Left hemispatial neglect with a splenial lesion.
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 damage can also exhibit spatial neglect. The goal of this report is to learn the role of the splenium of the corpus callosum in mediating visuospatial attention. A right-handed woman with Marchiafava-Bignami disease and damage to the splenium of her corpus callosum without evidence of a mesial f...
Source: Neurocase - October 28, 2018 Category: Neurology Authors: Kesayan T, Gasoyan H, Heilman KM Tags: Neurocase Source Type: research

A neuropathologic diagnosis of Alzheimer's disease in an older adult with HIV-associated neurocognitive disorder.
Abstract We discuss the challenges associated with diagnosing neurodegenerative disorders in older adults living with HIV, illustrated through a case report where neurologic co-diagnosis of Alzheimer's disease (AD) and HIV-associated Neurocognitive Disorder (HAND) are considered. The patient was followed and evaluated for over 4 years and underwent post-mortem neuropathologic evaluation. Further work is needed to identify diagnostic tests that can adequately distinguish HAND from early stage neurodegenerative disorders among older adults living with HIV and cognitive changes. PMID: 30304986 [PubMed - as suppl...
Source: Neurocase - October 10, 2018 Category: Neurology Authors: Hellmuth J, Milanini B, Masliah E, Tartaglia MC, Dunlop MB, Moore DJ, Javandel S, DeVaughn S, Valcour V Tags: Neurocase Source Type: research

Anti-NMDA receptor encephalitis presenting as isolated aphasia in an adult.
We report a case of an adult patient with anti-NMDA-r encephalitis presenting with isolated, abrupt-onset aphasia. Her condition remained unaltered over a period of 6 months. The patients' electroencephalogram findings were typical for NMDA-r encephalitis; however, her magnetic resonance imaging and cerebrospinal fluid analysis were normal. She responded well to immunotherapy, and aphasia eventually resolved. The natural course of the present case contradicts the rapidly progressive nature of typical NMDA-r encephalitis. Furthermore, it broadens the clinical spectrum of anti-NMDA-r encephalitis, to incorporate isolate...
Source: Neurocase - October 6, 2018 Category: Neurology Authors: Constantinides VC, Kasselimis DS, Paraskevas GP, Zacharopoulou M, Andreadou E, Evangelopoulos ME, Kapaki E, Kilidireas C, Stamboulis E, Potagas C Tags: Neurocase Source Type: research

Verbal adynamia in parkinsonian syndromes: behavioral correlates and neuroanatomical substrate.
Abstract Verbal adynamia (impaired language generation, as during conversation) has not been assessed systematically in parkinsonian disorders. We addressed this in patients with Parkinson's dementia, progressive supranuclear palsy and corticobasal degeneration. All disease groups showed impaired verbal fluency and sentence generation versus healthy age-matched controls, after adjusting for general linguistic and executive factors. Dopaminergic stimulation in the Parkinson's group selectively improved verbal generation versus other cognitive functions. Voxel-based morphometry identified left inferior frontal and p...
Source: Neurocase - October 6, 2018 Category: Neurology Authors: Magdalinou NK, Golden HL, Nicholas JM, Witoonpanich P, Mummery CJ, Morris HR, Djamshidian A, Warner TT, Warrington EK, Lees AJ, Warren JD Tags: Neurocase Source Type: research

Tracking of neurocognitive outcomes over time in children with perinatal stroke and associated complex medical conditions: a case series.
Abstract Neurocognitive outcomes in children with perinatal stroke are complicated by additional neurological/medical factors. The main objective of this study was to retrospectively examine long-term neurocognitive outcomes in five children with perinatal stroke with increasingly complex comorbidities; ages 5-7 at first evaluation. Results revealed that intelligence and academic skills were significantly decreased in cases with combined perinatal stroke and comorbidities. Early language development was particularly vulnerable to disruption over time in children with increasing medical complexity. The delayed emer...
Source: Neurocase - September 26, 2018 Category: Neurology Authors: Murdaugh D, Morris S, O'Toole K Tags: Neurocase Source Type: research

Kl üver & Bucy syndrome: an investigation of social and affective cognition.
We report the case of ASP, a patient who developed a complete KBS at 9 years that evolved into an incomplete KBS. Orbitofrontal and temporal damages were evidenced. While a classic neuropsychological assessment showed a preserved global functioning, an extensive evaluation of her social and affective cognition (reversal learning, decision-making, emotion recognition, theory of mind, creative thinking) showed remarkable deficits. The relevancy of such findings for the characterization KBS and the field of neuropsychology are discussed. PMID: 30247092 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - September 24, 2018 Category: Neurology Authors: Bertoux M, de Paula França Resende E, de Souza LC Tags: Neurocase Source Type: research

A case of Capgras syndrome and folie à duex in monozygotic twins.
We present a case of substance-induced Capgras syndrome emerging as folie à deux (insanity of two) in monozygotic twins with strongly overlapping life histories. Then, we discuss the etiology and the nosological position of these two conditions as well as their significance for understanding the concept of psychosis. PMID: 30139309 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - August 24, 2018 Category: Neurology Authors: Gbyl K, Balslev Jørgensen M Tags: Neurocase Source Type: research

Pathological correlates of white matter hyperintensities in a case of progranulin mutation associated frontotemporal dementia.
We examined the histological changes underlying WMH in a patient with GRN mutation associated behavioral variant FTD. In vivo and cadaveric MRI showed progressive, asymmetric frontotemporal and parietal atrophy, and asymmetrical WMH predominantly affecting frontal mid-zones. We first performed segmentation and localization analyses of WMH present on cadaveric MRI FLAIR images, then selected five different brain regions directly matched to differing severities of WMH for histological analysis. We used immunohistochemistry to assess vascular pathology, degree of spongiosis, neuronal and axonal loss, TDP-43, demyelination and...
Source: Neurocase - August 16, 2018 Category: Neurology Authors: Woollacott IOC, Bocchetta M, Sudre CH, Ridha BH, Strand C, Courtney R, Ourselin S, Cardoso MJ, Warren JD, Rossor MN, Revesz T, Fox NC, Holton JL, Lashley T, Rohrer JD Tags: Neurocase Source Type: research

Mitochondrial membrane protein-associated neurodegeneration: a case report and literature review.
We report a compound heterozygous c.[32C>T];[205G>A;424A>G] (p.[Thr11Met];[Gly69Arg;Lys142Glu]) Czech patient who manifested with right foot dystonia, impaired handwriting, attention deficit, and signs of iron accumulation on brain MRI. Gradually, he developed dysarthria, spastic-dystonic gait, pedes cavi, and atrophy of leg muscles. Additionally, we report demographic parameters, clinical signs, and allelic frequencies of C19orf12 mutations of all published MPAN cases. We compared the most frequent mutations, p.Thr11Met and p.Gly69ArgfsX10; the latter was associated with younger age at onset and more frequent opt...
Source: Neurocase - August 8, 2018 Category: Neurology Authors: Dušek P, Školoudík D, Roth J, Dušek P Tags: Neurocase Source Type: research

Illustrating where spatial perception versus memory-based representation: spatial neglect in a distinguished artist; a case report.
We present a case, in an artist who demonstrates selective impairment in a contralesional space during active Where spatial perception. His performance is better when asked to draw entirely from memory. This case highlights the variability in neglect, importance for comprehensive testing, and encourages further investigation. PMID: 30035662 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - July 23, 2018 Category: Neurology Authors: Hreha K, Chaudhari A, Kong Y, Maduri P, Barrett AM Tags: Neurocase Source Type: research

Bilateral upper limb rehabilitation with videogame-based feedback in corticobasal degeneration: a case reports study.
We reported two cases treated with a bilateral upper limb rehabilitation tool with videogame based feedback for 3 time per week for 8 weeks. Both patients showed an improvement of pinch and grasp forces and motor function. However, both of them reported an increased upper limb pain. Bilateral upper limb mechanical device with exergame feedback was effective also in the two patients suffering of CBD for limiting the effects of apraxia by performing intensive purposeful task training. PMID: 30015554 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - July 17, 2018 Category: Neurology Authors: Fusco FR, Iosa M, Fusco A, Paolucci S, Morone G Tags: Neurocase Source Type: research

Early stage memory impairment, visual hallucinations, and myoclonus combined with temporal lobe atrophy predict Alzheimer's disease pathology in corticobasal syndrome.
We report a 73-year-old Korean man who was clinically diagnosed with CBS whose underlying pathology was Alzheimer's disease (AD) at autopsy (CBS-AD). This case suggests that early developing memory impairment and myoclonus, severe temporoparietal atrophy, and visual hallucinations may support a more specific prediction of CBS-AD. PMID: 29987978 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - July 10, 2018 Category: Neurology Authors: Jung NY, Lee JH, Lee YM, Shin JH, Shin MJ, Lee MJ, Pak K, Hwang C, Ahn JW, Sung S, Choi KU, Huh GY, Kim EJ Tags: Neurocase Source Type: research

A new MAPT deletion in a case of speech apraxia leading to corticobasal syndrome.
We describe the clinical profile evolution of a patient presenting with slowly progressive isolated speech apraxia that eventually led to the diagnosis of corticobasal syndrome (CBS), supporting the evidence that this rare speech disorder can be the first presentation of CBS. Moreover, we found a novel variant in MAPT gene, which is hypothesized to be disease-causing mutation. These results underscore the importance of genetic analysis - particularly in selected atypical cases - for in vivo understanding of possible pathophysiological disease process. PMID: 29969053 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - July 3, 2018 Category: Neurology Authors: Mazzon G, Menichelli A, Fabretto A, Cattaruzza T, Manganotti P Tags: Neurocase Source Type: research

A progressive breakdown of the body in space.
Abstract A 74 year-old woman (MD), free of previous neurological history, presented with difficulty in handling cutlery, clothes, writing with what was initially described as an atypical apraxia in acts related to space. Initial neurological evaluation revealed mixed, asymmetric pyramidal, and extrapyramidal semiology. Νeuropsychological testing revealed dressing and constructional deficits, ideomotor apraxia and signs of executive dysfunction in absence of memory, language, and visual perception pathology. The final diagnosis was that of a corticobasal degeneration, where the rare occurrence of a pro...
Source: Neurocase - June 8, 2018 Category: Neurology Authors: Kourtidou E, Kasselimis D, Makrydakis G, Chatziantoniou L, Kyrozis A, Evdokimidis I, Potagas C Tags: Neurocase Source Type: research

Clinical and imaging progression over 10 years in a patient with primary progressive apraxia of speech and autopsy-confirmed corticobasal degeneration.
We report clinical and neuroimaging data spanning 10 years from disease onset-to-death in a 49 year-old male PPAOS patient, DY, who died with corticobasal degeneration. He presented with AOS with normal neuroimaging. Abnormalities in the caudate nucleus, supplementary motor area, cingulate, insula, and Broca's area were observed after five years, with involvement of motor cortex and development of agrammatism, Parkinsonism, and dysarthria three years later. Cognitive impairment and temporoparietal atrophy were late features. This data provides important insight into disease progression of corticobasal degeneration whe...
Source: Neurocase - May 25, 2018 Category: Neurology Authors: Tetzloff KA, Duffy JR, Strand EA, Machulda MM, Boland SM, Utianski RL, Botha H, Senjem ML, Schwarz CG, Josephs KA, Whitwell JL Tags: Neurocase Source Type: research

Connecting the dots: an association between opioids and acute hippocampal injury.
Abstract Acute hippocampal injury represents a relatively rare cause of amnesia. Interestingly however, between 2012 and 2017, 18 patients were reported at hospitals in Massachusetts with sudden-onset amnesia in the setting of complete diffusion-weighted hyperintensity of both hippocampi on magnetic resonance imaging. Notably, 17 of the 18 patients tested positive for opioids or had a recorded history of opioid use. This observation suggests an association between opioids and acute hippocampal injury. With particular attention to the Massachusetts cluster and data on fentanyl and its congeners, the epidemiological...
Source: Neurocase - May 18, 2018 Category: Neurology Authors: Barash JA, Kofke WA Tags: Neurocase Source Type: research

Relief from intractable phantom pain by combining psilocybin and mirror visual-feedback (MVF).
Abstract AL's leg was amputated resulting in phantom-limb pain (PLP). (1) When a volunteer placed her foot on or near the phantom - touching it evoked organized sensations in corresponding locations on AL's phantom. (2) Mirror-visual-feedback (MVF) relieved PLP, as did, "phantom massage". (3) Psilocybin-MVF pairing produced synergistic effects, complete elimination of PLP, and reduction in paroxysmal episodes. (4) Touching the volunteer's leg where AL previously had external fixators, evoked sensation of nails boring through the leg. Using a "telescoping" nail, we created the illusion of a...
Source: Neurocase - May 15, 2018 Category: Neurology Authors: Ramachandran V, Chunharas C, Marcus Z, Furnish T, Lin A Tags: Neurocase Source Type: research

Interpersonal gargalesthesia.
Abstract When someone touches, say, your upper arm, mirror neurons in your brain's area S2 fire. These neurons also fire when you merely watch another person being touched. However, you do not literally feel the touch from his arm on your own skin. Consistent with this view, we find when someone's arm is removed, he does start experiencing another's sensations [Ramachandran, V. S., & Brang, D. (2009)]. A congenital variant of this syndrome also exists, as in our subject TC. TC experienced referred touch, referred tickle in her axilla, and was able to tickle herself. PMID: 29693492 [PubMed -...
Source: Neurocase - April 25, 2018 Category: Neurology Authors: Ramachandran VS, Sellers CA Tags: Neurocase Source Type: research

Persistent anterograde amnesia due to the artery of Percheron occlusion: a case report.
Abstract Bilateral thalamic infarction involving the artery of Percheron (AOP) can cause diagnostic difficulties due to the varying clinical presentations. AOP infarcts presented with isolated memory impairment are not common and the factors affecting the persistence of memory disorders are still unknown. A 41-year-old male patient was hospitalized with acute unconsciousness. MRI disclosed bilateral paramedian thalamic infarction The patient had isolated memory deficit and his anterograde amnesia continued without any change in the past decade. More cases might answer the questions concerning the intra- and extra-...
Source: Neurocase - April 2, 2018 Category: Neurology Authors: Ince B, Asan F Tags: Neurocase Source Type: research

Molecular neuroimaging in primary progressive aphasia with predominant agraphia.
Abstract A 62-year-old male presented with progressive isolated writing and spelling difficulties. Neurological, neuropsychological, speech, and language evaluations identified only minimal additional abnormalities. The presenting characteristics did not meet criteria for any particular variant of primary progressive aphasia; his clinical presentation is best described as primary progressive aphasia, with a predominant, almost pure agraphia. Brain MRI showed asymmetric, bilateral parenchymal volume loss, with left hippocampal atrophy. Fluorodeoxyglucose-F18 positron emission tomography showed hypometabolism in the...
Source: Neurocase - March 23, 2018 Category: Neurology Authors: Utianski RL, Duffy JR, Savica R, Whitwell JL, Machulda MM, Josephs KA Tags: Neurocase Source Type: research

Pattern of cerebral hypoperfusion according to the clinical staging in dementia with Lewy bodies.
This study aimed to detect different patterns of cerebral hypoperfusion in DLB according to clinical staging. Thirty-three patients with DLB were recruited by clinical dementia rating (CDR) stage. Compared with control, cerebral hypoperfusion was mainly observed in the lingual gyrus, the cuneus, the occipital gyrus in CDR 0.5 group; the fusiform gyrus, the middle temporal gyrus, and the posterior cingulate in CDR 1; and the lingual gyrus, the cuneus, the hippocampus, the fusiform gyrus, and the inferior frontal gyrus in CDR 2. Our findings suggest that cerebral hypoperfusion spreads to the frontal cortex and temporal lobes...
Source: Neurocase - March 6, 2018 Category: Neurology Authors: Park KW, Hong YJ, Park JH, Park H, Cheon SM, Kim JW, Kim BC, Jeong YJ, Yoon HJ, Kang DY Tags: Neurocase Source Type: research

A case of semantic variant primary progressive aphasia with Pick's pathology.
Abstract Neurodegenerative diseases are caused by aggregation of specific proteins that catalyze a cascade of changes that ultimately lead to neurodegeneration. This concept guides current diagnostic approaches, as well as clinical trials, that focus on detecting or removing amyloid or tau from the brain. The semantic variant of primary progressive aphasia (svPPA), a clinical syndrome associated with frontotemporal lobar degeneration (FTLD) pathology, is usually associated with the molecular pathology TDP-C, but there are cases with TDP-B and Pick's disease. The existing literature on the clinical differentiation ...
Source: Neurocase - March 6, 2018 Category: Neurology Authors: Caplan A, Marx G, Elofson J, Lis C, Grinberg L, Miller B, Rosen H Tags: Neurocase Source Type: research

Novel cognitive insights from the first year after bi-thalamic infarct.
Abstract Neuropsychological consequences of bi-thalamic damage are scarcely known. This case study documents cognitive (in particular memory and executive) functioning in a man with a medial bi-thalamic infarct in the first year (8 and 12 months) post injury. NG showed persistent memory (including autobiographical) impairment, but improved executive functions at one year post injury. On a response inhibition task his speed of response improved but his ability to inhibit a "prepotent" automatic response declined, corresponding to an increase in behavioral disinhibition. Despite this, he showed intact...
Source: Neurocase - March 1, 2018 Category: Neurology Authors: Baird A, Robinson GA Tags: Neurocase Source Type: research

Unilateral Apraxic Agraphia without Ideomotor Apraxia from a callosal lesion in a patient with Marchiafava-Bignami disease.
Abstract Apraxic agraphia can be caused by left hemispheric cerebral lesions in the area that contains the spatial representations of the movements required to write, from a lesion in, or connections to, the frontal premotor cortex that converts these spatial representations to motor programs (Exner's area).  A right-handed woman with Marchiafava Bignami disease and lesions of the genu and splenium of her corpus callosum had apraxic agraphia without ideomotor apraxia of her left. A disconnection of Exner's area in the left hemisphere from the right hemisphere's premotor and motor areas may have led to her ina...
Source: Neurocase - February 26, 2018 Category: Neurology Authors: Kesayan T, Heilman KM Tags: Neurocase Source Type: research

Heterozygous genotype at codon 129 correlates with prolonged disease course in Heidenhain variant sporadic CJD: case report.
Abstract Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapid and fatal neurodegenerative disease defined by misfolded prion proteins accumulating in the brain. A minority of cases initially present with posterior cortical atrophy (PCA) phenotype, also known as Heidenhain variant or visual variant CJD. This case provides further evidence of sCJD presenting as PCA. The case also provides evidence for early DWI changes and cortical atrophy over 30 months before neurologic decline and subsequent death. The prolonged disease course correlates with prion protein codon 129 heterozygosity and coexistence of multiple...
Source: Neurocase - February 13, 2018 Category: Neurology Authors: Townley RA, Dawson ET, Drubach DA Tags: Neurocase Source Type: research

Massive weight loss following deep brain stimulation of the nucleus accumbens in a depressed woman.
i B Abstract Obese individuals share behavioral characteristics with drug/alcohol addicts as well as obsessive compulsive disease. Deep brain stimulation (DBS) has been used successfully in these disorders, thus warranting an evaluation in obesity. A woman with treatment-resistant depression as well as severe obesity was selected for DBS of the nucleus accumbens (NAcc) bilaterally with depression being the primary and obesity being the secondary target of treatment. Compared to earlier bariatric surgery, the patient showed accelerated weight loss after DBS. Also, depression was significantly reduced. The current c...
Source: Neurocase - February 1, 2018 Category: Neurology Authors: Tronnier VM, Rasche D, Thorns V, Alvarez-Fischer D, Münte TF, Zurowski B Tags: Neurocase Source Type: research

Retrieving autobiographical experience of painful events in a phantom limb: brain concomitants in a case report.
Abstract We report the case of a patient who had an important experience with painful events, allowing the investigation of brain concomitants to painful (P) memories in fMRI. The patient had to recall P events that were contrasted with non-painful (NP) memories. Painful memories of the right lower limb activated the left paracentral lobule,fronto-insular operculum and superior parietal cortex. Additionally, whilst the recall of non-painful events activated the hippocampus, the recall of painful events did not enhance the hippocampal signal to significant levels. These suggest that brain activations...
Source: Neurocase - February 1, 2018 Category: Neurology Authors: Borg C, Faillenot I, Peyron R, Laurent B Tags: Neurocase Source Type: research

Slowly progressive behavioral frontotemporal dementia with C9orf72 mutation. Case report and review of the literature.
We present a 86-year-old woman without relevant medical history and two brothers who died by dementia, who started at 55 years with depression and personality changes with ongoing worsening (>30 years) and functional decline. Screening dementia blood test and brain magnetic resonance imaging did not show results that pointed to a secondary cause. The patient met the diagnostic criteria for possible behavioral frontotemporal dementia with a slow progression (bvFTD-SP), suggesting a benign variant. A genetic study confirmed a C9ORF72 hexanucleotide expansion, making this the sixth case mentioned in the literatur...
Source: Neurocase - January 22, 2018 Category: Neurology Authors: Llamas-Velasco S, García-Redondo A, Herrero-San Martín A, Puertas Martín V, González-Sánchez M, Pérez-Martínez DA, Villarejo-Galende A Tags: Neurocase Source Type: research

Semantic language deficit developing following herpes simplex encephalitis: reorganization "cannibalising" language centers?
We describe a patient with HSVE who presented with a highly unusual late-onset language syndrome, which may reflect distal cortical changes after her original injury. PMID: 29350572 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - January 19, 2018 Category: Neurology Authors: Mangelsdorf S, Kelso W, Velakoulis D, Walterfang M Tags: Neurocase Source Type: research

The neural substrates of improved phonological processing following successful treatment in a case of phonological alexia and agraphia.
Abstract Phonological deficits are common in aphasia after left-hemisphere stroke, and can have significant functional consequences for spoken and written language. While many individuals improve through treatment, the neural substrates supporting improvements are poorly understood. We measured brain activation during pseudoword reading in an individual through two treatment phases. Improvements were associated with greater activation in residual left dorsal language regions and bilateral regions supporting attention and effort. Gains were maintained, while activation returned to pre-treatment levels. This case de...
Source: Neurocase - January 19, 2018 Category: Neurology Authors: DeMarco AT, Wilson SM, Rising K, Rapcsak SZ, Beeson PM Tags: Neurocase Source Type: research

Neuronal activity organization features in human consciousness: summary of introductory remarks in a dialogue with neurological surgery residents.
Abstract Agreeing with Damasio's statement defining the "process" of consciousness, we propose the self as created by mind-based knowledge and a combination of images of an organism's intentional motor responses interacting with its environment. The lemniscal system, with plastic capabilities, manages gravity in voluntary movement. The spinal segment motor reflex represents the schema of gravity-managing neuronal activity, and it can become "nested" in cortical areas participating in consciousness-building, allowing consideration of the brain as a hyper-evolved nervous system seg...
Source: Neurocase - January 9, 2018 Category: Neurology Authors: Vera CL, Patel SJ, Henderson FC, Roberts DR, Krishna V Tags: Neurocase Source Type: research

Posterior cortical atrophy: a single case cognitive and radiological follow-up.
, Manes F, Roca M Abstract Posterior cortical atrophy (PCA) is a rare neurodegenerative syndrome characterized by initial predominant visuoperceptual deficits followed by a progressive decline in other cognitive functions. This syndrome has not been as thoroughly described as other dementias, particularly from a neuropsychological evolution perspective with only a few studies describing the evolution of its cognitive progression. In this investigation we review the literature on this rare condition and we perform a 7-year neuropsychological and neuroradiological follow-up of a 64-year-old man with PCA. The subject...
Source: Neurocase - January 8, 2018 Category: Neurology Authors: Esteves S, Ramirez Romero DA, Torralva T, Martínez Cuitiño M, Herndon S, Couto B, Ibañez A, Manes F, Roca M Tags: Neurocase Source Type: research

Multiproteinopathy, neurodegeneration and old age: a case study.
Abstract A complex spectrum of mixed brain pathologies is common in older people. This clinical pathologic conference case study illustrates the challenges of formulating clinicopathologic correlations in late-onset neurodegenerative diseases featuring cognitive-behavioral syndromes with underlying multiple proteinopathy. Studies on the co-existence and interactions of Alzheimer's disease (AD) with neurodegenerative non-AD pathologies in the aging brain are needed to understand the pathogenesis of neurodegeneration and to support the development of diagnostic biomarkers and therapies. PMID: 29307276 [PubMed -...
Source: Neurocase - January 6, 2018 Category: Neurology Authors: Rojas JC, Stephens ML, Rabinovici GD, Kramer JH, Miller BL, Seeley WW Tags: Neurocase Source Type: research

Mixed language dominance: insights from a case of unexpected fluent aphasia with semantic jargon resulting from massive left perisylvian lesion.
We report a right-handed patient with a massive lesion in left perisylvian language cortex, who unexpectedly presented with fluent aphasia with semantic jargon. Language deficits were assessed with a comprehensive battery of language tests. Comprehension, naming, reading, and writing were severely impaired, and verbal expression was moderately fluent with semantic jargon. Although the patient's lesion included brain areas typically essential for motor speech coordination, he was neither nonfluent nor apraxic. He exhibited strikingly unexpected aphasia with semantic jargon and prominent comprehension deficits, suggesting th...
Source: Neurocase - December 25, 2017 Category: Neurology Authors: Kasselimis D, Potagas C, Simos P, Evdokimidis I, Whitaker H Tags: Neurocase Source Type: research

Alterations of emotional reactivity following righttemporal lobectomy.
Abstract To learn if a right temporal lobectomy altered a patient's autonomic and cognitive responses to aversive and appetitive pictures, we investigated a man whose right anterior temporal lobe and amygdala had been resected to remove an arteriovenous malformation. This patient revealed unusually low self-reports of arousal with a lack of heightened skin conductance responses to aversive pictures. For pleasant pictures, his self-reports of arousal and his physiological responses were similar to healthy men. This patient's responses are consistent with a bivalent model of emotional processing, with a right tempor...
Source: Neurocase - December 20, 2017 Category: Neurology Authors: Morris MK, Bowers D, Williamson J, Heilman KM Tags: Neurocase Source Type: research

The painter from Sinaloa: artistic analysis of a case of spatial agnosia and neglect of visual shapes.
end N Abstract Paintings produced spontaneously by patients with neurological lesions represent a fascinating opportunity to analyze some aspects of the underlying disease and involved brain mechanisms. Many cases of artists who have suffered spatial neglect following a neurological disease have been reported in the literature. However, only a few studies evaluating the different subtypes of graphic neglect and aspects related to the construction of perspective (three dimensionality) in works of art have been published. In the present article, we present the case of an artist who, after resection of a central neur...
Source: Neurocase - November 29, 2017 Category: Neurology Authors: Orjuela-Rojas JM, Sosa-Ortiz AL, Díaz-Victoria AR, Marrufo Melendez OR, Leyva Townsend N Tags: Neurocase Source Type: research

Accelerated Long-Term Forgetting Limited to the Recollection Component of Recognition Memory in a Temporal Lobe Epileptic Patient.
Abstract Accelerated long-term forgetting (ALF) is a frequent finding in patients with temporal lobe epilepsy (TLE). Here we report the case of a TLE patient who complained of marked difficulties in remembering personal events and information even though repeated neuropsychological assessments had failed to detect any deficit on common laboratory memory tests. The patient underwent an experimental investigation that involved estimating recollection and familiarity processes in the performance on verbal and visual recognition tests, over intervals ranging from 10 minutes to 7 days. Results showed accelerated forget...
Source: Neurocase - October 26, 2017 Category: Neurology Authors: Carlesimo GA, Daniele A, Lombardi MG, Lacidogna G, La Cara A, Fadda L, Caltagirone C Tags: Neurocase Source Type: research

Reorganized neural activation in motor cortex following subdural fluid collection: an fMRI and DTI study.
We report a patient with a cavernous malformation involving the right lentiform nucleus. Pre-surgical planning included fMRI localization of language, motor, and sensory processing, and DTI of white matter tracts. fMRI results revealed no activation near the planned resection zone. However, post-surgery the patient developed a subdural fluid collection, which applied pressure to the primary motor cortex (M1). Follow-up scans revealed that motor activation had shifted due to pressure, and then shifted to a new location after the fluid collection subsided. This case report suggests that long-term neural reorganization can oc...
Source: Neurocase - October 24, 2017 Category: Neurology Authors: Gould L, Kelly ME, Mickleborough MJS, Ekstrand C, Brymer K, Ellchuk T, Borowsky R Tags: Neurocase Source Type: research

The use of cannabidiol for seizure management in patients with brain tumor-related epilepsy.
Abstract Epilepsy, commonly encountered by patients with brain tumors, is often refractory to standard therapies. Our aim was to examine the safety and efficacy of pharmaceutical grade cannabidiol (CBD; Epidiolex, GW Pharmaceuticals) in those patients with epilepsy with concomitant tumors enrolled in The University of Alabama at Birmingham CBD Program (NCT02700412 and NCT02695537). Of the three patients with refractory seizures and a history of a primary brain tumor, two had improvement in seizure frequency and all three had improvement in seizure severity. These pilot results suggest that CBD should be further st...
Source: Neurocase - October 24, 2017 Category: Neurology Authors: Warren PP, Bebin EM, Nabors LB, Szaflarski JP Tags: Neurocase Source Type: research

Evaluating the roles of left middle frontal gyrus in word production using electrocorticography.
Abstract To assess the specific roles of left middle frontal gyrus (LMFG) in word production, electrocorticography signals were recorded from an epilepsy patient when he participated in language tasks. We found three sites of LMFG showed high-gamma perturbations with distinct patterns across tasks; and neural activities elicited in the same tasks shared similar patterns, while those elicited by stimuli leading to the same articulations did not. These findings confirmed that the LMFG takes active parts in word production, and suggested that it may serve as a temporal perceptual information storage space, supporting...
Source: Neurocase - October 20, 2017 Category: Neurology Authors: Wen J, Yu T, Liu L, Hu Z, Yan J, Li Y, Li X Tags: Neurocase Source Type: research

A patient with aphasia using the nonsemantic lexical route for Kanji reading.
We report a patient with aphasia, caused by cerebral hemorrhage, who probably used the nonsemantic lexical route when reading words aloud. To investigate the mechanisms underlying her reading dysfunction, we analyzed her reading abilities using the Dual-Route Cascaded Model. Language tests resulted in low correct percentages for both reading comprehension and reading nonwords aloud, suggesting problems in the semantic system and the nonlexical route. Conversely, the patient showed high scores on the reading words aloud task. Although she failed to understand many inconsistent-atypical words in the reading comprehension tes...
Source: Neurocase - October 16, 2017 Category: Neurology Authors: Hashimoto K, Uno A, Sambai A, Mizumoto G Tags: Neurocase Source Type: research

Coprolalia in aphasic patients with stroke: a longitudinal observation from the BLAS2T database.
This report discusses linguistic performance in four cases from the BLAS2T database who demonstrated coprolalia as an irresistible urge to say obscene words.  Coprolalia was found to partly resolve in a 30-day follow-up in three cases. Recognition of coprolalia and language recovery patterns in bilingual aphasic patients with stroke would potentially lead to their even better individualized care and neurolinguistic/cognitive rehabilitation. PMID: 29027506 [PubMed - as supplied by publisher] (Source: Neurocase)
Source: Neurocase - October 13, 2017 Category: Neurology Authors: Afshangian F, Nami M, Abolhasani Foroughi A, Rahimi A, Husak R, Fabbro F, Tomasino B, Kremer C, BLAS2T (Bilingual Aphasia in Stroke-Study Team) Tags: Neurocase Source Type: research

Partial resection of presurgical fMRI activation is associated with a postsurgical loss of language function after frontal lobe epilepsy surgery.
We describe five patients with frontal lobe epilepsy who underwent electrocortical stimulation (ES) for language localization and language functional magnetic resonance imaging (fMRI) prior to epilepsy surgery. Six months after surgery, three patients suffered from a drop of verbal fluency. In all of them, frontal areas with presurgical language fMRI activity were resected. Our results suggest that resection in regions of areas with presurgical fMRI activation is not without risk for a postsurgical loss of function, even when ES results were negative for language function in these areas. Using fMRI activations might be spe...
Source: Neurocase - September 27, 2017 Category: Neurology Authors: Labudda K, Mertens M, Kalbhenn T, Schulz R, Woermann FG Tags: Neurocase Source Type: research

Clinical implications of interictal epileptiform discharges in cognitive functioning in CEC syndrome with evolution into epileptic encephalopathy.
Abstract In epileptic encephalopathies (EE), interictal epileptiform discharges (IEDs) contribute to cognitive impairment. The EE process has been studied in a patient affected by epilepsy with occipital calcification and celiac disease (CEC syndrome) by combining the administration of brain area stimulus specific (visual and auditory) reaction times (RT) during continuous EEG monitoring with the off-line reconstruction of auditory and visual evoked potentials (EP). Visual RT and VEP were abnormal only if recorded concomitantly to the IEDs. Auditory RT and EP were normal. When the EE process is going on, IEDs tran...
Source: Neurocase - September 20, 2017 Category: Neurology Authors: Bonanni P, Negrin S, Antoniazzi L, Da Rold M, Fabbro F, Serafini A Tags: Neurocase Source Type: research

Fractionating controlled memory processes and recall of context in recognition memory: a case report.
Abstract Recollection has been described as both a recognition memory judgment requiring cognitive control and the ability to retrieve contextual information about a prior occurrence. At the core of this article is the question whether or not these two subcomponents of recollection are dissociable in amnesia. In three experiments, we explored the influence of exclusion task instructions on performance in a single case (CJ), with the view to understand the relative contributions of control and source memory to recognition memory decisions. First, contrasting findings were obtained between tasks requiring strategic ...
Source: Neurocase - September 1, 2017 Category: Neurology Authors: Pauly-Takacs K, Moulin CJA Tags: Neurocase Source Type: research

Present and future selves in Parkinson's disease.
Abstract The study of the self in neuropsychological patients raises not only theoretical questions on the relationships between the self, autobiographical memory (AM), and episodic future thinking but also clinical issues for patients' daily life and care. We addressed this issue in Parkinson's disease patients for whom AM and future thinking impairments have been documented. All patients and controls generated and dated up past and future self-images and provided associated past and future events. Our findings suggest a subtle pattern of preservation/impairment of different dimensions (quantitative and qualitati...
Source: Neurocase - August 13, 2017 Category: Neurology Authors: Ernst A, Allen J, Dubourg L, Souchay C Tags: Neurocase Source Type: research

Reading on the right when there's nothing left? Probabilistic tractography reveals hemispheric asymmetry in pure alexia.
We present a patient with reading inexpertise and right hemianopia following left posterior cerebral artery (PCA) stroke. We examine the extent of disruption to reading performance and the extent of white matter tract damage relative to a patient with more limited PCA infarction and isolated right hemianopia. We show white matter disconnection of the temporal occipital fusiform cortex in our pure alexia patient. Connectivity-based laterality indices revealed right hemisphere laterality in the alexia patient; this was not associated with improved reading function. We speculate that the degree of premorbid laterality may be ...
Source: Neurocase - August 9, 2017 Category: Neurology Authors: Veldsman M, Loetscher T, Wood A, Brodtmann A Tags: Neurocase Source Type: research

Enhanced cognition and emotional recognition, and reduced obsessive compulsive symptoms in two adults with high-functioning autism as a result of deep Transcranial Magnetic Stimulation (dTMS): a case report.
We report reduced repetitive behaviors similar to obsessive compulsive disorder and improved emotional recognition and cognitive abilities in two young patients diagnosed with high-functioning Autism as a result of deep transcranial magnetic stimulation (dTMS). The patients received daily high-frequency (5 Hz) dTMS with HAUT-coil over the medial prefrontal cortex for a period of 5-6 weeks. A computerized cognitive battery, tasks for testing emotional recognition, and clinical questionnaires were used to measure the effects of treatment. TMS might have modulated networks related to metalizing abilities and self-re...
Source: Neurocase - August 8, 2017 Category: Neurology Authors: Avirame K, Stehberg J, Todder D Tags: Neurocase Source Type: research

What role for prism adaptation in the rehabilitation of pure neglect dyslexia?
Abstract Prism adaptation (PA) has been successfully applied in the rehabilitation of spatial neglect, with significant transfer to classic neglect tests and activities of daily living. However, well-controlled studies were unable to replicate these findings, and recent reports suggest that PA may affect selectively visuo-motor symptoms. Here, a patient with pure left neglect dyslexia was tested before, immediately after, and 24 h after PA. Despite a significant adaptation aftereffect adaptation had no effect omissions, substitutions and letter-based errors. PA does not affect pure neglect dyslexia and should ther...
Source: Neurocase - August 4, 2017 Category: Neurology Authors: Ptak R Tags: Neurocase Source Type: research

Hyper-brain connectivity in binge drinking college students: a diffusion tensor imaging study.
Abstract The current study used diffusion tensor imaging to examine patterns/degree of brain connectivity in 12 college-aged binge drinking (BD) and 12 moderate drinking individuals. Voxel-level and region-of-interest analyses revealed increased connectivity of the BD brain in the right corona radiata, right external capsule, and both the right and left cingulum. Also, fractional anisotropy and axial diffusivity values of these regions correlated with a number of drinking behaviors of the BD as well as both groups combined. It is hypothesized that increased connectivity in the BD may produce difficulties with regu...
Source: Neurocase - July 10, 2017 Category: Neurology Authors: Kashfi K, Al-Khalil K, Hou J, Fang D, Anderson R, Rajmohan R, Syapin P, O'Boyle MW Tags: Neurocase Source Type: research