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https://www.readbyqxmd.com/read/28801086/slowly-progressive-leukodystrophy-in-an-adolescent-male-with-phosphoglycerate-kinase-deficiency
#1
Shimpei Baba, Ayumi Kobayashi, Haruna Yokoyama, Kengo Moriyama, Ayako Kashimada, Jun Oyama, Ayako Owada, Shoichi Oyama, Tomohiro Morio, Masatoshi Takagi
We report the case of an 18-year-old man with a phosphoglycerate kinase (PGK) deficiency who had slowly progressive leukodystrophy during adolescence. The patient had a history of severe neonatal jaundice, hemolytic crisis with rhabdomyolysis triggered by febrile viral infections, dysarthria, and intellectual disability during early childhood. Clumsiness in walking and writing became obvious at ∼10years of age. Evaluations performed by us on the 18-year-old patient confirmed the presence of pyramidal tract signs, increased muscle tone, and generalized dystonia...
August 8, 2017: Brain & Development
https://www.readbyqxmd.com/read/28779978/effect-of-prenatal-exposure-to-ethanol-on-the-pyramidal-tract-in-developing-rats
#2
Michael W Miller
Prenatal exposure to ethanol induces a relative increase in the numbers of pyramidal tract axons relative to the number of corticospinal projection neurons in somatosensory/motor cortices in the adult rat. The present study examines the effects of ethanol on the numbers of axons in the developing caudal pyramidal tract, i.e., corticospinal axons. Electron microscopic analyses of the pyramidal tracts of the offspring of pregnant rat dams fed a control diet ad libitum, pair-fed a liquid control diet, or fed an ethanol-containing diet ad libitum were performed...
August 3, 2017: Brain Research
https://www.readbyqxmd.com/read/28769756/an-lmnb1-duplication-caused-adult-onset-autosomal-dominant-leukodystrophy-in-chinese-family-clinical-manifestations-neuroradiology-and-genetic-diagnosis
#3
Yi Dai, Yaling Ma, Shengde Li, Santasree Banerjee, Shengran Liang, Qing Liu, Yinchang Yang, Bin Peng, Liying Cui, Liri Jin
Autosomal dominant adult-onset demyelinating leukodystrophy (ADLD) is a very rare neurological disorder featured with late onset, slowly progressive central nervous system demyelination. Duplication or over expression of the lamin B1 (LMNB1) gene causes ADLD. In this study, we undertook a comprehensive clinical evaluation and genetic detection for a Chinese family with ADLD. The proband is a 52-year old man manifested with autonomic abnormalities, pyramidal tract dysfunction. MRI brain scan identified bilateral symmetric white matter (WM) hyper-intensities in periventricular and semi-oval WM, cerebral peduncles and middle cerebellar peduncles...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28738366/neuroanatomical-correlates-of-hypophonia-in-subcortical-vascular-cognitive-impairment
#4
Ji Hye Yoon, Yisuh Ahn, Sook Hui Kim, Juhee Chin, Seongbeom Park, Duk L Na
BACKGROUND/AIMS: Early detection and intervention may alter the disease course of subcortical vascular cognitive impairment (SVCI). Patients with SVCI have white matter ischemia that disrupts connections between the cortex and subcortical gray matter and therefore manifest various symptoms such as motor disturbances and behavioral/cognitive dysfunction. Reduced vocal loudness, or hypophonia, is one of the common motor symptoms of SVCI, but few studies have systematically investigated it in this patient population...
2017: European Neurology
https://www.readbyqxmd.com/read/28735100/attention-modulates-specific-motor-cortical-circuits-recruited-by-transcranial-magnetic-stimulation
#5
J L Mirdamadi, L Y Suzuki, S K Meehan
Skilled performance and acquisition is dependent upon afferent input to motor cortex. The present study used short-latency afferent inhibition (SAI) to probe how manipulation of sensory afference by attention affects different circuits projecting to pyramidal tract neurons in motor cortex. SAI was assessed in the first dorsal interosseous muscle while participants performed a low or high attention-demanding visual detection task. SAI was evoked by preceding a suprathreshold transcranial magnetic stimulus with electrical stimulation of the median nerve at the wrist...
July 20, 2017: Neuroscience
https://www.readbyqxmd.com/read/28726023/benefits-and-risks-of-non-approved-injection-regimens-for-botulinum-toxins-in-spasticity
#6
Andrea Santamato, Francesco Panza
Spasticity with muscle paresis and loss of dexterity is a common feature of upper motor neuron syndrome due to injuries or the pyramidal tract in several neurological conditions. Botulinum toxin type A has been considered the gold standard treatment for spasticity and movement disorders, with efficacy, reversibility, and low prevalence of complications. During the last 30 years, thousands of studies of its use have been performed, but few guidelines are available. Therefore, there is great variability in both the doses and intervals of administration and the approaches taken by clinicians with considerable experience in spasticity and movement disorder treatment...
July 19, 2017: Drugs
https://www.readbyqxmd.com/read/28701996/aberrant-pyramidal-tract-in-comparison-with-pyramidal-tract-on-diffusion-tensor-tractography-a-mini-review
#7
REVIEW
Sungho Jang, Soyoung Kwak
The pyramidal tract (PT) is a major neural tract that controls voluntary movements in the human brain. The PT has several collateral pathways, including the aberrant pyramidal tract (APT), which passes through the medial lemniscus location at the midbrain and pons. Diffusion tensor tractography (DTT) allows visualization and estimation of the APT in three dimensions. In this mini-review, eight DTT studies on the APT were reviewed. Two studies for normal subjects reported the prevalence (17-18% of hemispheres) and the different characteristics (different cortical origin, less directionality, and fewer neural fibers) of the APT compared with the PT...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28644906/cholinesterases-in-normal-and-alzheimer-s-disease-primary-olfactory-gyrus
#8
Hayam Hamodat, Meghan K Cash, John D Fisk, Sultan Darvesh
AIMS: Alzheimer's disease (AD) is characterized by cholinergic dysfunction and deposition of β-amyloid (Aβ) plaques and tau neurofibrillary tangles (NFTs) in the brain. Olfactory abnormalities often precede cognitive symptoms in AD, indicating early involvement of pathology in olfactory structures. The cholinergic system is important not only in cognition but also in modulation of the olfactory system. The primary olfactory gyrus (POG) is comprised of the olfactory tract, anterior olfactory nucleus (AON) and olfactory area (OA)...
June 23, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28641772/susac-syndrome-a-differential-diagnosis-of-white-matter-lesions
#9
Mariano Marrodan, Jorge Correale, Lucas Alessandro, Mariela Amaya, Maria Eugenia Fracaro, Alejandro Alfredo Köhler, Marcela Fiol
INTRODUCTION: Susac Syndrome is an autoimmune endotheliopathy affecting capillaries and precapillary arterioles of the brain, inner ear and retina. The classic symptom triad includes visual disturbances, hypoacusia, and encephalopathy, but is rarely fully manifest at onset. The syndrome typically follows an active fluctuating monophasic course. Typical imaging findings on brain magnetic resonance show central fiber microischemias/microinfarctions of the corpus callosum known as "snow balls", as well as lineal infarcts and upper callosal fiber involvement...
July 2017: Multiple Sclerosis and related Disorders
https://www.readbyqxmd.com/read/28638211/psychogenic-blepharospasm-a-diagnostic-dilemma
#10
Soumitra DAS, Roopchand Pandrantil Sreedharan, Prasanth Sudhakaran Remadevi, Cheruvallil Velayudhan Saji
Blepharospasm is an uncontrolled spasmodic contraction of the orbicularis muscles of the eye resulting in an abnormal tic or twitch of eyes. It usually lasts for seconds to minutes but in severe cases eyes may be closed for hours. It may be essentially benign or secondary due to a lesion in basal ganglia, pyramidal tract, and trauma, local pathology in the eyes or drug induced. Here we are presenting a case of psychogenic blepharospasm. A 65 year old woman presented with a history of episodic inability of opening her eyes for the past 6 months...
December 25, 2016: Shanghai Archives of Psychiatry
https://www.readbyqxmd.com/read/28627942/diffusion-imaging-of-mild-traumatic-brain-injury-in-the-impact-accelerated-rodent-model-a-pilot-study
#11
Zora Kikinis, Marc Muehlmann, Ofer Pasternak, Sharon Peled, Praveen Kulkarni, Craig Ferris, Sylvain Bouix, Yogesh Rathi, Inga K Koerte, Steve Pieper, Alexander Yarmarkovich, Caryn L Porter, Bruce S Kristal, Martha E Shenton
PRIMARY OBJECTIVE: There is a need to understand pathologic processes of the brain following mild traumatic brain injury (mTBI). Previous studies report axonal injury and oedema in the first week after injury in a rodent model. This study aims to investigate the processes occurring 1 week after injury at the time of regeneration and degeneration using diffusion tensor imaging (DTI) in the impact acceleration rat mTBI model. RESEARCH DESIGN: Eighteen rats were subjected to impact acceleration injury, and three rats served as sham controls...
June 19, 2017: Brain Injury: [BI]
https://www.readbyqxmd.com/read/28619087/longitudinal-estimation-of-intramuscular-tibialis-anterior-coherence-during-subacute-spinal-cord-injury-relationship-with-neurophysiological-functional-and-clinical-outcome-measures
#12
Elisabeth Bravo-Esteban, Julian Taylor, Manuel Aleixandre, Cristina Simón-Martínez, Diego Torricelli, Jose Luis Pons, Gerardo Avila-Martín, Iriana Galán-Arriero, Julio Gómez-Soriano
BACKGROUND: Estimation of surface intramuscular coherence has been used to indirectly assess pyramidal tract activity following spinal cord injury (SCI), especially within the 15-30 Hz bandwidth. However, change in higher frequency (>40 Hz) muscle coherence during SCI has not been characterised. Thus, the objective of this study was to identify change of high and low frequency intramuscular Tibialis Anterior (TA) coherence during incomplete subacute SCI. METHODS: Fifteen healthy subjects and 22 subjects with motor incomplete SCI (American Spinal Injury Association Impairment Scale, AIS, C or D grade) were recruited and tested during 4 sessions performed at 2-week intervals up to 8 months after SCI...
June 15, 2017: Journal of Neuroengineering and Rehabilitation
https://www.readbyqxmd.com/read/28606574/dietary-habits-concentration-of-copper-zinc-and-cu-to-zn-ratio-in-serum-and-ability-status-of-patients-with-relapsing-remitting-multiple-sclerosis
#13
Katarzyna Socha, Elżbieta Karpińska, Jan Kochanowicz, Jolanta Soroczyńska, Marta Jakoniuk, Marianna Wilkiel, Zenon D Mariak, Maria H Borawska
OBJECTIVE: Dietary habits and adequate intake of antioxidants in the diet-for example, copper (Cu) and zinc (Zn)-may be an environmental factor in the occurrence of multiple sclerosis (MS). The aim of this study was to estimate the influence of dietary habits on the concentration of Cu, Zn in the serum, and the effect of Cu-to-Zn ratio on the ability status of patients with relapsing-remitting MS. METHODS: This was an observational case-control study that included 101 individuals with MS and 68 healthy individuals (controls)...
July 2017: Nutrition
https://www.readbyqxmd.com/read/28598277/surgical-strategy-to-avoid-ischemic-complications-of-the-pyramidal-tract-in-resective-epilepsy-surgery-of-the-insula-technical-case-report
#14
Naoki Ikegaya, Akio Takahashi, Takanobu Kaido, Yuu Kaneko, Masaki Iwasaki, Nobutaka Kawahara, Taisuke Otsuki
Surgical treatment of the insula is notorious for its high probability of motor complications, particularly when resecting the superoposterior part. Ischemic damage to the pyramidal tract in the corona radiata has been regarded as the cause of these complications, resulting from occlusion of the perforating arteries to the pyramidal tract through the insular cortex. The authors describe a strategy in which a small piece of gray matter is spared at the bottom of the periinsular sulcus, where the perforating arteries pass en route to the pyramidal tract, in order to avoid these complications...
June 9, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28590508/pyramidal-tract-activation-due-to-subthalamic-deep-brain-stimulation-in-parkinson-s-disease
#15
Philipp Mahlknecht, Harith Akram, Dejan Georgiev, Elina Tripoliti, Joseph Candelario, Andre Zacharia, Ludvic Zrinzo, Jonathan Hyam, Marwan Hariz, Thomas Foltynie, John C Rothwell, Patricia Limousin
BACKGROUND: Subthalamic deep brain stimulation (STN-DBS) is an effective treatment for Parkinson's disease (PD), but can have side effects caused by stimulus spread to structures outside the target volume such as the pyramidal tract. OBJECTIVES: To assess the relevance of pyramidal tract activation with STN-DBS in PD. METHODS: In a multimodal, blinded study in 20 STN-DBS patients, we measured stimulation thresholds for evoking electromyographic activity in orbicularis oris and first dorsal interosseous muscles at each of 150 electrode sites...
June 7, 2017: Movement Disorders: Official Journal of the Movement Disorder Society
https://www.readbyqxmd.com/read/28562670/increased-threshold-of-short-latency-motor-evoked-potentials-in-transgenic-mice-expressing-channelrhodopsin-2
#16
Wei Wu, Wenhui Xiong, Ping Zhang, Lifang Chen, Jianqiao Fang, Christopher Shields, Xiao-Ming Xu, Xiaoming Jin
Transgenic mice that express channelrhodopsin-2 or its variants provide a powerful tool for optogenetic study of the nervous system. Previous studies have established that introducing such exogenous genes usually does not alter anatomical, electrophysiological, and behavioral properties of neurons in these mice. However, in a line of Thy1-ChR2-YFP transgenic mice (line 9, Jackson lab), we found that short-latency motor evoked potentials (MEPs) induced by transcranial magnetic stimulation had a longer latency and much lower amplitude than that of wild type mice...
2017: PloS One
https://www.readbyqxmd.com/read/28560845/spinocerebellar-ataxia-type-10-common-haplotype-and-disease-progression-rate-in-peru-and-brazil
#17
T C Gheno, G V Furtado, J A M Saute, K C Donis, A M V Fontanari, V E Emmel, J L Pedroso, O Barsottini, C Godeiro-Junior, H van der Linden, E Ternes Pereira, V P Cintra, W Marques, R M de Castilhos, I Alonso, J Sequeiros, M Cornejo-Olivas, P Mazzetti, V B Leotti, L B Jardim, M L Saraiva-Pereira
BACKGROUND AND PURPOSE: Spinocerebellar ataxia type 10 is a neurodegenerative disorder that is due to an expanded ATTCT repeat tract in the ATXN10 gene. Our aim was to describe clinical characteristics and intragenic haplotypes of patients with spinocerebellar ataxia type 10 from Brazil and Peru. METHODS: Expanded alleles were detected by repeat-primed polymerase chain reaction. Disease progression was measured by the Scale for the Assessment and Rating of Ataxia, and the Neurological Examination Score for Spinocerebellar Ataxias when possible...
July 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28544025/phase-imaging-of-axonal-integrity-of-cranial-corticospinal-tract-in-experimental-spinal-cord-injury-at-9-4t
#18
Genxia He, Junchao Qian
It was known that Spinal cord injury (SCI) leads to neuronal cell death, axonal damage and demyelination, and often results in severe functional loss. Noninvasive imaging of axonal integrity may hold great importance in clinic. This study evaluated the role of phase imaging in detection of axonal integrity of cranial corticospinal tract after spinal cord injury. Quantitative MR phase imaging and diffusion tensor imaging were performed on a group of rats prior to and after spinal cord injury at 9.4 T in this study...
May 23, 2017: Microscopy Research and Technique
https://www.readbyqxmd.com/read/28543952/structural-signature-of-classical-versus-late-onset-friedreich-s-ataxia-by-multimodality-brain-mri
#19
Thiago Junqueira R Rezende, Alberto Rolim M Martinez, Ingrid Faber, Karen Girotto, José Luiz Pedroso, Orlando G Barsottini, Iscia Lopes-Cendes, Fernando Cendes, Andreia V Faria, Marcondes C França
INTRODUCTION: Friedreich's ataxia (FRDA) is the most common autosomal-recessive ataxia worldwide. It is characterized by early onset, sensory abnormalities, and slowly progressive ataxia. However, some individuals manifest the disease after the age of 25 years and are classified as late-onset FRDA (LOFA). Therefore, we propose a transversal multimodal MRI-based study to investigate which anatomical substrates are involved in classical (cFRDA) and LOFA. METHODS: We enrolled 36 patients (13 with LOFA) and 29 healthy controls...
May 23, 2017: Human Brain Mapping
https://www.readbyqxmd.com/read/28529055/intraoperative-mapping-and-monitoring-of-the-pyramidal-tract-using-endoscopic-depth-electrodes
#20
Yukinori Akiyama, Shunya Ohtaki, Katsuya Komatsu, Kentaro Toyama, Rei Enatsu, Takeshi Mikami, Masahiko Wanibuchi, Nobuhiro Mikuni
OBJECTIVE: To evaluate motor function during neuroendoscopic resectioning of deep-seated brain tumors using endoscopically guided depth electrodes. METHODS: For 12 cases of thalamic tumors including high-grade gliomas, germinomas, and malignant lymphomas, depth electrodes were inserted using endoscopic guides between the tumor and the pyramidal tract in the thalamus. Motor evoked potentials (MEPs) were continuously recorded during neuroendoscopic resectioning of the tumors...
May 18, 2017: World Neurosurgery
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