28 research outputs found
Variably protease-sensitive prionopathy mimicking frontotemporal dementia
No full textSporadic prion diseases are fatal neurodegenerative disorders characterized clinically by rapidly progressive dementia and myoclonus. Variably protease-sensitive prionopathy (VPSPr) is a recently identified sporadic human prion disorder that may present with a lengthy atypical clinical history. Here, we describe a case of VPSPr in a patient with a long history of suspected frontotemporal dementia (FTD). A 61-year-old man presented with speech difficulties, including naming objects and constructing multipart sentences, while there was no difficulty in comprehension. Movement abnormalities included slightly jerky pursuit, minor dysmetria of saccades and brisk reflexes. There was no family history of dementia. Later he developed swallowing difficulties and the possibility of FTD with motor neuron disease was suspected. He died at the age of 71 and his brain was donated to the London Neurodegenerative Diseases Brain Bank. The brain (1004 g) showed mild to moderate atrophy, predominantly in the frontal lobe. Histology revealed moderate spongiform microvacuolation mostly affecting the frontal and parietal cortices, but also present focally in the basal ganglia and the cerebellum. Only mild Alzheimer pathology was found by extensive immunohistochemistry, in keeping with BrainNet Europe stage II. Trans-activation response DNA-binding protein 43 kDa and α-synuclein immunostains were negative. Immunostaining for prion protein (PrP) showed granular/synaptic positivity in a patchy distribution, mainly within the deeper cortex, and also revealed microplaques in the cerebellum and basal ganglia. Western blotting confirmed a low molecular weight protease-resistant PrP band with a faint ladder-like pattern in the absence of types 1 and 2 isoforms. These features are diagnostic of VPSPr. VPSPr can mimic various neurodegenerative conditions; diagnosis requires both PrP immunohistochemistry and Western blotting. The presence of patchy spongiform change in the absence of other neurodegenerative pathology should raise suspicion of VPSPr, even in elderly patients with a lengthy clinical history.</p
The Impact of Race and Socioeconomic Status on Access to Accommodations in Postsecondary Education
No full textIn this paper, the author argues that even though individuals who grew up in poverty may experience more severe impairment associated with the behavioral characteristics of autism, they are less likely to receive a psychiatric diagnosis. Therefore, when colleges and require students with mental impairments to present evidence of a psychiatric diagnosis as a pre-requisite for access to accommodations, students who grew up in poverty are disproportionately prevented from accessing accommodations, which are required for them to compete on equal footing with their non-disabled peers. This in turn perpetuates a lack of educational attainment among individuals who grew up in poverty (disproportionately individuals from minority groups), leading to lower adult productivity and earning among these individuals. The paper examines federal policies and legislation underlying the provision of educational opportunities to all students (including students with disabilities).Journal of Gender, Social Policy & the La
A novel seven-octapeptide repeat insertion in the prion protein gene (PRNP) in a Dutch pedigree with Gerstmann-Sträussler-Scheinker disease phenotype: comparison with similar cases from the literature.
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97585.pdf (Publisher’s version ) (Open Access)Human prion diseases can be sporadic, inherited or acquired by infection and show considerable phenotypic heterogeneity. We describe the clinical, histopathological and pathological prion protein (PrP(Sc)) characteristics of a Dutch family with a novel 7-octapeptide repeat insertion (7-OPRI) in PRNP, the gene encoding the prion protein (PrP). Clinical features were available in four, neuropathological features in three and biochemical characteristics in two members of this family. The clinical phenotype was characterized by slowly progressive cognitive decline, personality change, lethargy, depression with anxiety and panic attacks, apraxia and a hypokinetic-rigid syndrome. Neuropathological findings consisted of numerous multi- and unicentric amyloid plaques throughout the cerebrum and cerebellum with varying degrees of spongiform degeneration. Genetic and molecular studies were performed in two male family members. One of them was homozygous for valine and the other heterozygous for methionine and valine at codon 129 of PRNP. Sequence analysis identified a novel 168 bp insertion [R2-R2-R2-R2-R3g-R2-R2] in the octapeptide repeat region of PRNP. Both patients carried the mutation on the allele with valine at codon 129. Western blot analysis showed type 1 PrP(Sc) in both patients and detected a smaller ~8 kDa PrP(Sc) fragment in the cerebellum in one patient. The features of this Dutch kindred define an unusual neuropathological phenotype and a novel PRNP haplotype among the previously documented 7-OPRI mutations, further expanding the spectrum of genotype-phenotype correlations in inherited prion diseases
Bringing Sanctuary to School: Assessing School Climate as a Foundation for Culturally Responsive Trauma-Informed Approaches for Urban Schools
No full text© The Author(s) 2016. Decades of federal economic policies that have concentrated poverty into isolated communities have devastated urban education, and expose youth and families to high stress and trauma. Disproportionately negative outcomes for students of color and those who are economically disadvantaged can be understood as manifestations of negative racial school climate and inadequate responsiveness to students’ trauma. As part of a school–university partnership to inform culturally responsive trauma-informed pedagogy, this study assessed the climate of a racially diverse high-poverty elementary school. Findings explored the application of the trauma-informed Sanctuary Model to address students’ trauma and a social justice response for urban education
Variably protease-sensitive prionopathy mimicking frontotemporal dementia
No full textSporadic prion diseases are fatal neurodegenerative disorders characterized clinically by rapidly progressive dementia and myoclonus. Variably protease‐sensitive prionopathy (VPSPr) is a recently identified sporadic human prion disorder that may present with a lengthy atypical clinical history. Here, we describe a case of VPSPr in a patient with a long history of suspected frontotemporal dementia (FTD). A 61‐year‐old man presented with speech difficulties, including naming objects and constructing multipart sentences, while there was no difficulty in comprehension. Movement abnormalities included slightly jerky pursuit, minor dysmetria of saccades and brisk reflexes. There was no family history of dementia. Later he developed swallowing difficulties and the possibility of FTD with motor neuron disease was suspected. He died at the age of 71 and his brain was donated to the London Neurodegenerative Diseases Brain Bank. The brain (1004 g) showed mild to moderate atrophy, predominantly in the frontal lobe. Histology revealed moderate spongiform microvacuolation mostly affecting the frontal and parietal cortices, but also present focally in the basal ganglia and the cerebellum. Only mild Alzheimer pathology was found by extensive immunohistochemistry, in keeping with BrainNet Europe stage II. Trans‐activation response DNA‐binding protein 43 kDa and α‐synuclein immunostains were negative. Immunostaining for prion protein (PrP) showed granular/synaptic positivity in a patchy distribution, mainly within the deeper cortex, and also revealed microplaques in the cerebellum and basal ganglia. Western blotting confirmed a low molecular weight protease‐resistant PrP band with a faint ladder‐like pattern in the absence of types 1 and 2 isoforms. These features are diagnostic of VPSPr. VPSPr can mimic various neurodegenerative conditions; diagnosis requires both PrP immunohistochemistry and Western blotting. The presence of patchy spongiform change in the absence of other neurodegenerative pathology should raise suspicion of VPSPr, even in elderly patients with a lengthy clinical history
Valdosta Project Change, Scrapbook, August 1999
No full textValdosta Project Change. “Valdosta Project Change Scrapbook, August 1999 Lowndes County Historical Society. Valdosta State University Archives and Special Collections, MS-181: Valdosta Project Change Scrapbook Collection, 1997-2003. 1 PDF document and scans, 17 pages. 633 MB (664,576,706 bytes).8/2- Minorities not shut out in Atlanta- Bill Shipp; 8/8- Conservative group fights affirmative action: Critics say foundation wants to reverse gains made by blacks- Associated Press; 8/11- Police end attempt to block rally- Associated Press; 8/11- Court allows minority groups to join affirmative action case- Associated Press; 8/12- Retired educator elected to national post- Staff reports; 8/14- Defining racism- Abigail Van Buren; 8/14- Park files suit over use of race- Associated Press; 8/20- Coming together: Race forum finds common ground- Brian Lawson; 8/21- Cops emphasize contact with citizens in new community policing program- Jodi M. Scott; 8/25- Finding the Means for the End- Rebecca Yull; 8/25- ‘Racist’ act wasn’t racist at all- Kathleen Caldwell, Valdosta; 8/27- Foundation sues Atlanta affirmative action program- Associated Press; 8/27- The Perils of Crying ‘Hate Crime’: Two attacks might appear racially inspired, but was booze the real trigger? - Carl Quintanilla and Kevin Helliker; 8/29- One area where it takes the village- no author; 8/29- Elected officials must show they care- Marjorie G. White, Valdosta; 8/31- Valdosta man reflects on local race relations- Leigh Amiot
Valdosta Project Change, Scrapbook, November 1999
No full textValdosta Project Change. “Valdosta Project Change Scrapbook, November 1999,” Lowndes County Historical Society. Valdosta State University Archives and Special Collections, MS-181: Valdosta Project Change Scrapbook Collection, 1997-2003. 1 PDF document and scans, 18 pages. 498 MB (522,659,571 bytes).11/7- When do we finally move beyond color? - Juana Jordan; 11/11- Hiding from race- Bill Maxwell; 11/11- Students from 60 countries attend high school where no one is a minority- Dan Hulbert; 11/12- City’s racial divisions exposed- no author; 11/12- Commander: Zero tolerance to racial incidents- Senior Airman Nickol Houston; 11/14- President lagging in race report- Associated Press; 11/14- Third race forum digging into key topics- staff reports; 11/15- Diversity needs rethinking- Nat Hentoff; 11/17- A tall task for Jesse Jackson- Bonnie Erbe; 11/18- Third race issues forum planned for tonight- staff reports; 11/18- Let racism die- Matt Flumerfelt, Valdosta; 11/19- Closing in on racism: Affirmative Action, stereotypes addressed in third race forum- Brian Lawson; 11/21- Black business see decline in South Africa: President blames fall on racism- Associated Press; 11/23- Weed and See reports progress- staff reports; 11/24- Cohen: Military must do more to end racism- Associated Press ; 11/24- Citizens talk, progress at forum- Rebecca Yull; 11/30- City schools in limbo: BOE wants explanation as consolidation talk spreads- Weenam Chua
RECLAIMING POWER. ACTIVATING BLACK MIDDLE-CLASS MOTHER ENGAGEMENT IN THE COMMITTEE ON SPECIAL EDUCATION
No full textFrom conversations in school hallways, to SEPTA meetings, to parking lots and church benches, Black middle-class parents have engaged in talk of disillusionment with the IEP development process for their special needs child. Previous literature has provided limited insight into the navigational capital of Black middle-class parents in the special education system. This author interviewed Black middle-class parents who felt overwhelmed by the IEP development process and came to utilize their cultural capital (Yosso, 2005) to overcome systemic barriers to their active engagement in the CSE (Ladson-Billings, 1995) and to explore opportunities they have had for positive interactions. Findings of this research show that limited navigational capital was the greatest hindrance to Black middle-class parent engagement in the CSE. This study illuminates school culture’s grasp of the deficit theory model, which lends itself to ill approached relationships with Black parents that hinders positive, active parent engagement crucial for child development and growth (Yull et al., 2014). Parents and the larger school community benefit from cultural reciprocity as it develops the navigational capital parents require for effective CSE engagement
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Heterogeneous surface expression of EspA translocon filaments by Escherichia coli O157:H7 is controlled at the posttranscriptional level
No full textType III secretion systems of enteric bacteria enable translocation of effector proteins into host cells. Secreted proteins of verotoxigenic Escherichia coli O157 strains include components of a translocation apparatus, EspA, -B, and -D, as well as “effectors” such as the translocated intimin receptor (Tir) and the mitochondrion-associated protein (Map). This research has investigated the regulation of LEE4 translocon proteins, in particular EspA. EspA filaments could not be detected on the bacterial cell surface when E. coli O157:H7 was cultured in M9 minimal medium but were expressed from only a proportion of the bacterial population when cultured in minimal essential medium modified with 25 mM HEPES. The highest proportions of EspA-filamented bacteria were detected in late exponential phase, after which filaments were lost rapidly from the bacterial cell surface. Our previous research had shown that human and bovine E. coli O157:H7 strains exhibit marked differences in EspD secretion levels. Here it is demonstrated that the proportion of the bacterial population expressing EspA filaments was associated with the level of EspD secretion. The ability of individual bacteria to express EspA filaments was not controlled at the level of LEE1-4 operon transcription, as demonstrated by using both β-galactosidase and green fluorescent protein (GFP) promoter fusions. All bacteria, whether expressing EspA filaments or not, showed equivalent levels of GFP expression when LEE1-4 translational fusions were used. Despite this, the LEE4-espADB mRNA was more abundant from populations with a high proportion of nonsecreting bacteria (low secretors) than from populations with a high proportion of secreting and therefore filamented bacteria (high secretors). This research demonstrates that while specific environmental conditions are required to induce LEE1-4 expression, a further checkpoint exists before EspA filaments are produced on the bacterial surface and secretion of effector proteins occurs. This checkpoint in E. coli O157:H7 translocon expression is controlled by a posttranscriptional mechanism acting on LEE4-espADB mRNA. The heterogeneity in EspA filamentation could arise from phase-variable expression of regulators that control this posttranscriptional mechanism
Metabolic syndrome and the immunological affair with the blood-brain barrier
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