Gerstmann-Sträussler-Scheinker syndrome with the P102L pathogenic mutation presenting as familial Creutzfeldt-Jakob disease: a case report and review of the literature.

abstract：:A 57-year-old man suffered severe amnesia and disorientation, accompanied by content-specific confabulation, due to an alcoholic Wernicke-Korsakoff syndrome. For months, he was deeply concerned about a single obligation that he thought he had to respond to, but which he had already assumed 20 years previously. This mo...

journal_title：Neurocase

authors： Schnider A,Nahum L,Pignat JM,Leemann B,Lövblad KO,Wissmeyer M,Ptak R

更新日期：2013-01-01 00:00:00

abstract：:A 68-year-old man had a cerebral infarction affecting the right parietal lobe, right insula, and the left prefrontal region. On admission, left hand tactile extinction was noted when the hands were placed both in anatomical and crossed positions, but was noted only in crossed positions from day 20 and later. Five mont...

journal_title：Neurocase

authors： Asada T,Takeda K,Okiyama R,Isozaki E,Fukuyama H

更新日期：2014-01-01 00:00:00

abstract：:A hexanucleotide repeat expansion in the chromosome 9 open reading frame 72 (C9ORF72) gene was recently discovered as the cause underlying frontotemporal degeneration (FTD) and/or amyotrophic lateral sclerosis (ALS) linked to chromosome 9 (c9FTD/ALS). In this atypical case of c9FTD/ALS, the proband presented with amne...

journal_title：Neurocase

authors： Adeli A,Savica R,Lowe VJ,Vemuri P,Knopman DS,Dejesus-Hernandez M,Rademakers R,Fields JA,Crum BA,Jack CR,Petersen RC,Boeve BF

更新日期：2014-01-01 00:00:00

abstract：:While language characteristics of logopenic variant primary progressive aphasia (lvPPA) are well-defined, behavioral characteristics are less understood. We investigated correlations between language and behavioral scores across three variants of primary progressive aphasia (PPA) and found language performance and beh...

journal_title：Neurocase

authors： Keator LM,Wright AE,Saxena S,Kim K,Demsky C,Sebastian R,Sheppard SM,Breining B,Hillis AE,Tippett DC

更新日期：2019-01-01 00:00:00

abstract：:A 54-year-old art teacher, experienced a right putaminal hemorrhage, and thereafter suffered severe left hemiplegia and unilateral spatial neglect, and was transferred to the rehabilitation department of the University Hospital 1 month after the onset. Although the unilateral spatial neglect was improving, the patient...

journal_title：Neurocase

authors： Kondo M,Mori T,Makino K,Okazaki T,Hachisuka K

更新日期：2012-06-01 00:00:00

abstract：:We characterized the effect of virtual visual feedback (VVF) on supernumerary phantom limb pain (SPLP) in a patient with high cervical cord injury. The subject was a 22-year-old man diagnosed with complete spinal cord injury (level C2) approximately 5 years ago. We applied the ABA'B' single-case design and set phases ...

journal_title：Neurocase

authors： Katayama O,Iki H,Sawa S,Osumi M,Morioka S

更新日期：2015-01-01 00:00:00

abstract：:One typical feature of the neglect syndrome in patients with right hemisphere damage is that they bisect horizontal lines to the right of centre. It has been argued that to a large extent these bisection errors can be attributed to a perceptual change whereby the patient experiences the left half of a line as shorter ...

journal_title：Neurocase

authors： Pritchard CL,Dijkerman HC,McIntosh RD,Milner AD

更新日期：2001-01-01 00:00:00

abstract：:In this paper, we adopt a neurodevelopmental stance to examining frontal variant frontotemporal dementia (fv-FTD) by using experimental procedures from the literature on the growth of social behaviour in children to examine the deficits in social reasoning which may underpin behavioural disturbance in fv-FTD. We prese...

journal_title：Neurocase

authors： Lough S,Gregory C,Hodges JR

更新日期：2001-01-01 00:00:00

abstract：:After a unilateral brain lesion, patients may show cross-modal, visual-tactile extinction. Such patients may fail to report tactile stimuli on the contralesional hand when presented together with competing visual stimuli near the ipsilesional hand. In this work we tested the hypothesis that this cross-modal extinction...

journal_title：Neurocase

authors： Maravita A,Clarke K,Husain M,Driver J

更新日期：2002-01-01 00:00:00

abstract：:This paper reports the case of a patient, M.P., who developed delusion of inanimate doubles, without Capgras syndrome, after traumatic brain injury. His delusional symptoms were studied longitudinally and the cognitive impairments associated with delusion were investigated. Data suggest that M.P. did 'perceive' the ac...

journal_title：Neurocase

authors： Abbate C,Trimarchi PD,Salvi GP,Quarenghi AM,Vergani C,Luzzatti C

更新日期：2012-01-01 00:00:00

abstract：:We observed a 59-year-old right-handed man with an infarction in his right-middle cerebral artery that included the parietal lobe, who abnormally manipulated mental images in the horizontal direction, resulting in calculation disturbances. Three years later, the patient suffered an infarction in the left parietal lobe...

journal_title：Neurocase

authors： Asada T,Takayama Y,Oita J,Fukuyama H

更新日期：2014-04-01 00:00:00

abstract：:The noradrenergic system modulates performance on tasks dependent on semantic and associative network flexibility (NF) in individuals without neurodevelopmental diagnoses in experiments using a beta-adrenergic antagonist, propranolol. Some studies suggest drugs decreasing noradrenergic activity are beneficial in ASD. ...

journal_title：Neurocase

authors： Beversdorf DQ,Carpenter AL,Miller RF,Cios JS,Hillier A

更新日期：2008-01-01 00:00:00

abstract：:Both prosody and sentence structure (e.g., canonical versus non-canonical) affect sentence comprehension. However, few previous studies have examined a possible interaction between prosody and sentence structure. In adult controls we found a significant interaction: typical sentence prosody, versus list prosody, facil...

journal_title：Neurocase

authors： LaCroix AN,Blumenstein N,Houlihan C,Rogalsky C

更新日期：2019-01-01 00:00:00

abstract：:Studies of patients with brain lesions have demonstrated that language and praxis are mediated by dissociable networks. However, language has the capacity to influence the selection of purposeful actions. The abilities to use language and to program purposeful movements are often mediated by networks that have anatomi...

journal_title：Neurocase

authors： Falchook AD,Burtis DB,Acosta LM,Salazar L,Shushrutha Hedna V,Khanna AY,Heilman KM

更新日期：2014-06-01 00:00:00

abstract：:In large early cortical lesions, one of the most devastating consequences is the impaired motor dexterity of the contralateral limb. We present the case of an 8-year-old girl with a large left hemispherical porencephaly. Unlike previous reports, the girl exhibited high dexterity of the contralateral hand with preserve...

journal_title：Neurocase

authors： Weber B,Fliessbach K,Elger CE

更新日期：2006-10-01 00:00:00

abstract：:The purpose of this study was to determine if differential reading and spelling mechanisms were involved in a Japanese patient with aphasia. In our case, the patient scored low on all of the administered reading tasks, suggesting that both the reading lexical and non-lexical routes were impaired. In contrast, his writ...

journal_title：Neurocase

authors： Hashimoto K,Uno A

更新日期：2016-06-01 00:00:00

abstract：:We investigated the nature of covertly processed visual elements in a patient with simultanagnosia, a disorder characterized by the inability to perceive multiple aspects of a visual scene all at once. Using the first letter of the color words red, green, or blue, we created a novel testing paradigm that combined Navo...

journal_title：Neurocase

authors： Shenker JI,Roberts MH

更新日期：2016-06-01 00:00:00

abstract：:Neglect dyslexia is a reading disorder often associated with right-sided brain lesions. In reading single words, errors are mostly substitutions or omissions of letters that occupy the left-sided positions. Typically, these errors have been thought to depend on a single mechanism. Conversely, we propose that they are ...

journal_title：Neurocase

authors： Martelli M,Arduino LS,Daini R

更新日期：2011-01-01 00:00:00

abstract：:Metamemory is a multifaceted concept, which deals with an individual's knowledge and control of his or her own memory system. The ability to monitor memory performance accurately was examined in 16 patients with Alzheimer's disease (AD), 6 patients with frontotemporal lobe dementia (FTD) and 16 elderly subjects. Parti...

journal_title：Neurocase

authors： Souchay C,Isingrini M,Pillon B,Gil R

更新日期：2003-12-01 00:00:00

abstract：:Patients with memory disorders have severely restricted learning and memory. For instance, patients with anterograde amnesia can learn motor procedures and retain some restricted ability to learn new words and factual information. However, such learning is inflexible and frequently inaccessible to conscious awareness....

journal_title：Neurocase

authors： Olson IR,Berryhill ME,Drowos DB,Brown L,Chatterjee A

更新日期：2010-06-01 00:00:00

abstract：:BVR was 77 years old when he sustained a large posterior cerebral artery territory infarct. Medical, cognitive and functional data collected on four occasions over 10 years initially revealed circumscribed neurological signs, no functional or cognitive deficits. BVR became significantly impaired only after two other s...

journal_title：Neurocase

authors： Piguet O,Bennett HP,Waite LM,Kril JJ,Creasey H,Anthony Broe G,Halliday GM

更新日期：2006-04-01 00:00:00

abstract：:Individuals with posterior cortical atrophy (PCA) report a host of unusual and poorly explained visual disturbances. This preliminary report describes a single patient (CRO), and documents and investigates abnormally prolonged colour afterimages (concurrent and prolonged perception of colours complimentary to the colo...

journal_title：Neurocase

authors： Crutch SJ,Lehmann M,Gorgoraptis N,Kaski D,Ryan N,Husain M,Warrington EK

更新日期：2011-01-01 00:00:00

abstract：:The semantic variant of primary progressive aphasia (PPA-S) is characterized by impairments in confrontation naming and single word comprehension. Although episodic memory may be relatively spared, there can be impairment in verbal learning tasks. We report a patient with PPA-S and impaired verbal learning who was tes...

journal_title：Neurocase

authors： Milano NJ,Williamson JB,Heilman KM

更新日期：2015-01-01 00:00:00

abstract：:We have seen a patient with a profound, isolated, and quite selective deficit in proverb interpretation-aproverbia. The patient presented to us after an anoxic brain injury with aproverbia. Interestingly, the aproverbia appeared to be premorbid to the presenting event. Furthermore, the patient had no brain lesion that...

journal_title：Neurocase

authors： Brown C,Smith-Benjamin S,Patira R,Altschuler EL

更新日期：2016-06-01 00:00:00

abstract：:A 57-year-old male with no family history was diagnosed with semantic dementia. He also showed some unusual cognitive features such as episodic memory and executive dysfunctions, spatial disorientation, and dyscalculia. Rapidly progressive cognitive and physical decline occurred. About 1.5 years later, he developed cl...

journal_title：Neurocase

authors： Clerc MT,Deprez M,Leuba G,Lhermitte B,Lopez U,von Gunten A

更新日期：2015-02-01 00:00:00

abstract：:The impact of epileptiform activity on the functional organization of various cortical regions is currently a widely discussed topic. Our patient, suffering from left-sided temporal lobe epilepsy with typical mesiotemporal sclerosis, had a remarkable history of a clear-cut handedness shift in early childhood. In this ...

journal_title：Neurocase

authors： Chlebus P,Brázdil M,Hlustík P,Mikl M,Pazourková M,Krupa P

更新日期：2004-08-01 00:00:00

abstract：UNLABELLED:Neuropathologic change underlying primary progressive aphasia (PPA) most commonly includes one of the frontotemporal lobar degenerations, such as FTLD-tau or FTLD-ubiquitin. The next most frequent etiology of PPA is Alzheimer's disease (AD). We describe 5 subjects with clinical diagnoses of semantic dementia...

journal_title：Neurocase

authors： Chow TW,Varpetian A,Moss T,Vinters HV,Marquez S,Miller C

更新日期：2010-02-01 00:00:00

abstract：:Frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) is one of the most common pathological findings associated with the clinical FTLD syndromes. However, molecular characterization with genetic sequencing and protein expression techniques are recognizing many new subtypes for FTLDs. FTLDs are...

journal_title：Neurocase

authors： Farmer JG,Crain BJ,Harris BT,Turner RS

更新日期：2013-01-01 00:00:00

abstract：:With injury of the anterior two-thirds of the corpus callosum, each hemisphere's attentional bias to contralateral hemispace becomes manifest with each hand deviating ipsilaterally during line bisection tasks. Patients with infarctions in the right posterior cerebral artery distribution with occipital and splenial dam...

journal_title：Neurocase

authors： Kesayan T,Gasoyan H,Heilman KM

更新日期：2018-08-01 00:00:00

abstract：:The psychoses of epilepsy are well recognized complications of seizure disorders, diagnosed easily from the history. However, in the absence of recognized seizures, the diagnosis can be challenging. We present a 27-year-old female, who suffered a treatment refractory psychosis for 6 years. She did not report, or dis...

journal_title：Neurocase

authors： Needham E,Hamelijnck J

更新日期：2012-01-01 00:00:00