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cerebral atrophy

Mathieu Verdurand, Elise Levigoureux, Sophie Lancelot, Waël Zeinyeh, Thierry Billard, Isabelle Quadrio, Armand Perret-Liaudet, Luc Zimmer, Fabien Chauveau
The accumulation of aggregated alpha-synuclein ( α -syn) in multiple brain regions is a neuropathological hallmark of synucleinopathies. Multiple system atrophy (MSA) is a synucleinopathy characterized by the predominant cerebral accumulation of aggregated α -syn as cytoplasmic glial inclusions (CGI). A premortem diagnosis tool would improve early diagnosis and help monitoring disease progression and therapeutic efficacy. One Positron Emission Tomography (PET) study suggested [11 C]BF-227 as a promising radiotracer for monitoring intracellular α -syn deposition in MSA patients...
2018: Contrast Media & Molecular Imaging
Rumiko Takayama, Katsumi Imai, Hiroko Ikeda, Koichi Baba, Naotaka Usui, Yukitoshi Takahashi, Yushi Inoue
We describe two cases of refractory epilepsy with cerebral hemiatrophy and contralateral electroencephalographic (EEG) abnormalities, in which hemispherotomy of the atrophic hemisphere effectively controlled seizures. Case 1 was a 5-year-1-month-old girl with refractory bilateral asymmetrical tonic posturing seizures predominantly in the right arm. Magnetic resonance imaging showed left porencephaly corresponding to a left middle cerebral artery infarction. Case 2 was a 3-year-8-month-old boy with refractory bilateral asymmetrical tonic posturing seizures predominantly in the right arm due to atrophy of the left cerebral hemisphere after septic meningitis...
March 14, 2018: Brain & Development
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
Maria Luíza de Medeiros Rêgo, Daniel Aranha Rego Cabral, Eduardo Bodnariuc Fontes
Heart Failure is a clinical syndrome prevalent throughout the world and a major contribution to mortality of cardiac patients in Brazil. In addition, this pathology is strongly related to cerebral dysfunction, with a high prevalence of cognitive impairment. Many mechanisms may be related to cognitive loss, such as cerebral hypoperfusion, atrophy and loss of gray matter of the brain, and dysfunction of the autonomic nervous system. The literature is clear regarding the benefits of aerobic physical activity in healthy populations in the modulation of the autonomic nervous system and in brain functions...
January 2018: Arquivos Brasileiros de Cardiologia
Mariko Nishibe, Yu Katsuyama, Toshihide Yamashita
The motor deficit of the reeler mutants has largely been considered cerebellum related, and the developmental consequences of the cortex on reeler motor behavior have not been examined. We herein showed that there is a behavioral consequence to reeler mutation in models examined at cortex-dependent bimanual tasks that require forepaw dexterity. Using intracortical microstimulation, we found the forelimb representation in the motor cortex was significantly reduced in the reeler. The reeler cortex required a significantly higher current to evoke skeletal muscle movements, suggesting the cortical trans-synaptic propagation is disrupted...
March 13, 2018: Brain Structure & Function
Crystal White, Jeremy Mortier, Ranieri Verin, Thomas Maddox, Rita Goncalves, Daniel Sanchez-Masian
Case summary: A 2-year-old male domestic shorthair cat presented to the University of Liverpool Small Animal Teaching Hospital with a 2 week history of altered mentation, blindness and focal epileptic seizures. MRI examination revealed generalised cerebral and cerebellar atrophy, diffuse T2-weighted hyperintensity of the white matter and meningeal thickening. Neuronal ceroid lipofuscinosis was confirmed on post-mortem examination. Relevance and novel information: This is the first report of the MRI findings of neuronal ceroid lipofuscinosis in a cat...
January 2018: JFMS Open Reports
Sándor Csizmadia, Erika Vörös
Cerebral amyloid angiopathy (CAA) is most commonly recognized by β-amyloid deposition in the small and medium sized vessels of the brain. The 71-year-old female presented with a sudden onset of vertigo and headache. By native computer tomography (CT) examination we found cerebral atrophy and the sign of chronic vascular injury. The complaints of the patient worsened, thus magnetic resonance imaging (MRI) was performed. The MRI scan revealed a bleeding transformation of an ischemic lesion in the right occipital region...
March 2018: Orvosi Hetilap
Sandhitsu R Das, Long Xie, Laura E M Wisse, Ranjit Ittyerah, Nicholas J Tustison, Bradford C Dickerson, Paul A Yushkevich, David A Wolk
We examined the relationship between in vivo estimates of tau deposition as measured by18 F-AV-1451 tau positron emission tomography imaging and cross-sectional cortical thickness, as well as rates of antecedent cortical thinning measured from magnetic resonance imaging in individuals with and without evidence of cerebral amyloid in 63 participants from the Alzheimer's Disease Neuroimaging Initiative study, including 32 cognitively normal individuals (mean age 74 years), 27 patients with mild cognitive impairment (mean age 76...
February 9, 2018: Neurobiology of Aging
N Crespo-Eguilaz, P D Dominguez, M Vaquero, J Narbona
AIM: To contribute to neuropsychological profiling of developmental amnesia subsequent to bilateral damage to both hippocampi in early age. SUBJECTS AND METHODS: The total sample of 24 schoolchildren from both sexes is distributed in three groups: perinatal hypoxic-ischaemic encephalopathy and everyday complaints of memory in school age (n = 8); perinatal hypoxic-ischaemic encephalopathy without memory complaints (n = 7); and a group of typically developing (n = 9)...
March 1, 2018: Revista de Neurologia
Maurizio Gallucci, Cinzia Piovesan, Maria Elena Di Battista
BACKGROUND: Frailty is a condition which is characterized by a reduction in the homeostatic reserves of the individual and which entails an increased vulnerability to stressful endogenous and exogenous agents. The Frailty Index (FI), proposed by Rockwood, was designed following an accumulation of deficits model: the greater the number of deficits in a given individual, the greater the degree of frailty. OBJECTIVE: The aim of this study was to verify the existence of associations between FI and cerebral atrophy...
February 28, 2018: Journal of Alzheimer's Disease: JAD
Serafeim Katsavos, Alasdair Coles
Alemtuzumab, the first monoclonal antibody to be used as a therapy and the first to be humanized, was introduced into the treatment of multiple sclerosis in 1991 after its successful use in hematology, oncology, and transplantation medicine. One phase 2 and two phase 3 trials of this lymphocyte-depleting agent have established alemtuzumab's superior efficacy to interferon β-1a over the short term (2-3 years) with greater relapse rate reduction, reduced accumulation of disability, and more frequent sustained improvement in disability...
March 2, 2018: Cold Spring Harbor Perspectives in Medicine
Abdullah Bin Zahid, David Balser, Rebekah Thomas, Margaret Y Mahan, Molly E Hubbard, Uzma Samadani
OBJECTIVE Chronic subdural hematoma (cSDH) is a highly morbid condition associated with brain atrophy in the elderly. It has a reported 30% 1-year mortality rate. Approximately half of afflicted individuals report either no or relatively unremarkable trauma preceding their diagnosis, raising the possibility that cSDH is a manifestation of degenerative or inflammatory disease rather than trauma. The purpose of this study was to compare the rates of cerebral atrophy before and after cSDH to determine whether it is more likely that cSDH causes atrophy or that atrophy causes cSDH...
March 2, 2018: Journal of Neurosurgery
Lucy V Hiscox, Curtis L Johnson, Matthew D J McGarry, Michael Perrins, Aimee Littlejohn, Edwin J R van Beek, Neil Roberts, John M Starr
Volumetric structural magnetic resonance imaging (MRI) is commonly used to determine the extent of neuronal loss in aging, indicated by cerebral atrophy. The brain, however, exhibits other biophysical characteristics such as mechanical properties, which can be quantified with magnetic resonance elastography (MRE). MRE is an emerging noninvasive imaging technique for measuring viscoelastic tissue properties, proven to be sensitive metrics of neural tissue integrity, as described by shear stiffness, μ and damping ratio, and ξ parameters...
February 6, 2018: Neurobiology of Aging
Charlotte Gary, Anne-Sophie Hérard, Zoé Hanss, Marc Dhenain
Accumulation of amyloid-β (Aβ) peptides in the brain is a critical early event in the pathogenesis of Alzheimer's disease (AD), the most common age-related neurodegenerative disorder. There is increasing interest in measuring levels of plasma Aβ since this could help in diagnosis of brain pathology. However, the value of plasma Aβ in such a diagnosis is still controversial and factors modulating its levels are still poorly understood. The mouse lemur ( Microcebus murinus ) is a primate model of cerebral aging which can also present with amyloid plaques and whose Aβ is highly homologous to humans'...
2018: Frontiers in Aging Neuroscience
Kumail Khandwala, Anwar Ahmed, Taha Sheikh
Mitochondrial encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a rare multisystem disorder and is the most common maternally inherited mitochondrial disease. This condition has a special predilection for the nervous system and muscles. Typical findings on brain imaging include stroke-like areas, calcification of basal ganglia and brain atrophy. This accounts for the disease being, both clinically and radiologically, mistaken for ischemic stroke. The differentiation features from stroke include comparatively young age of the patients, site of the lesions, and relative overlap between the cerebral vasculature territories...
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Bryce Tan, Narayanaswamy Venketasubramanian, Henri Vrooman, Ching-Yu Cheng, Tien Yin Wong, Mohammad Kamran Ikram, Christopher Chen, Saima Hilal
BACKGROUND: Plasma homocysteine levels are increasingly studied as a potential risk factor for dementia. Elevated homocysteine levels have been linked with gray and white matter volume reduction among individuals with mild cognitive impairment and Alzheimer's disease. However, the effects of homocysteine on brain changes in preclinical stages of dementia remain unexplored. OBJECTIVE: To examine the association of elevated homocysteine levels with markers of neurodegeneration, i...
2018: Journal of Alzheimer's Disease: JAD
Kazushi Ichikawa, Megumi Tsuji, Yu Tsuyusaki, Moyoko Tomiyasu, Noriko Aida, Tomohide Goto
Gamma-aminobutyric acid transaminase (GABA-T) deficiency is a rare, autosomal recessive disorder characterized by severe psychomotor retardation, early-onset epileptic encephalopathy, intractable seizures, hypotonia, and hyperreflexia. The disease is caused by mutation in the 4-aminobutyrate aminotransferase (ABAT) gene, which encodes an enzyme involved in GABA catabolism. In this chapter, a 10-year follow-up of GABA-T deficiency in a rare case of a long-term survivor patient is discussed. The patient showed a progression of clinical phases with increasing age...
February 25, 2018: JIMD Reports
Fanny Kortüm, Rami Abou Jamra, Malik Alawi, Susan A Berry, Guntram Borck, Katherine L Helbig, Sha Tang, Dagmar Huhle, Georg Christoph Korenke, Malavika Hebbar, Anju Shukla, Katta M Girisha, Maja Steinlin, Sandra Waldmeier-Wilhelm, Martino Montomoli, Renzo Guerrini, Johannes R Lemke, Kerstin Kutsche
Pontocerebellar hypoplasia (PCH) represents a group of autosomal-recessive progressive neurodegenerative disorders of prenatal onset. Eleven PCH subtypes are classified according to clinical, neuroimaging and genetic findings. Individuals with PCH type 9 (PCH9) have a unique combination of postnatal microcephaly, hypoplastic cerebellum and pons, and hypoplastic or absent corpus callosum. PCH9 is caused by biallelic variants in AMPD2 encoding adenosine monophosphate deaminase 2; however, a homozygous AMPD2 frameshift variant has recently been reported in two family members with spastic paraplegia type 63 (SPG63)...
February 20, 2018: European Journal of Human Genetics: EJHG
Xue Cheng, Haiping Zhao, Feng Yan, Zhen Tao, Rongliang Wang, Ziping Han, Guangwen Li, Yumin Luo, Xunming Ji
Maladaptive alterations of astrocytic plasticity may cause brain edema in the acute stage of stroke and glial scar formation in the recovery stage. The present study was designed to investigate the potential regulation of limb remote ischemic post-conditioning (RIPC) on astrocytic plasticity in experimental cerebral ischemia-reperfusion injury. Cerebral ischemia was induced by transient middle cerebral artery occlusion (tMCAO) for 1 h in C57BL/6 mice, who were treated with RIPC immediately after reperfusion...
February 17, 2018: Brain Research
Axel Lipp, Cornelia Skowronek, Andreas Fehlner, Kaspar-Josche Streitberger, Jürgen Braun, Ingolf Sack
OBJECTIVES: To apply three-dimensional multifrequency MR-elastography (3DMRE) for the measurement of local cerebral viscoelasticity changes in patients with Parkinson's disease (PD) and progressive supranuclear palsy (PSP). METHODS: T1-weighted anatomical imaging and 3DMRE were performed in 17 PD and 20 PSP patients as well as 12 controls. Two independent viscoelasticity parameters, |G*| and φ, were reconstructed combining seven harmonic vibration frequencies (30-60 Hz)...
February 19, 2018: European Radiology
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