scholarly journals AGR - 2 Diagnosis of Posterior Cortical Atrophy Through Inter-Departmental Collaboration

2019 ◽  
Vol 34 (6) ◽  
pp. 831-831
Author(s):  
S John ◽  
M Silva ◽  
N Newman ◽  
D Loring
Keyword(s):  
Neuropsychological Testing ◽  
Cortical Atrophy ◽  
White Female ◽  
Posterior Cortical Atrophy ◽  
Visual Spatial ◽  
Field Evidence ◽  
Confrontation Naming ◽  
Unsteady Gait ◽  
History Of ◽  
The Right

Abstract Objective We present a patient with rapidly progressive visual decline of 2-year duration that interfered with daily functioning. She was evaluated by neuro-ophthalmology and neurology prior to neuropsychological referral. A series of evaluations led to diagnosis of posterior cortical atrophy, demonstrating the importance of inter-departmental collaboration. Method A 66-year old white female presented with a 2-year history of progressive changes to vision and memory. Medical history included hypertension, dyslipidemia, and a strong family history of optic neuropathy causing blindness. She was diagnosed with a left homonymous hemianopia. MRI revealed "significant cortical atrophy more remarkable on the right temporal, parietal, and occipital regions." She reported dressing apraxia, unsteady gait, declines in reading and writing, and difficulty recalling well-learned information. Results She was a good historian, had fluent speech and no apparent comprehension difficulty. Neuropsychological evaluation revealed relatively preserved language and verbal abilities, including confrontation naming, in the presence of otherwise impaired performances across all domains of functioning. She demonstrated agraphia, acalculia, left-right confusion, and difficulties with motor programming. Perceptual and constructional tasks revealed prominent deficits in visual integration, map orientation, form discrimination, and construction of simple geometric designs. She was perseverative and susceptible to verbal and visual stimulus pull. Conclusions The pattern on neuropsychological testing, with prominent visual spatial and perceptual difficulties, was consistent with posterior cortical atrophy. The decline in visual ability is likely exacerbated but not entirely explained by left hemianopia. Neuropsychological, neuroimaging, and visual field evidence demonstrated posterior cortical atrophy in the absence of positive biomarker evidence, leading to initiation of anti-cholinesterase therapy.

Z-line degradation and phagocytosis of sarcomeric fragments in myonecrosis

1983 ◽  
Vol 41 ◽  
pp. 790-791
Author(s):  
Melinda L. Estes ◽  
Samuel M. Chou
Keyword(s):  
Vastus Lateralis ◽  
Inflammatory Myopathy ◽  
Muscle Disease ◽  
White Female ◽  
Inflammatory Cell Infiltrate ◽  
Limb Weakness ◽  
Muscle Diseases ◽  
History Of ◽  
The Right ◽  
Mononuclear Inflammatory Cell

Many muscle diseases show common pathological features although their etiology is different. In primary muscle diseases a characteristic finding is myofiber necrosis. The mechanism of myonecrosis is unknown. Polymyositis is a primary muscle disease characterized by acute and subacute degeneration as well as regeneration of muscle fibers coupled with an inflammatory infiltrate. We present a case of polymyositis with unusual ultrastructural features indicative of the basic pathogenetic process involved in myonecrosis.The patient is a 63-year-old white female with a one history of proximal limb weakness, weight loss and fatigue. Examination revealed mild proximal weakness and diminished deep tendon reflexes. Her creatine kinase was 1800 mU/ml (normal < 140 mU/ml) and electromyography was consistent with an inflammatory myopathy which was verified by light microscopy on biopsy muscle. Ultrastructural study of necrotizing myofiber, from the right vastus lateralis, showed: (1) degradation of the Z-lines with preservation of the adjacent Abands including M-lines and H-bands, (Fig. 1), (2) fracture of the sarcomeres at the I-bands with disappearance of the Z-lines, (Fig. 2), (3) fragmented sarcomeres without I-bands, engulfed by invading phagocytes, (Fig. 3, a & b ), and (4) mononuclear inflammatory cell infiltrate in the endomysium.

scholarly journals Latent atrophy factors related to phenotypical variants of posterior cortical atrophy

Neurology ◽  
2020 ◽  
Vol 95 (12) ◽  
pp. e1672-e1685 ◽  
Author(s):  
Colin Groot ◽  
B.T. Thomas Yeo ◽  
Jacob W. Vogel ◽  
Xiuming Zhang ◽  
Nanbo Sun ◽  
...  
Keyword(s):  
Gray Matter ◽  
Visual Processing ◽  
Space Perception ◽  
Object Perception ◽  
Cortical Atrophy ◽  
Intracranial Volume ◽  
Posterior Cortical Atrophy ◽  
Modeling Framework ◽  
Β Amyloid ◽  
The Right

ObjectiveTo determine whether atrophy relates to phenotypical variants of posterior cortical atrophy (PCA) recently proposed in clinical criteria (i.e., dorsal, ventral, dominant-parietal, and caudal) we assessed associations between latent atrophy factors and cognition.MethodsWe employed a data-driven Bayesian modeling framework based on latent Dirichlet allocation to identify latent atrophy factors in a multicenter cohort of 119 individuals with PCA (age 64 ± 7 years, 38% male, Mini-Mental State Examination 21 ± 5, 71% β-amyloid positive, 29% β-amyloid status unknown). The model uses standardized gray matter density images as input (adjusted for age, sex, intracranial volume, MRI scanner field strength, and whole-brain gray matter volume) and provides voxelwise probabilistic maps for a predetermined number of atrophy factors, allowing every individual to express each factor to a degree without a priori classification. Individual factor expressions were correlated to 4 PCA-specific cognitive domains (object perception, space perception, nonvisual/parietal functions, and primary visual processing) using general linear models.ResultsThe model revealed 4 distinct yet partially overlapping atrophy factors: right-dorsal, right-ventral, left-ventral, and limbic. We found that object perception and primary visual processing were associated with atrophy that predominantly reflects the right-ventral factor. Furthermore, space perception was associated with atrophy that predominantly represents the right-dorsal and right-ventral factors. However, individual participant profiles revealed that the large majority expressed multiple atrophy factors and had mixed clinical profiles with impairments across multiple domains, rather than displaying a discrete clinical–radiologic phenotype.ConclusionOur results indicate that specific brain behavior networks are vulnerable in PCA, but most individuals display a constellation of affected brain regions and symptoms, indicating that classification into 4 mutually exclusive variants is unlikely to be clinically useful.

scholarly journals Toward an Alaskan Critical Regionalist Pedagogy

Lateral ◽  
2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Richard Simpson
Keyword(s):  
Critical Pedagogy ◽  
Urban Space ◽  
Decisive Role ◽  
Cruise Ship ◽  
Production Of Space ◽  
Productive Force ◽  
Spatial History ◽  
Visual Spatial ◽  
History Of ◽  
The Right

In an era when urban space is theorized as an educative science enhancing productivity, business, and management, we witness the emergence of teaching as a dominant productive force for the first time in the history of capital. Given the decisive role of knowledge production in the development of globalized urbanization it becomes vital to identify critical pedagogies that not only engage the production of space but grasp the production of space as pedagogical. To do so, I attend to interventions into regionalist studies and the global city to argue for visual spatial tactics as a tool for a critical regionalist pedagogy capable of linking material, affective, and discursive practices with a placed-based approach to globalized urbanization. Students design a collaborative website documenting the spatial history of cruise ship tourism in Alaska as an argument over the right to the city. Identifying this living process—framing the cruise industry as a constitutive system fusing discourse, space, and identity to restructure history, nature, and region—becomes a means of questioning and revising otherwise generalized theories often brought to bear on tourist landscapes, on Alaska, and on critical pedagogy itself. This case study shows the emergence of the cruise ship city as inseparable from the onset of globalized urbanization and how it, in turn, provides edifying material to mobilize a critical regionalist pedagogy within contemporary forms of educative landscapes.

scholarly journals C-55 Normal Brain Cells in Abnormal Places: Contributions of Conjoint Neuropsychological, Pediatric, Neurologic, and Psychiatric Assessment in the Diagnosis and Behavioral Management of Pediatric Subependymal Heterotopia

2019 ◽  
Vol 34 (6) ◽  
pp. 1084-1084
Author(s):  
A Lewandowski ◽  
S Brown ◽  
C Lewandowski ◽  
J Jacob ◽  
B Tross
Keyword(s):  
Neuropsychological Assessment ◽  
Neuropsychological Testing ◽  
Psychometric Testing ◽  
Emotional Dysregulation ◽  
Self Report ◽  
Normal Brain ◽  
Motor Speed ◽  
Visual Spatial ◽  
Medical Evaluation ◽  
The Right

Abstract Objective Subependymal heterotopia (SH) is a development brain malformation from abnormal neuronal proliferation and migration resulting in developmental delays and focal childhood epilepsy (Barkovich, 2000). SH results from the interruption of normal gray matter migration beneath the ependyma of the lateral ventricles. Most males are spontaneously aborted, and although there is some genetic uncertainty, affected females are thought to be X-linked (Xq28) recessive (Mitchell, et.al., 2000). SH prevalence in epileptic children with brain dysgenesis is between 13-20% and manifests in motor, visual-spatial, cognitive, and psychological deficits (Data, et.al., 2018). Method We present a case study of a seventeen-year-old female who was diagnosed at age eight with ADD and LD by self-report check-lists and achievement tests. Behavioral irregularities and seizure onset lead pediatrics to request neuropsychological testing. Findings revealed cognitive and emotional impairment and consequently further medical evaluation was recommended. Pediatric, neurological, and psychiatric examination, EEG, and MRI confirmed SH along the lateral margin of the right lateral ventricle. Results Psychometric testing revealed low average FSIQ, PRI, and PSI, impaired arithmetic operations and problem solving, graphomotor deficits, motor speed and executive/planning difficulties, emotional dysregulation, age-inappropriate interpersonal skills, and social-behavioral regression. Conclusions Neuropsychological assessment provided clear evidence of cognitive impairment beyond the initial diagnoses, suggested lateralized dysfunction, and implicated frontal and subcortical behavioral correlates not explained by other dispositional variables. Comprehensive neuropsychological assessment provided detailed information toward conjoint treatment planning by pediatrics, neurology, psychology, psychiatry, and teachers. Findings provided meaningful diagnostic clarification of SH and furthered clinical understanding of this adolescent’s social, academic, neuropsychological, and psychiatric needs.

scholarly journals Brain structural and functional anomalies associated with simultanagnosia in patients with posterior cortical atrophy

2021 ◽  
Author(s):  
Yue Cui ◽  
Yang Liu ◽  
Caishui Yang ◽  
Chunlei Cui ◽  
Donglai Jing ◽  
...  
Keyword(s):  
Functional Connectivity ◽  
Gray Matter ◽  
Gray Matter Volume ◽  
Cortical Atrophy ◽  
Posterior Cortical Atrophy ◽  
Matter Volume ◽  
Intrinsic Functional Connectivity ◽  
Middle Occipital Gyrus ◽  
The Right ◽  
Left Middle

AbstractSimultanagnosia is a common symptom of posterior cortical atrophy, and its association with brain structural and functional changes remains unclear. In our study, 18 posterior cortical atrophy patients with simultanagnosia, 29 patients with Alzheimer’s disease and 20 cognitively normal controls were recruited and subjected to full neuropsychological evaluation, including simultanagnosia tests, and structural and resting-state functional MRI. The gray matter volume was assessed by voxel-based morphometry, while the intrinsic functional connectivity was evaluated using the reduced gray matter volume regions of interest as the seed. In contrast to the patients with Alzheimer’s disease, those with posterior cortical atrophy showed the following: (1) markedly lower simultanagnosia test scores, (2) an altered regional gray matter volume of the left middle occipital gyrus and ventral occipital areas, and (3) lowered intrinsic functional connectivity with the left middle occipital gyrus, left lingual gyrus and right middle occipital gyrus separately. Additionally, the gray matter volume of the left middle occipital gyrus and left inferior occipital gyrus were each correlated with simultanagnosia in posterior cortical atrophy patients. The intrinsic functional connectivity of the left middle occipital gyrus with the right superior occipital gyrus and that of the right middle occipital gyrus with the left superior parietal gyrus were also correlated with simultanagnosia in posterior cortical atrophy patients. In summary, this study indicated that simultanagnosia is associated with gray matter reductions and decreased functional connectivity in the left middle occipital gyrus and the left inferior occipital gyrus in patients with posterior cortical atrophy.

scholarly journals Posterior Cortical Atrophy with Right Lower Egocentric Quadrantic Neglect and Lower Vertical Allocentric Neglect

2020 ◽  
Vol 35 (4) ◽  
pp. 448-457
Author(s):  
Usama Tariq ◽  
Alicia Parker ◽  
Leila Saadatpour ◽  
Leilani Doty ◽  
Kenneth M Heilman
Keyword(s):  
Parietal Lobe ◽  
Visual Space ◽  
Vertical Line ◽  
Cortical Atrophy ◽  
Horizontal Line ◽  
Posterior Cortical Atrophy ◽  
Lower Quadrant ◽  
Visuospatial Neglect ◽  
Parietal Lobes ◽  
The Right

Abstract Background/Objectives Whereas rare cases of hemispatial visual neglect have been reported in patients with a neurodegenerative disease, quadrantic visuospatial neglect has not been described. We report a patient with probable posterior cortical atrophy who demonstrated lower right-sided quadrantic visuospatial neglect, together with allocentric vertical neglect. Methods/Results A 68-year-old man initially noted deficits in reading and writing. Subsequently, he developed other cognitive deficits. On vertical line bisections, he deviated upward, and on horizontal line bisections, he deviated to the left. These deviations together suggest that this man’s neglect might be most severe in his right (head/body-centered) lower (below eye level) visual space. When attempting to perform vertical line bisections in all four egocentric quadrants, his upward deviations were largest in the right lower quadrant. On a cancelation test, he revealed bilateral lower (ventral) allocentric neglect but not egocentric neglect. This patient’s magnetic resonance imaging revealed cortical atrophy, most prominent in the left parietal lobe. Discussion Previous research in stroke patients has demonstrated that the parietal lobes are important in mediating attention to contralateral and inferior visual space. The presence of left parietal atrophy may have induced this right lower (ventral) egocentric inattention as well as bilateral ventral allocentric inattention. Although to our knowledge there have been no prior reports of a patient with right lower quadrantic and lower vertical allocentric visuospatial neglect, patients are rarely tested for these forms of neglect, and this patient illustrates the importance of evaluating patients for these and other forms of neglect.

scholarly journals F2-01-03: “AM I THE RIGHT WAY UP?”: INVESTIGATING BALANCE PROBLEMS IN POSTERIOR CORTICAL ATROPHY

2019 ◽  
Vol 15 ◽  
pp. P516-P516
Author(s):  
Keir Yong ◽  
Amy Peters ◽  
Dilek Ocal ◽  
David M. Cash ◽  
Matthew Bancroft ◽  
...  
Keyword(s):  
Cortical Atrophy ◽  
Posterior Cortical Atrophy ◽  
The Right ◽  
Balance Problems

scholarly journals Survey outcomes on neuropsychological testing in posterior cortical atrophy

2021 ◽  
Vol 17 (S5) ◽  
Author(s):  
Victoria S. Pelak ◽  
Samantha K. Holden ◽  
Sebastian J. Crutch ◽  
Keir X.X. Yong
Keyword(s):  
Neuropsychological Testing ◽  
Cortical Atrophy ◽  
Posterior Cortical Atrophy

THUR 101 SCA-1 with unusual early dysautonomia and delayed cerebellar signs

2018 ◽  
Vol 89 (10) ◽  
pp. A10.3-A10
Author(s):  
d’Ersu Eleanor ◽  
Anwar NurAizaan ◽  
Al-Mayhani Talal ◽  
Sidhu Meneka
Keyword(s):  
Autonomic Dysfunction ◽  
Cerebellar Atrophy ◽  
Intention Tremor ◽  
Initial Examination ◽  
Autonomic Changes ◽  
Unsteady Gait ◽  
History Of ◽  
The Right

A 59-year-old right-handed bus driver presented with gradually worsening erectile dysfunction, urinary frequency and nocturia over 18 months. He had a past medical history of hypertension with regular medications including amlodipine, aspirin and atorvastatin. Initial examination revealed a mild bilateral intention tremor.The patient had undergone brain imaging showing brainstem and cerebellar atrophy with a ‘hot cross bun sign’. The patient was then referred for autonomic function tests that demonstrated mild cardiovascular autonomic changes, and suggested sympathetic dysfunction. A working diagnosis of multiple system atrophy (MSA) was made.Over the next two years, his tremor worsened with the development of slurred speech and unsteady gait. On examination, he had prominent fasciculations and wasting in the right shoulder, severe bilateral intention tremor with past-pointing. In addition he had Parkinsonism and signs of a sensorimotor neuropathy.This atypical clinical evolution with prominent neuropathy and cerebellar signs led to investigations for spinocerebellar ataxia (SCA), particularly type 2 or 3 (given the autonomic dysfunction). However, the genetic testing showed a mutation in ATXN1, confirming a diagnosis of SCA type 1.To our knowledge, this is the first reported case of prominent early autonomic dysfunction associated with SCA type 1.

scholarly journals Data-driven detection of latent atrophy factors related to phenotypical variants of posterior cortical atrophy

2019 ◽  
Author(s):  
Colin Groot ◽  
B.T. Thomas Yeo ◽  
Jacob W Vogel ◽  
Xiuming Zhang ◽  
Nanbo Sun ◽  
...  
Keyword(s):  
Gray Matter ◽  
Visual Processing ◽  
Amyloid Β ◽  
Space Perception ◽  
Object Perception ◽  
Data Driven ◽  
Cortical Atrophy ◽  
Intracranial Volume ◽  
Posterior Cortical Atrophy ◽  
The Right

AbstractPosterior cortical atrophy is a clinical-radiological syndrome characterized by visual processing deficits and atrophy in posterior parts of the brain, most often caused by Alzheimer’s disease pathology. Recent consensus criteria describe four distinct phenotypical variants of posterior cortical atrophy defined by clinical and radiological features; i) object perception/occipitotemporal (ventral), ii) space perception/temporoparietal (dorsal), iii) non-visual/dominant parietal and iv) primary visual (caudal). We employed a data-driven approach to identify atrophy factors related to these proposed variants in a multi-center cohort of 119 individuals with posterior cortical atrophy (age: 64 SD 7, 38% male, MMSE: 21 SD 5, 71% amyloid-β positive, 29% amyloid-β status unknown). A Bayesian modelling framework based on latent Dirichlet allocation was used to compute four latent atrophy factors in accordance with the four proposed variants. The model uses standardized gray matter density images as input (adjusted for age, sex, intracranial volume, field strength and whole-brain gray matter volume) and provides voxelwise probabilistic maps for all atrophy factors, allowing every individual to express each factor to a degree without a priori classification. The model revealed four distinct yet partially overlapping atrophy factors; right-dorsal, right-ventral, left-ventral, and limbic. Individual participant profiles revealed that the vast majority of participants expressed multiple factors, rather than predominantly expressing a single factor. To assess the relationship between atrophy factors and cognition, neuropsychological test scores covering four posterior cortical atrophy-specific cognitive domains were assessed (object perception, space perception, non-visual parietal functions and primary visual processing) and we used general linear models to examine the association between atrophy factor expression and cognition. We found that object perception and primary visual processing were associated with atrophy that predominantly reflects the right-ventral factor. Furthermore, space perception was associated with atrophy that predominantly represents the right-ventral and right-dorsal factors. Similar to the atrophy factors, most participants had mixed clinical profiles with impairments across multiple domains. However, when selecting four participants with an isolated impairment, we observed atrophy patterns and factor expressions that were largely in accordance with the hypothesized variants. Taken together, our results indicate that variants of posterior cortical atrophy exist but these constitute phenotypical extremes and most individuals fall along a broad clinical-radiological spectrum, indicating that classification into four mutually exclusive variants is unlikely to be clinically useful.

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