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Gait abnormality and white matter hyperintensity

Christina Evers, Angelika Seitz, Birgit Assmann, Thomas Opladen, Stephanie Karch, Katrin Hinderhofer, Martin Granzow, Nagarajan Paramasivam, Roland Eils, Nicolle Diessl, Claus R Bartram, Ute Moog
Neurodegeneration with brain iron accumulation (NBIA) is a group of neurodegenerative disorders characterized by iron accumulation in the basal ganglia. Recently, mutations in CoA synthase (COASY) have been identified as a cause of a novel NBIA subtype (COASY Protein-Associated Neurodegeneration, CoPAN) in two patients with dystonic paraparesis, parkinsonian features, cognitive impairment, behavior abnormalities, and axonal neuropathy. COASY encodes an enzyme required for Coenzyme A (CoA) biosynthesis. Using whole exome sequencing (WES) we identified compound heterozygous COASY mutations in two siblings with intellectual disability, ataxic gait, progressive spasticity, and obsessive-compulsive behavior...
May 10, 2017: American Journal of Medical Genetics. Part A
Zeynep Tufekcioglu, Arman Cakar, Basar Bilgic, Hasmet Hanagasi, Hakan Gurvit, Murat Emre
Phenylketonuria (PKU) is an autosomal recessive metabolic disorder due to mutations in the phenylalanine hydroxylase (PAH) gene, which converts phenylalanine (PHE) to tyrosine. Although it is principally a childhood disorder, in rare cases, the first signs of PKU may develop in late adulthood resembling common neurological diseases. Here we report a 59-year-old, previously normal functioning man who was admitted with blurred vision, cognitive problems, and gait difficulty that began 8 months before. He had brisk reflexes and left side dominant parkinsonism...
June 2016: Neurocase
Dae Hyun Kim, Francine Grodstein, Anne B Newman, Paulo H M Chaves, Michelle C Odden, Ronald Klein, Mark J Sarnak, Lewis A Lipsitz
OBJECTIVES: To evaluate and compare the associations between microvascular and macrovascular abnormalities and cognitive and physical function DESIGN: Cross-sectional analysis of the Cardiovascular Health Study (1998-1999). SETTING: Community. PARTICIPANTS: Individuals with available data on three or more of five microvascular abnormalities (brain, retina, kidney) and three or more of six macrovascular abnormalities (brain, carotid artery, heart, peripheral artery) (N = 2,452; mean age 79...
September 2015: Journal of the American Geriatrics Society
Kevin Berendse, Marc Engelen, Sacha Ferdinandusse, Charles B L M Majoie, Hans R Waterham, Frédéric M Vaz, Johannes H T M Koelman, Peter G Barth, Ronald J A Wanders, Bwee Tien Poll-The
INTRODUCTION: We describe the natural history of patients with a Zellweger spectrum disorder (ZSD) surviving into adulthood. METHODS: Retrospective cohort study in patients with a genetically confirmed ZSD. RESULTS: All patients (n = 19; aged 16-35 years) had a follow-up period of 1-24.4 years (mean 16 years). Seven patients had a progressive disease course, while 12 remained clinically stable during follow-up. Disease progression usually manifests in adolescence as a gait disorder, caused by central and/or peripheral nervous system involvement...
January 2016: Journal of Inherited Metabolic Disease
Joaquin A Vizcarra, Anthony E Lang, Kapil D Sethi, Alberto J Espay
Progressive ambulatory impairment and abnormal white matter (WM) signal on neuroimaging come together under the diagnostic umbrella of vascular parkinsonism (VaP). A critical appraisal of the literature, however, suggests that (1) no abnormal structural imaging pattern is specific to VaP; (2) there is poor correlation between brain MRI hyperintensities and microangiopathic brain disease and parkinsonism from available clinicopathologic data; (3) pure parkinsonism from vascular injury ("definite" vascular parkinsonism) consistently results from ischemic or hemorrhagic strokes involving the SN and/or nigrostriatal pathway, but sparing the striatum itself, the cortex, and the intervening WM; and (4) many cases reported as VaP may represent pseudovascular parkinsonism (e...
June 2015: Movement Disorders: Official Journal of the Movement Disorder Society
Nobuo Yamashiro, Takamura Nagasaka, Ryusuke Takaki, Michiaki Miwa, Kazumasa Shindo, Yoshihisa Takiyama
A 54-year-old man was admitted due to progressive gait disturbance and cognitive impairment. On MRI, a hyperintense region was observed in the periventricular white matter on FLAIR imaging, with Gd-enhancement in the choroid plexus and periventricular wall. Cerebrospinal fluid (CSF) examination showed marked abnormalities including a high white blood cell count (WBC, 360 cells/mm(3). 83% lymphocytes), an elevated protein level (1,416 mg/dl), a low glucose level (12 mg/dl), and elevated cryptococcal antigen with positive Indian ink staining...
2015: Rinshō Shinkeigaku, Clinical Neurology
Rukmini Mridula Kandadai, Praveen Yada, Megha S Uppin, Shaik Afshan Jabeen, Ajith Cherian, Meena Angamuthu Kanikannan, Rupam Borgohain, Sundaram Challa
BACKGROUND: Subacute sclerosing panencephalitis (SSPE) is a delayed and fatal manifestation of measles infection. Fulminant SSPE is a rare presentation in which the disease progresses to death over a period of 6 months. The clinical features are atypical and can be misleading. CASE REPORT: We report herein a teenage boy who presented with acute-onset gait ataxia followed by right hemiparesis that evolved over 1 month, with left-hemispheric, delta-range slowing on the electroencephalogram (EEG)...
October 2014: Journal of Clinical Neurology
Fernando Gonzalez-Ibarra, Waheed Abdul, Sahar Eivaz-Mohammadi, Christopher Foscue, Srinivas Gongireddy, Amer Syed
A 50-year-old AIDS patient with a CD4 T-cell count of 114/mm(3) was admitted with cerebellar symptoms of left CN XI weakness, wide-based gait with left-sided dysmetria, abnormal heel-knee-shin test, and dysdiadochokinesia. MRI showed region of hyperintensity in the left inferior cerebellar hemisphere involving the cortex and underlying white matter. Serological tests for HSV1, HSV2, and syphilis were negative. Her CSF contained high protein content and a WBC of 71/mm(3), predominantly lymphocytes. The CSF was also negative for cryptococcal antigen and VDRL...
2014: Case Reports in Neurological Medicine
Hiroki Tani, Hideto Nakajima, Kazushi Yamane, Hiroyuki Ohnishi, Fumiharu Kimura, Toshiaki Hanafusa
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare central nervous system inflammatory disease characterized by the punctate gadolinium enhancement peppering the pons and the cerebellar peduncles as neuroimaging. We report the case of a 66-year-old woman who presented with CLIPPERS associated with swelling in the brainstem. She was hospitalized because of gait ataxia and consciousness disturbance. MRI of the brain showed FLAIR hyperintense lesions in the pons, cerebellar peduncles, cerebellum and the subcortical white matter lesion in the right occipital lobe with significant swelling in the brainstem...
2014: Rinshō Shinkeigaku, Clinical Neurology
Andrea L Rosso, Megan J Olson Hunt, Mei Yang, Jennifer S Brach, Tamara B Harris, Anne B Newman, Suzanne Satterfield, Stephanie A Studenski, Kristine Yaffe, Howard J Aizenstein, Caterina Rosano
Step length variability (SLV) increases with age in those without overt neurologic disease, is higher in neurologic patients, is associated with falls, and predicts dementia. Whether higher SLV in older adults without neurologic disease indicates presence of neurologic abnormalities is unknown. Our objective was to identify whether SLV in older adults without overt disease is associated with findings from multimodal neuroimaging. A well-characterized cohort of 265 adults (79-90 years) was concurrently assessed by gait mat, magnetic resonance imaging with diffusion tensor, and neurological exam...
2014: Gait & Posture
Matthew D Macfarlane, Jeffrey C L Looi, Mark Walterfang, Gabriela Spulber, Dennis Velakoulis, Martin Styner, Milita Crisby, Eva Orndahl, Timo Erkinjuntti, Gunhild Waldemar, Ellen Garde, Michael G Hennerici, Hansjörg Bäzner, Christian Blahak, Anders Wallin, Lars-Olof Wahlund
OBJECTIVE: Functional deficits seen in several neurodegenerative disorders have been linked with dysfunction in frontostriatal circuits and with associated shape alterations in striatal structures. The severity of visible white matter hyperintensities (WMHs) on magnetic resonance imaging has been found to correlate with poorer performance on measures of gait and balance. This study aimed to determine whether striatal volume and shape changes were correlated with gait dysfunction. METHODS: Magnetic resonance imaging scans and clinical gait/balance data (scores from the Short Physical Performance Battery [SPPB]) were sourced from 66 subjects in the previously published LADIS trial, performed in nondisabled individuals older than age 65 years with WMHs at study entry...
January 2015: American Journal of Geriatric Psychiatry
Candace K Mathiason, Amy V Nalls, Davis M Seelig, Susan L Kraft, Kevin Carnes, Kelly R Anderson, Jeanette Hayes-Klug, Edward A Hoover
Domestic and nondomestic cats have been shown to be susceptible to feline spongiform encephalopathy (FSE), almost certainly caused by consumption of bovine spongiform encephalopathy (BSE)-contaminated meat. Because domestic and free-ranging nondomestic felids scavenge cervid carcasses, including those in areas affected by chronic wasting disease (CWD), we evaluated the susceptibility of the domestic cat (Felis catus) to CWD infection experimentally. Cohorts of 5 cats each were inoculated intracerebrally (i...
February 2013: Journal of Virology
C Annweiler, O Beauchet, R Bartha, M Montero-Odasso
Slow gait is ubiquitous among older adults and predicts cognitive decline and progression to dementia. Age-related structural brain changes could be responsible for abnormal gait. The purpose of this study was to determine whether brain lateral ventricle volume, a measure of brain atrophy, was associated with gait velocity among older adults with mild cognitive impairment (MCI), while considering the effects of age and brain vascular burden. Twenty community-dwellers with MCI, free of hydrocephalus, aged 76 years (69/80) [median (25th/75th percentile)] (35 % female) from the 'Gait and Brain Study' were included in this analysis...
July 2013: Journal of Neural Transmission
Floriane Courtois, Michel Gille, Frédéric Haven, Philippe Hantson
A 29-year-old immunocompetent woman was admitted in 2006 with ataxia, limb weakness, generalized dystonia, and vertical diplopia that developed after a febrile episode. Brain magnetic resonance imaging (MRI) revealed the presence of extensive periventricular white matter lesions that did not enhance after gadolinium injection. As low titers of cytomegalovirus-IgM antibodies were found in the serum, a presumed diagnosis of postviral acute disseminated encephalomyelitis (ADEM) was made, and the patient received a 5-day course of 1 g methylprednisolone...
September 2012: Case Reports in Neurology
Joshua Z Willey, Nikolaos Scarmeas, Frank A Provenzano, José A Luchsinger, Richard Mayeux, Adam M Brickman
Gait speed is associated with multiple adverse outcomes of aging. White matter hyperintensities (WMH) on magnetic resonance imaging (MRI) have been associated with gait speed, though few studies have examined changes in gait speed over time in population-based studies comprising participants from diverse cultural backgrounds. The purpose of this study was to examine the association between a decline in gait speed and total and regional WMH volumes in a community-based study of aging. Participants (n = 701) underwent gait-speed measurement via a 4-m walk test at the time of initial enrollment and MRI at a second time interval (mean 4...
March 2013: Journal of Neurology
Fanny Mochel, Raphael Schiffmann, Marjan E Steenweg, Hasan O Akman, Mary Wallace, Frédéric Sedel, Pascal Laforêt, Richard Levy, J Michael Powers, Sophie Demeret, Thierry Maisonobe, Roseline Froissart, Bruno Barcelos Da Nobrega, Brent L Fogel, Marvin R Natowicz, Catherine Lubetzki, Alexandra Durr, Alexis Brice, Hanna Rosenmann, Varda Barash, Or Kakhlon, J Moshe Gomori, Marjo S van der Knaap, Alexander Lossos
OBJECTIVE: Adult polyglucosan body disease (APBD) is an autosomal recessive leukodystrophy characterized by neurogenic bladder, progressive spastic gait, and peripheral neuropathy. Polyglucosan bodies accumulate in the central and peripheral nervous systems and are often associated with glycogen branching enzyme (GBE) deficiency. To improve clinical diagnosis and enable future evaluation of therapeutic strategies, we conducted a multinational study of the natural history and imaging features of APBD...
September 2012: Annals of Neurology
Yufuko Saito, Yasushi Iwasaki, Ikuko Aiba, Tetsuyuki Kitamoto, Mari Yoshida, Yoshio Hashizume
A 59-year-old Japanese man presented with depressed mood, insomnia, abnormal behavior and dementia. Visual and gait disturbance with ataxia also developed. Diffusion-weighted MRI showed widespread regions of hyperintensity in the bilateral cerebral cortex. The patient died at 62 after a progressive clinical course of 32 months. Myoclonus, periodic sharp-wave complexes on EEG, and akinetic mutism state were not observed. Neuropathologic examination showed widespread cerebral neocortical involvement with both large confluent vacuole-type, alongside fine vacuole-type spongiform changes...
October 2011: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Refeeque A Bhadelia, Lori Lyn Price, Kurtis L Tedesco, Tammy Scott, Wei Qiao Qiu, Samuel Patz, Marshal Folstein, Irwin Rosenberg, Louis R Caplan, Peter Bergethon
BACKGROUND AND PURPOSE: Gait impairment is common in the elderly, especially those with stroke and white matter hyperintensities on conventional brain MRI. Diffusion tensor imaging (DTI) is more sensitive to white matter damage than conventional MRI. The relationship between DTI measures and gait has not been previously evaluated. Our purpose was to investigate the relationship between the integrity of white matter in the corpus callosum as determined by DTI and quantitative measures of gait in the elderly...
December 2009: Stroke; a Journal of Cerebral Circulation
Kazumi Iseki, Takashi Hanakawa, Kazuo Hashikawa, Hidekazu Tomimoto, Manabu Nankaku, Hiroshi Yamauchi, Mark Hallett, Hidenao Fukuyama
To clarify the mechanisms underlying gait disturbance secondary to age-related white matter changes (ARWMC), cerebral perfusion was investigated during treadmill walking. Twenty subjects with extensive hyperintensities in the periventricular and deep white matter on T(2)-weighted magnetic resonance images (MRI) were recruited. The ARWMC subjects were classified into gait-disturbed (GD) and non-GD groups according to clinical criteria. All the subjects underwent gait analyses and cerebral perfusion measurements during both gait and rest by using single photon emission computed tomography...
January 15, 2010: NeuroImage
Daisuke Inoue, Takeshi Hamamura, Taira Uehara, Katsue Miyoshi, Takefumi Katsuragi, Iwao Takeshita, Tetsuo Hamada
A 60-year-old female with intravascular lymphoma (IVL) presented with the complaint of urinary dysfunction and gait disturbance. T2 weighted MR imaging of the thoracic spinal cord showed a hyperintense lesion, and brain MR imaging indicated hyperintense lesions in the deep white matter. Multiple sclerosis was assumed, so steroid pulse therapy was administered. However, her level of consciousness decreased and her paraplegia progressed. Laboratory data showed that anemia and thrombocytopenia had worsened with high serum LDH and soluble IL-2 receptor levels...
April 2009: No Shinkei Geka. Neurological Surgery
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