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https://www.readbyqxmd.com/read/30139309/a-case-of-capgras-syndrome-and-folie-%C3%A3-duex-in-monozygotic-twins
#1
Krzysztof Gbyl, Martin Balslev Jørgensen
Both Capgras syndrome and folie à deux (insanity of two) are rare and fascinating psychopathological syndromes. Their etiology and the nosological position remain unclear. 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.
August 24, 2018: Neurocase
https://www.readbyqxmd.com/read/30112957/pathological-correlates-of-white-matter-hyperintensities-in-a-case-of-progranulin-mutation-associated-frontotemporal-dementia
#2
Ione O C Woollacott, Martina Bocchetta, Carole H Sudre, Basil H Ridha, Catherine Strand, Robert Courtney, Sebastien Ourselin, M Jorge Cardoso, Jason D Warren, Martin N Rossor, Tamas Revesz, Nick C Fox, Janice L Holton, Tammaryn Lashley, Jonathan D Rohrer
White matter hyperintensities (WMH) are often seen on MRI brain scans in frontotemporal dementia (FTD) due to progranulin (GRN) mutations, but their pathological correlates are unknown. 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...
August 16, 2018: Neurocase
https://www.readbyqxmd.com/read/30088953/mitochondrial-membrane-protein-associated-neurodegeneration-a-case-report-and-literature-review
#3
Pavel Dušek, David Školoudík, Jan Roth, Petr Dušek
Mitochondrial membrane protein-associated neurodegeneration (MPAN) is an autosomal recessive disorder caused by mutation in the C19orf12 gene. 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...
August 8, 2018: Neurocase
https://www.readbyqxmd.com/read/30035662/illustrating-where-spatial-perception-versus-memory-based-representation-spatial-neglect-in-a-distinguished-artist-a-case-report
#4
Kimberly Hreha, Amit Chaudhari, Yekyung Kong, Prathusha Maduri, A M Barrett
Spatial neglect is a disorder of attention, perception, and processing of stimuli in contralesional space. The heterogeneous behaviors involve diverse neuroanatomical mechanisms. Anecdotal evidence suggests that neural circuitry of active spatial perception may differ from that used to encode spatial memory. These differences can escape detection by conventional assessments, thereby leading to missed diagnoses. We present a case, in an artist who demonstrates selective impairment in a contralesional space during active Where spatial perception...
July 23, 2018: Neurocase
https://www.readbyqxmd.com/read/30015554/bilateral-upper-limb-rehabilitation-with-videogame-based-feedback-in-corticobasal-degeneration-a-case-reports-study
#5
Francesca Romana Fusco, Marco Iosa, Augusto Fusco, Stefano Paolucci, Giovanni Morone
Corticobasal degeneration (CBD) is a neurodegenerative disorder characterized by a combination of cortical and basal ganglia signs. 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...
July 17, 2018: Neurocase
https://www.readbyqxmd.com/read/29987978/early-stage-memory-impairment-visual-hallucinations-and-myoclonus-combined-with-temporal-lobe-atrophy-predict-alzheimer-s-disease-pathology-in-corticobasal-syndrome
#6
Na-Yeon Jung, Jae-Hyeok Lee, Young Min Lee, Jin-Hong Shin, Myung-Jun Shin, Myung Jun Lee, Kyoungjune Pak, Chungsu Hwang, Jae Woo Ahn, Suk Sung, Kyung-Un Choi, Gi Yeong Huh, Eun-Joo Kim
Corticobasal syndrome (CBS) is a typical phenotype of corticobasal degeneration (CBD). However, autopsy series have shown that many CBS cases emerge from various types of non-CBD pathology. 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...
July 10, 2018: Neurocase
https://www.readbyqxmd.com/read/29969053/a-new-mapt-deletion-in-a-case-of-speech-apraxia-leading-to-corticobasal-syndrome
#7
Giulia Mazzon, Alina Menichelli, Antonella Fabretto, Tatiana Cattaruzza, Paolo Manganotti
Speech apraxia is a disorder of speech motor planning/programming leading to slow rate, articulatory distortion, and distorted sound substitutions. 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...
July 3, 2018: Neurocase
https://www.readbyqxmd.com/read/29882467/a-progressive-breakdown-of-the-body-in-space
#8
Evie Kourtidou, Dimitrios Kasselimis, George Makrydakis, Lina Chatziantoniou, Andreas Kyrozis, Ioannis Evdokimidis, Constantin Potagas
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 progressively emerging syndrome of self-management loss within peripersonal space is observed...
June 8, 2018: Neurocase
https://www.readbyqxmd.com/read/29799310/clinical-and-imaging-progression-over-10-years-in-a-patient-with-primary-progressive-apraxia-of-speech-and-autopsy-confirmed-corticobasal-degeneration
#9
Katerina A Tetzloff, Joseph R Duffy, Edythe A Strand, Mary M Machulda, Sarah M Boland, Rene L Utianski, Hugo Botha, Matthew L Senjem, Christopher G Schwarz, Keith A Josephs, Jennifer L Whitwell
Primary progressive apraxia of speech (PPAOS) is a neurodegenerative disorder in which AOS is the sole presenting complaint. 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...
April 2018: Neurocase
https://www.readbyqxmd.com/read/29774783/connecting-the-dots-an-association-between-opioids-and-acute-hippocampal-injury
#10
Jed A Barash, W Andrew Kofke
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 and pathophysiological evidence that supports this hypothesis is presented, as are potential underlying mechanisms...
April 2018: Neurocase
https://www.readbyqxmd.com/read/29764303/relief-from-intractable-phantom-pain-by-combining-psilocybin-and-mirror-visual-feedback-mvf
#11
Vilayanur Ramachandran, Chaipat Chunharas, Zeve Marcus, Timothy Furnish, Albert Lin
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...
April 2018: Neurocase
https://www.readbyqxmd.com/read/29693492/interpersonal-gargalesthesia
#12
Vilayanur Subramanian Ramachandran, Claudia Alyse Sellers
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...
April 2018: Neurocase
https://www.readbyqxmd.com/read/29606081/persistent-anterograde-amnesia-due-to-the-artery-of-percheron-occlusion-a-case-report
#13
Birsen Ince, Furkan Asan
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...
April 2018: Neurocase
https://www.readbyqxmd.com/read/29569990/molecular-neuroimaging-in-primary-progressive-aphasia-with-predominant-agraphia
#14
Rene L Utianski, Joseph R Duffy, Rodolfo Savica, Jennifer L Whitwell, Mary M Machulda, Keith A Josephs
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...
April 2018: Neurocase
https://www.readbyqxmd.com/read/29508660/a-case-of-semantic-variant-primary-progressive-aphasia-with-pick-s-pathology
#15
Alyssa Caplan, Gabe Marx, Jonathan Elofson, Claire Lis, Lea Grinberg, Bruce Miller, Howard Rosen
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...
April 2018: Neurocase
https://www.readbyqxmd.com/read/29508646/pattern-of-cerebral-hypoperfusion-according-to-the-clinical-staging-in-dementia-with-lewy-bodies
#16
Kyung Won Park, Yun Jeong Hong, Jong Hwan Park, Hyuntae Park, Sang Myung Cheon, Jae Woo Kim, Byeong C Kim, Young Jin Jeong, Hyun Jin Yoon, Do-Young Kang
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...
April 2018: Neurocase
https://www.readbyqxmd.com/read/29516779/erratum
#17
(no author information available yet)
No abstract text is available yet for this article.
February 2018: Neurocase
https://www.readbyqxmd.com/read/29493434/novel-cognitive-insights-from-the-first-year-after-bi-thalamic-infarct
#18
Amee Baird, Gail A Robinson
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...
February 2018: Neurocase
https://www.readbyqxmd.com/read/29482459/unilateral-apraxic-agraphia-without-ideomotor-apraxia-from-a-callosal-lesion-in-a-patient-with-marchiafava-bignami-disease
#19
T Kesayan, K M Heilman
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 inability to write with her left hand...
February 2018: Neurocase
https://www.readbyqxmd.com/read/29436943/heterozygous-genotype-at-codon-129-correlates-with-prolonged-disease-course-in-heidenhain-variant-sporadic-cjd-case-report
#20
Ryan A Townley, Elliot T Dawson, Daniel A Drubach
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 prion strains...
February 2018: Neurocase
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