Mahmut Şamil Sağıroğlu, Uğur Özbek, Esra Serdaroğlu, Başar Bilgiç, Sevim Erdem, Sibel Aylin Uğur İşeri, Haşmet Hanağası, Hakan Gürvit, Rıza Köksal Özgül, Ali Dursun
Hereditary spastic paraplegias (HSPs) are a group of genetic disorders resulting in pyramidal tract impairment, predominantly in lower limbs. KIF1C gene has recently been identified as one of the genetic causes of HSP and associated with pure or complicated HSP. We present three patients with complicated HSP from two unrelated families, who had early onset progressive cerebellar signs and developed pyramidal tract signs during follow-up. Whole exome sequencing in these patients followed by segregation analysis identified novel truncating KIF1C mutations (c...
March 12, 2018: Brain & Development
David T Fernandes Cabral, Georgios A Zenonos, Maximiliano Nuñez, Pinar Celtikci, Carl Snyderman, Eric Wang, Paul A Gardner, Juan C Fernandez-Miranda
BACKGROUND: The endoscopic endonasal approach (EEA) has been proposed as a potential alternative for ventral brainstem lesions. The surgical anatomy, feasibility, and limitations of the EEA for intrinsic brainstem lesions are still poorly understood. OBJECTIVE: To describe the surgical planning, anatomy, and technique of an intrinsic pontine glioma operated via EEA. METHODS: Six-human brainstems were prepared for white matter microdissection...
March 12, 2018: Operative Neurosurgery (Hagerstown, Md.)
Ning Chen, Michael Chopp, Ye Xiong, Jian-Yong Qian, Mei Lu, Dong Zhou, Li He, Zhongwu Liu
In addition to thrombolysis, tissue plasminogen activator (tPA) can evoke neurorestorative processes. We therefore investigated the therapeutic effect of subacute intranasal administration of tPA post stroke on neurological recovery and on corticospinal innervation in mice. A transgenic mouse line, in which the pyramidal neurons and corticospinal tract (CST) axons are specifically labeled by yellow fluorescent protein (YFP) was employed. Adult CST-YFP mice were subjected to right unilateral middle cerebral artery occlusion (MCAo), and were randomly divided into groups treated with saline or tPA intranasally in the subacute phase...
March 5, 2018: Experimental Neurology
Miran Skrap, Maria Caterina Vescovi, Giada Pauletto, Marta Maieron, Tomasino Barbara, Daniele Bagatto, Francesco Tuniz
BACKGROUND: Cavernous malformations (CMs) are congenital malformations and may be located anywhere in the brain. We present a series of CMs located close to or inside of the motor-sensory cortex or corticospinal tract (CST) with clinical onset due to hemorrhage or mass effect. In such cases, surgery becomes an acceptable option. OBJECTIVE: To evaluate the role of diffusion tensor imaging (DTI), functional-magnetic-resonance imaging (fMRI), intraoperative neurophysiological monitoring, neuronavigation, and brain-mapping and the clinical results of surgical treatment of CMs in this critical location...
February 16, 2018: Operative Neurosurgery (Hagerstown, Md.)
Diane F van Rappard, Marsh Königs, Marjan E Steenweg, Jaap Jan Boelens, Jaap Oosterlaan, Marjo S van der Knaap, Nicole I Wolf, Petra J W Pouwels
OBJECTIVE: We aimed to gain more insight into the pathomechanisms of metachromatic leukodystrophy (MLD), by comparing magnitude and direction of diffusion between patients and controls at diagnosis and during follow-up. METHODS: Four late-infantile, 16 juvenile and 8 adult onset MLD patients [of which 13 considered eligible for hematopoietic cell transplantation (HCT)] and 47 controls were examined using diffusion tensor imaging. Fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD) were quantified and compared between groups using tract-based spatial statistics (TBSS)...
January 30, 2018: Journal of Neurology
Franca Wagner, David S Titelbaum, Renate Engisch, Emily K Coskun, Jeff L Waugh
PURPOSE: Hereditary spastic paraplegia (HSP) and hereditary spastic ataxia (HSA) are a heterogeneous group of genetic disorders characterized by progressive lower limb spasticity resulting from pyramidal tract dysfunction. By identifying critical imaging findings within the clinical context of spasticity, radiologists are uniquely positioned to recommend specific genetic testing, and thus facilitate diagnosis. METHODS: We present two examples of HSP and HSA that had gone clinically unrecognized for years, and in which magnetic resonance imaging played a critical role in the diagnosis...
January 29, 2018: Clinical Neuroradiology
Pengfei Lin, Dong Zhang, Guangrun Xu, Chuanzhu Yan
Spinocerebellar ataxias (SCAs) are a group of autosomal dominant, clinically heterogeneous neurodegenerative disorders. SCA18 is a rare autosomal dominant sensory/motor neuropathy with ataxia (OMIM#607458) associated with a single missense variant c.514 A>G in the interferon related developmental regulator 1 (IFRD1) gene previously reported in a five-generation American family of Irish origin. However, to date, there have been no other reports of the IFRD1 mutation to confirm its role in SCA. Here, we report a Han Chinese family with SCA18; the family members presented with a slowly progressing gait ataxia, pyramidal tract signs, and peripheral neuropathy...
January 23, 2018: Journal of Human Genetics
Michael Gurevich, Roy Waknin, Evan Stone, Anat Achiron
AIMS: Fingolimod hydrochloride is an effective immunomodulatory drug in improving relapsing-remitting multiple sclerosis (RRMS). However, data on the neuroradiologic effects on white matter (WM) have not been demonstrated. In this study, we aimed elucidating the impact of 1-year fingolimod treatment on WM integrity in patients with RRMS. METHODS: Diffusion tensor imaging (DTI) was applied to assess axonal and myelin integrity in specific WM tracts of patients with RRMS prior to and 1 year postfingolimod treatment (n = 30)...
January 5, 2018: CNS Neuroscience & Therapeutics
Lorenzo Ugga, Cinzia Coppola, Sirio Cocozza, Dario Saracino, Ferdinando Caranci, Francesco Tuccillo, Elisabetta Signoriello, Sara Casertano, Giuseppe Di Iorio, Enrico Tedeschi
Motor neuron disease (MND) is a neurodegenerative disease determining progressive and relentless motor deterioration involving both upper and lower motor neurons (UMN and LMN); several variants at onset are described. Here we describe a case of MND presenting as pure spastic monoparesis in which magnetic resonance imaging (MRI) gave a substantial contribution in confirming the diagnosis and assessing the severity of UMN involvement. An isolated pyramidal syndrome, with complete absence of LMN signs, is a rare phenotype in the context of MND (less than 4% of total cases), especially if restricted to only one limb...
December 2017: Quantitative Imaging in Medicine and Surgery
Ricardo P Vaz, Armando Cardoso, Paula Serrão, Pedro A Pereira, M Dulce Madeira
A recent study reported that the integrity of the nucleus of the lateral olfactory tract (nLOT) is required for normal olfaction and for the display of odor-driven behaviors that are critical for species survival and reproduction. In addition to being bi-directionally connected with a key element of the neural circuitry that mediates stress response, the basolateral nucleus of the amygdala, the nLOT is a potential target for glucocorticoids as its cells express glucocorticoid receptors. Herein, we have addressed this hypothesis by exploring, first, if chronic variable stress (CVS) disrupts odor detection and discrimination, and innate olfactory-driven behaviors, namely predator avoidance, sexual behavior and aggression in male rats...
January 4, 2018: Hormones and Behavior
Takumi Takeuchi, Masahito Takahashi, Kazuhiko Satomi, Hideaki Ohne, Atsushi Hasegawa, Shunsuke Sato, Shoichi Ichimura
The objective of this study was to electrophysiologically assess the corticospinal tracts of adult rats and the recovery of motor function of their forelimbs after cervical cord hemisection. Of 39 adult rats used, compound muscle action potentials (CMAPs) of the forelimbs of 15 rats were evaluated, before they received left C5 segmental hemisection of the spinal cord, by stimulating the pyramid of the medulla oblongata on one side using an exciting microelectrode. All 15 rats exhibited contralateral electrical activity, but their CMAPs disappeared after hemisection...
2017: Behavioural Neurology
Jian Mao
Although there are unified criteria for the clinical diagnosis and grading of neonatal hypoxic-ischemic encephalopathy (HIE), clinical features and neuropathological patterns vary considerably among the neonates with HIE due to birth asphyxia in the same classification. The patterns and progression of brain injury in HIE, which is closely associated with long-term neurodevelopment outcomes, can be well shown on magnetic resonance imaging (MRI), but different sequences may lead to different MRI findings at the same time...
December 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
Masharip Atadzhanov, Danielle C Smith, Mwila H Mwaba, Omar K Siddiqi, Alan Bryer, L Jacquie Greenberg
Background: To date, 43 types of Spinocerebellar Ataxias (SCAs) have been identified. A subset of the SCAs are caused by the pathogenic expansion of a CAG repeat tract within the corresponding gene. Ethnic and geographic differences are evident in the prevalence of the autosomal dominant SCAs. Few descriptions of the clinical phenotype and molecular genetics of the SCAs are available from the African continent. Established studies mostly concern the South African populations, where there is a high frequency of SCA1, SCA2 and SCA7...
2017: Cerebellum & Ataxias
Muhammad Waqas, Syed Ather Enam, Fauzan A Hashmi, Fatima Mubarak, Fazal Arain
High-grade gliomas possess internal pathological heterogeneity. Selective sampling of different tumor regions can help in the study of this heterogeneity. In this report, we have described the use of a novel navigation and optical system for the selective regional sampling of a high-grade glioma lesion. A 45-year-old gentleman presented to us with complaints of intermittent frontal headaches for past eight months. On examination, he had subtle pyramidal weakness in left upper and lower extremities. Magnetic resonance imaging (MRI) showed a large contrast-enhancing, space-occupying lesion in the right frontal lobe causing perilesional edema and midline shift...
October 2, 2017: Curēus
Jonathan Levy, Sarah Hartley, Elsa Mauruc-Soubirac, Antoine Leotard, Frederic Lofaso, Maria-Antonia Quera-Salva, Djamel Bensmail
BACKGROUND: Spasticity and spasms are distressing features of the upper motor neuron syndrome (UMNS) following spinal cord injuries (SCI) or multiple sclerosis (MS), and have common therapeutic implications. Despite an increase of antispastic drugs and strategies, sometimes up to the surgical implant of intrathecal baclofen pump, some patients still complain of disabling spasms, which poses diagnostic and therapeutic challenges. Although clinically similar, flexor spasms due to pyramidal tract disruption must be clearly differentiated from periodic limb movements (PLM), often accompanying restless leg syndrome (RLS) and occurring during sleep...
December 4, 2017: European Journal of Physical and Rehabilitation Medicine
Thomas J Whitford, Lena K L Oestreich, Judith M Ford, Brian J Roach, Rachel L Loewy, Barbara K Stuart, Daniel H Mathalon
Self-generated speech produces a smaller N1 amplitude in the auditory-evoked potential than externally generated speech; this phenomenon is known as N1-suppression. Schizophrenia patients show less N1-suppression than healthy controls. This failure to self-suppress may underlie patients' characteristic tendency to misattribute self-generated thoughts and actions to external sources. While the cause of N1-suppression deficits to speech in schizophrenia remains unclear, structural damage to the arcuate fasciculus is a candidate, due to its ostensible role in transmitting the efference copy of the motor plan to speak...
November 29, 2017: Schizophrenia Bulletin
Yusuke Fukui, Nozomi Hishikawa, Kota Sato, Yumiko Nakano, Ryuta Morihara, Jingwei Shang, Mami Takemoto, Yasuyuki Ohta, Toru Yamashita, Koji Abe
The objective of this study was to determine alteration of corticospinal tract in patients with amyotrophic lateral sclerosis (ALS) using diffusion tensor tractograhy (DTT) focusing on the cervical spinal cord (C5) and transcranial magnetic stimulation (TMS). We recruited 38 ALS, 6 spinal and bulbar muscular atrophy (SBMA), 7 spastic paraplegia (SP) patients, and 8 age-matched normal controls, and then ALS were divided into two subgroups according to their clinical type: 28 ALS-limb and 10 ALS-bulbar. DTT was performed using the diffusion tensor image (DTI) track module to reconstruct two fiber tracts via C5...
November 22, 2017: Neuroscience Research
Fumio Yamaguchi, Hirotomo Ten, Tadashi Higuchi, Tomoko Omura, Toyoyuki Kojima, Koji Adachi, Takayuki Kitamura, Shiro Kobayashi, Hiroshi Takahashi, Akira Teramoto, Akio Morita
Intraoperative 3D recognition of the motor tract is indispensable to avoiding neural fiber injury in brain tumor surgery. However, precise localization of the tracts is sometimes difficult with conventional mapping methods. Thus, the authors developed a novel brain mapping method that enables the 3D recognition of the motor tract for intrinsic brain tumor surgeries. This technique was performed in 40 consecutive patients with gliomas adjacent to motor tracts that have a risk of intraoperative pyramidal tract damage...
November 24, 2017: Journal of Neurosurgery
L Chimelli, E Avvad-Portari
BACKGROUND: A relationship between Zika virus (ZikV) infection in pregnancy and the occurrence of microcephaly was established during the Zika outbreak in Brazil (2015-2016). Neuropathological findings in congenital Zika syndrome helped to understand its pathogenetic mechanisms. RESULTS: The most relevant postmortem findings in the central nervous system (CNS) of fetuses and neonates infected with ZikV early in gestation are microcephaly with ex-vacuo ventriculomegaly and large head circumference associated with obstructive hydrocephalus due to severe midbrain and aqueduct distortion...
January 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
Gordon M Shepherd, Timothy B Rowe
The neocortex is characterized by lamination of its neuron cell bodies in six layers, but there are few clues as to how this comes about and what is its function. Recent studies provide evidence that evolution from simple three-layer cortex may give insight into this problem. Three-layer cortex arose in the olfactory, hippocampal and dorsal cortex of the early amniote forebrain based on a cortical module of excitatory and inhibitory inputs to an intratelencephalic (IT) type of pyramidal neuron with feedback excitation and inhibition and related interneurons...
2017: Frontiers in Neuroanatomy
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