1,000 results match your criteria Neurocase[Journal]


TARDBP mutation associated with semantic variant primary progressive aphasia, case report and review of the literature.

Neurocase 2019 Feb 16:1-5. Epub 2019 Feb 16.

a Department of Neurology , Hospital Universitario 12 de Octubre , Madrid , Spain.

Semantic variant primary progressive aphasia (svPPA) is a clinical syndrome included in the frontotemporal dementia (FTD) spectrum. Unlike other forms of FTD, it is sporadic in the majority of cases and not commonly associated with motor neuron disease (MND). We describe a case of svPPA associated with MND in the same family, due to a mutation of the transactive response DNA binding protein (TARDBP) gene, and review the literature. Read More

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http://dx.doi.org/10.1080/13554794.2019.1581225DOI Listing
February 2019

An unusual presentation of late-onset Alexander's disease with slow orthostatic tremor and a novel GFAP variant.

Neurocase 2019 Feb 12:1-3. Epub 2019 Feb 12.

a Department of Neurology , Mayo Clinic , Rochester , MN , USA.

Alexander disease (AxD) is a leukodystrophy, described in infantile, juvenile and adult onset forms, due to mutations in the glial fibrillary acid protein (GFAP) gene. Adult-onset AxD (AOAD) has a range of clinical and radiographic phenotypes with the oldest reported onset in the seventh decade.We report a case of AOAD, with onset in the eighth decade, presenting with slow variant orthostatic tremor, which has not been previously described. Read More

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http://dx.doi.org/10.1080/13554794.2019.1580749DOI Listing
February 2019

A case report of the neurocognitive and behavioral phenotype of mosaic trisomy 14.

Neurocase 2019 Feb 4:1-5. Epub 2019 Feb 4.

a Department of Psychology , Drexel University , Philadelphia , PA , USA.

Mosaic trisomy 14 is associated with impaired intellectual functioning, although no study has examined the cognitive-behavioral profile associated with the syndrome. This study provides the first case description of the cognitive-behavioral phenotype associated with mosaic trisomy 14 by contrasting the performance an adolescent female (T14) with a group of females with Down syndrome (DS; n=9). T14 performed below age expectations on most direct assessments and demonstrated weaknesses relative to the DS group on aspects of language, adaptive functioning, and executive functioning. Read More

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http://dx.doi.org/10.1080/13554794.2019.1572196DOI Listing
February 2019
1 Read

Altered perception might be a symptom of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.

Neurocase 2019 Jan 25:1-4. Epub 2019 Jan 25.

b Department of Neuropsychiatry , Keio University School of Medicine , Tokyo , Japan.

Most patients with N-methyl-D-aspartate receptor (NMDAR) encephalitis initially present with psychiatric symptoms. Although a delayed diagnosis may lead to a poor outcome, psychiatric symptoms that could differentiate anti-NMDAR encephalitis from other psychoses have not been fully investigated. We evaluated two patients with anti-NMDAR encephalitis who were observed by psychiatrists from onset throughout the course of disease. Read More

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https://www.tandfonline.com/doi/full/10.1080/13554794.2019.1
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http://dx.doi.org/10.1080/13554794.2019.1573260DOI Listing
January 2019
9 Reads

Effect of kinesthetic illusion induced by visual stimulation on ankle dorsiflexion dysfunction in a stroke patient: ABAB single-case design.

Neurocase 2019 Jan 18:1-5. Epub 2019 Jan 18.

a Graduate School of Human Health Sciences , Tokyo Metropolitan University , Tokyo , Japan.

The purpose of this study was to investigate the effect of simultaneous intervention with the kinesthetic illusion induced by visual stimulation (KiNvis) and voluntary exercise on ankle dorsiflexion dysfunction in a patient with right-sided stroke hemiparesis. Within an ABAB single-case design, we conducted two phases each lasting five days. Phase A represented the baseline during which only voluntary ankle dorsiflexion (VAD) was performed. Read More

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http://dx.doi.org/10.1080/13554794.2019.1566477DOI Listing
January 2019

Temporal-occipital glioblastoma presenting with Alice in Wonderland Syndrome in a patient with a long-time history of migraine without aura.

Neurocase 2019 Jan 2:1-3. Epub 2019 Jan 2.

a Department of Human Neurosciences , "Sapienza" University of Rome , Rome , Italy.

Alice in Wonderland Syndrome (AIWS) is a rare perceptual disorder characterized by an erroneous perception of the body or the surrounding space. AIWS may be caused by different pathologies, ranging from infections to migraine. We present the case of a 54-year-old man, with a long-time history of migraine without aura, diagnosed with AIWS due to a glioblastoma located in the left temporal-occipital junction. Read More

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http://dx.doi.org/10.1080/13554794.2018.1562079DOI Listing
January 2019

Neuro-Behçet's disease presenting as an isolated progressive cognitive and behavioral syndrome.

Neurocase 2018 Dec 25:1-4. Epub 2018 Dec 25.

a Second Division of Neurology , Center for Rare Neurological and Neuromuscular Diseases & Inter University Center for Research in Neurosciences, Department of Advanced Medical and Surgical Sciences, University of Campania Luigi Vanvitelli , Naples , Italy.

Behçet's disease is a chronic inflammatory disorder manifesting as a vasculitis that affects arteries and veins of any size. Up to 44% of cases may also present with neurological symptoms, thus defining Neuro-Behçet's disease. We describe a case of Neuro-Behçet's disease characterized by progressive behavioral and cognitive deterioration prevailing over other neurological symptoms, without evident systemic involvement. Read More

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http://dx.doi.org/10.1080/13554794.2018.1561898DOI Listing
December 2018

Longitudinal neuropsychological outcomes in treatment-resistant depression following bed nucleus of the stria terminalis-area deep brain stimulation: a case review.

Neurocase 2018 Dec 3:1-7. Epub 2018 Dec 3.

e Medtronic , Mumbai , India.

Studies have demonstrated the effectiveness of deep brain stimulation (DBS) as a treatment modality for psychiatric conditions. We present a case reviewing the longitudinal neuropsychological performance outcomes following bed nucleus of the stria terminalis-area (BNST) DBS in a patient with treatment-resistant depression (TRD). The cognitive safety of DBS is well documented for various targets, however cognitive outcomes of BNST-area DBS have not been extensively reported for patients with TRD. Read More

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http://dx.doi.org/10.1080/13554794.2018.1549680DOI Listing
December 2018

Phelan-McDermid syndrome in adult patient with atypical bipolar psychosis repeatedly triggered by febrility.

Neurocase 2018 Aug 30;24(4):227-230. Epub 2018 Oct 30.

a Department of Molecular and Biochemical Genetics, Institute of Medical Biology, Genetics and Clinical Genetics , Comenius University Faculty of Medicine and University Hospital in Bratislava , Bratislava , Slovakia.

Phelan-McDermid syndrome (PMD) is a rare genetic condition with only a few cases describing patients diagnosed as adults. We describe a long diagnostic odyssey of a 30-year-old woman who was diagnosed with Phelan-McDermid syndrom. Array comparative genomic hybridization analysis confirmed a 22q13. Read More

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http://dx.doi.org/10.1080/13554794.2018.1542007DOI Listing
August 2018
5 Reads

Left hemispatial neglect with a splenial lesion.

Neurocase 2018 Aug 28;24(4):220-226. Epub 2018 Oct 28.

d Neurology Service and GRECC , Malcom Randall Veterans Affairs Medical Center , Gainesville , FL , USA.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/13554794.2018.1
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http://dx.doi.org/10.1080/13554794.2018.1539501DOI Listing
August 2018
27 Reads

A neuropathologic diagnosis of Alzheimer's disease in an older adult with HIV-associated neurocognitive disorder.

Neurocase 2018 Aug 10;24(4):213-219. Epub 2018 Oct 10.

a Memory and Aging Center, Department of Neurology , University of California, San Francisco , California, CA , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1530362DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6226354PMC
August 2018
11 Reads

Verbal adynamia in parkinsonian syndromes: behavioral correlates and neuroanatomical substrate.

Neurocase 2018 Aug 6;24(4):204-212. Epub 2018 Oct 6.

b Dementia Research Centre , UCL Institute of Neurology , London , UK.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/13554794.2018.1
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http://dx.doi.org/10.1080/13554794.2018.1527368DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6234546PMC
August 2018
14 Reads

Anti-NMDA receptor encephalitis presenting as isolated aphasia in an adult.

Neurocase 2018 Aug 6;24(4):188-194. Epub 2018 Oct 6.

a 1st Department of Neurology , National and Kapodistrian University of Athens, Eginition Hospital , Athens , Greece.

Anti-NMDA receptor (NMDA-r) encephalitis is a relatively rare cause of autoimmune encephalitis with divergent clinical presentations. 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. Read More

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https://www.tandfonline.com/doi/full/10.1080/13554794.2018.1
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http://dx.doi.org/10.1080/13554794.2018.1524915DOI Listing
August 2018
24 Reads

Tracking of neurocognitive outcomes over time in children with perinatal stroke and associated complex medical conditions: a case series.

Neurocase 2018 Aug 26;24(4):195-203. Epub 2018 Sep 26.

b Department of Neuropsychology , Children's Healthcare of Atlanta , Atlanta , GA , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1525410DOI Listing
August 2018
2 Reads

Klüver & Bucy syndrome: an investigation of social and affective cognition.

Neurocase 2018 Aug 24;24(4):180-187. Epub 2018 Sep 24.

b Programa de Pós-Graduação em Neurociências , Universidade Federal de Minas Gerais (UFMG) , Belo Horizonte , Brazil.

Klüver-Bucy syndrome (KBS) leads to important behavioral symptoms and social maladaptation. Rarely described, no previous study has investigated its social and affective cognitive profile. We report the case of ASP, a patient who developed a complete KBS at 9 years that evolved into an incomplete KBS. Read More

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https://www.tandfonline.com/doi/full/10.1080/13554794.2018.1
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http://dx.doi.org/10.1080/13554794.2018.1524497DOI Listing
August 2018
10 Reads

A case of Capgras syndrome and folie à duex in monozygotic twins.

Neurocase 2018 Jun 24;24(3):175-179. Epub 2018 Aug 24.

a Psychiatric Centre Copenhagen , Copenhagen University Hospital , Copenhagen , Denmark.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/13554794.2018.1
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http://dx.doi.org/10.1080/13554794.2018.1512633DOI Listing
June 2018
17 Reads

Pathological correlates of white matter hyperintensities in a case of progranulin mutation associated frontotemporal dementia.

Neurocase 2018 Jun 16;24(3):166-174. Epub 2018 Aug 16.

a Dementia Research Centre, Department of Neurodegenerative Disease , UCL Institute of Neurology , London , UK.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/13554794.2018.1
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http://dx.doi.org/10.1080/13554794.2018.1506039DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6168954PMC
June 2018
17 Reads

Mitochondrial membrane protein-associated neurodegeneration: a case report and literature review.

Neurocase 2018 Jun 8;24(3):161-165. Epub 2018 Aug 8.

a Department of Neurology, Centre of Clinical Neurosciences, 1st Faculty of Medicine , Charles University and General University Hospital , Prague , Czech Republic.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1506038DOI Listing
June 2018
1 Read

Illustrating where spatial perception versus memory-based representation: spatial neglect in a distinguished artist; a case report.

Neurocase 2018 Jun 23;24(3):151-155. Epub 2018 Jul 23.

a Inpatient Rehabilitation Department , Kessler Institute for Rehabilitation , West Orange , NJ, USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1495741DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6162150PMC
June 2018
5 Reads

Bilateral upper limb rehabilitation with videogame-based feedback in corticobasal degeneration: a case reports study.

Neurocase 2018 Jun 17;24(3):156-160. Epub 2018 Jul 17.

b Clinical Laboratory of Experimental Neurorehabilitation , Santa Lucia Foundation, IRCCS , Rome , Italy.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1499938DOI Listing
June 2018
4 Reads

Early stage memory impairment, visual hallucinations, and myoclonus combined with temporal lobe atrophy predict Alzheimer's disease pathology in corticobasal syndrome.

Neurocase 2018 Jun 10;24(3):145-150. Epub 2018 Jul 10.

d Department of Neurology , Pusan National University Hospital, Pusan National University School of Medicine and Medical Research Institute , Busan , South Korea.

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). Read More

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http://dx.doi.org/10.1080/13554794.2018.1494290DOI Listing
June 2018
14 Reads

A new MAPT deletion in a case of speech apraxia leading to corticobasal syndrome.

Neurocase 2018 Jun 3;24(3):140-144. Epub 2018 Jul 3.

a Neurological Clinic, Department of Medical, Surgical and Health Sciences , University of Trieste , Trieste , Italy.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1492729DOI Listing
June 2018
8 Reads

A progressive breakdown of the body in space.

Neurocase 2018 Jun 8;24(3):133-139. Epub 2018 Jun 8.

a Neuropsychology and Language Disorders Unit, Department of Neurology , School of Medicine, National and Kapodistrian University of Athens , Greece.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1482356DOI Listing
June 2018
5 Reads

Clinical and imaging progression over 10 years in a patient with primary progressive apraxia of speech and autopsy-confirmed corticobasal degeneration.

Neurocase 2018 Apr 25;24(2):111-120. Epub 2018 May 25.

a Department of Radiology , Mayo Clinic , Rochester , MN , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1477963DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6095655PMC
April 2018
5 Reads

Connecting the dots: an association between opioids and acute hippocampal injury.

Neurocase 2018 Apr 18;24(2):124-131. Epub 2018 May 18.

b Department of Anesthesiology and Critical Care , University of Pennsylvania School of Medicine , Philadelphia , PA , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1475572DOI Listing
April 2018
4 Reads

Relief from intractable phantom pain by combining psilocybin and mirror visual-feedback (MVF).

Neurocase 2018 Apr 15;24(2):105-110. Epub 2018 May 15.

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". Read More

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http://dx.doi.org/10.1080/13554794.2018.1468469DOI Listing
April 2018
209 Reads

Interpersonal gargalesthesia.

Neurocase 2018 Apr 25;24(2):98-104. Epub 2018 Apr 25.

b Department of Psychology , University of California , San Diego , La Jolla, CA , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1464583DOI Listing
April 2018
4 Reads

Persistent anterograde amnesia due to the artery of Percheron occlusion: a case report.

Neurocase 2018 Apr 2;24(2):95-97. Epub 2018 Apr 2.

a Department of Neurology, Division of Cerebrovascular Disease, Cerrahpasa Medical Faculty , Istanbul University , Istanbul , Turkey.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/13554794.2018.1
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http://dx.doi.org/10.1080/13554794.2018.1458884DOI Listing
April 2018
5 Reads

Molecular neuroimaging in primary progressive aphasia with predominant agraphia.

Neurocase 2018 Apr 23;24(2):121-123. Epub 2018 Mar 23.

a Department of Neurology , Mayo Clinic , Rochester , MN , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1454963DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6101047PMC
April 2018
9 Reads

Erratum.

Authors:

Neurocase 2018 Feb;24(1)

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http://dx.doi.org/10.1080/13554794.2018.1446389DOI Listing
February 2018
3 Reads

A case of semantic variant primary progressive aphasia with Pick's pathology.

Neurocase 2018 Apr 6;24(2):90-94. Epub 2018 Mar 6.

a Memory and Aging Center , University of California San Francisco , San Francisco , CA , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1447134DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5990456PMC
April 2018
5 Reads

Pattern of cerebral hypoperfusion according to the clinical staging in dementia with Lewy bodies.

Neurocase 2018 Apr 6;24(2):83-89. Epub 2018 Mar 6.

b Institute of Convergence Bio-Health , Dong-A University , Busan , Korea.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/13554794.2018.1
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http://dx.doi.org/10.1080/13554794.2018.1447133DOI Listing
April 2018
9 Reads

Novel cognitive insights from the first year after bi-thalamic infarct.

Neurocase 2018 Feb 1;24(1):76-81. Epub 2018 Mar 1.

c Neuropsychology Research Unit, School of Psychology , The University of Queensland , Brisbane , Australia.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1444779DOI Listing
February 2018
5 Reads

Unilateral Apraxic Agraphia without Ideomotor Apraxia from a callosal lesion in a patient with Marchiafava-Bignami disease.

Neurocase 2018 Feb 26;24(1):59-67. Epub 2018 Feb 26.

c Neurology Service and GRECC , Malcom Randall Veterans Affairs Medical Center , Gainesville , FL , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1444780DOI Listing
February 2018
7 Reads

Heterozygous genotype at codon 129 correlates with prolonged disease course in Heidenhain variant sporadic CJD: case report.

Neurocase 2018 Feb 13;24(1):54-58. Epub 2018 Feb 13.

a Department of Neurology , Mayo Clinic , Rochester , MN , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1439067DOI Listing
February 2018
8 Reads

Retrieving autobiographical experience of painful events in a phantom limb: brain concomitants in a case report.

Neurocase 2018 Feb 1;24(1):41-48. Epub 2018 Feb 1.

a Neurology/Neuropsychology CMRR Unit , CHU Nord Saint-Etienne , Saint-Priest-en-Jarez , France.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1429636DOI Listing
February 2018
7 Reads

Massive weight loss following deep brain stimulation of the nucleus accumbens in a depressed woman.

Neurocase 2018 Feb 1;24(1):49-53. Epub 2018 Feb 1.

b Department of Psychiatry , University of Lübeck , Lübeck , Germany.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1431678DOI Listing
February 2018
5 Reads

Slowly progressive behavioral frontotemporal dementia with C9orf72 mutation. Case report and review of the literature.

Neurocase 2018 Feb 22;24(1):68-71. Epub 2018 Jan 22.

a Department of Neurology , Hospital Universitario 12 de Octubre , Madrid , Spain.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1428353DOI Listing
February 2018
6 Reads

The neural substrates of improved phonological processing following successful treatment in a case of phonological alexia and agraphia.

Neurocase 2018 Feb 19;24(1):31-40. Epub 2018 Jan 19.

a Department of Speech, Language, and Hearing Sciences , University of Arizona , Tucson , AZ , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1428352DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5843561PMC
February 2018
7 Reads

Semantic language deficit developing following herpes simplex encephalitis: reorganization "cannibalising" language centers?

Neurocase 2018 Feb 19;24(1):72-75. Epub 2018 Jan 19.

a Neuropsychiatry Unit , Royal Melbourne Hospital , Melbourne , Australia.

Herpes simplex virus encephalitis (HSVE) commonly presents with severe amnesia due to virus-mediated destruction of key regions in the temporal lobes, although language and executive impairment has been described. Little is known however of the long-term cognitive changes in these patients, including changes that may happen with cortical reorganization. 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. Read More

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http://dx.doi.org/10.1080/13554794.2018.1428351DOI Listing
February 2018
6 Reads

Neuronal activity organization features in human consciousness: summary of introductory remarks in a dialogue with neurological surgery residents.

Neurocase 2018 Feb 9;24(1):7-9. Epub 2018 Jan 9.

c Department of Neurological Surgery, Center for Neuromodulation , Ohio State University Wexner Medical Center , Columbus , OH , USA.

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 segment harboring atavic spinal organization. Read More

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http://dx.doi.org/10.1080/13554794.2017.1420200DOI Listing
February 2018
9 Reads

Posterior cortical atrophy: a single case cognitive and radiological follow-up.

Neurocase 2018 Feb 8;24(1):16-30. Epub 2018 Jan 8.

g National Scientific and Technical Research Council (CONICET) , Buenos Aires , Argentina.

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. Read More

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http://dx.doi.org/10.1080/13554794.2017.1421667DOI Listing
February 2018
6 Reads

Multiproteinopathy, neurodegeneration and old age: a case study.

Neurocase 2018 Feb 6;24(1):1-6. Epub 2018 Jan 6.

a Memory and Aging Center, Department of Neurology , University of California San Francisco , San Francisco, USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2017.1420199DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5877460PMC
February 2018
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Mixed language dominance: insights from a case of unexpected fluent aphasia with semantic jargon resulting from massive left perisylvian lesion.

Neurocase 2018 Feb 25;24(1):10-15. Epub 2017 Dec 25.

d Department of Psychology , Northern Michigan University , Marquette , MI , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2017.1420805DOI Listing
February 2018
8 Reads

Alterations of emotional reactivity following righttemporal lobectomy.

Neurocase 2017 Oct - Dec;23(5-6):314-320. Epub 2017 Dec 20.

a Departments of Clinical Health Psychology and the Center for Neuropsychological Studies , Gainesville , University of Florida , USA.

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. Read More

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http://dx.doi.org/10.1080/13554794.2017.1416148DOI Listing
December 2017
7 Reads

The painter from Sinaloa: artistic analysis of a case of spatial agnosia and neglect of visual shapes.

Neurocase 2017 Oct - Dec;23(5-6):304-313. Epub 2017 Nov 29.

d Independent researcher , Bogotá , Colombia.

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. Read More

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https://www.tandfonline.com/doi/full/10.1080/13554794.2017.1
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http://dx.doi.org/10.1080/13554794.2017.1408842DOI Listing
November 2017
14 Reads

Accelerated Long-Term Forgetting Limited to the Recollection Component of Recognition Memory in a Temporal Lobe Epileptic Patient.

Neurocase 2017 Oct - Dec;23(5-6):278-286. Epub 2017 Oct 26.

a Department of Systems Medicine , "Tor Vergata" University , Rome , Italy.

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. Read More

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http://dx.doi.org/10.1080/13554794.2017.1390141DOI Listing
October 2017
11 Reads

The use of cannabidiol for seizure management in patients with brain tumor-related epilepsy.

Neurocase 2017 Oct - Dec;23(5-6):287-291. Epub 2017 Oct 24.

a Department of Neurology , University of Alabama at Birmingham , Birmingham , AL , USA.

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; Greenwich Biosciences) 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. Read More

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http://dx.doi.org/10.1080/13554794.2017.1391294DOI Listing
October 2017
8 Reads

Reorganized neural activation in motor cortex following subdural fluid collection: an fMRI and DTI study.

Neurocase 2017 Oct - Dec;23(5-6):292-303. Epub 2017 Oct 24.

a Department of Surgery, Division of Neurosurgery , Saskatoon , SK , Canada.

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. Read More

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http://dx.doi.org/10.1080/13554794.2017.1395056DOI Listing
October 2017
13 Reads

Evaluating the roles of left middle frontal gyrus in word production using electrocorticography.

Neurocase 2017 Oct - Dec;23(5-6):263-269. Epub 2017 Oct 20.

a State Key Laboratory of Cognitive Neuroscience and Learning & IDG/McGovern Institute for Brain Research , Beijing Normal University , Beijing , China.

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 the hierarchical state feedback control model of word production. Read More

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http://dx.doi.org/10.1080/13554794.2017.1387275DOI Listing
October 2017
11 Reads