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Expanding the spectrum of neonate subpial hemorrhage: More common than you think [Meeting Abstract]

Rispoli, J; Prabhu, S
Purpose: Subpial hemorrhage is an underrecognized imaging finding in neonates and is often mischaracterized as subarachnoid hemorrhage. Little is known regarding risk factors for developing subpial hemorrhage or the clinical outcomes of patients with subpial hemorrhage beyond studies involving handful of patients. Our aim is to better investigate the related antecedent etiologies of this entity and clinical outcomes for these patients using a larger cohort of patients at a tertiary care institution.
Material(s) and Method(s): A retrospective chart review was performed identifying cases from 2010-2020 with the words "subpial hemorrhage" in the final radiology report. Images were reviewed confirming subpial hemorrhage and clinical chart review for gestational age, delivery mechanism, coagulation profile, and neurologic symptoms at clinical follow-up was performed. Inclusion criteria included large subpial hemorrhage (defined as >1 sulcus) and imaging within the first month of life.
Result(s): Upon review, 178 cases of subpial hemorrhage were identified in our database and 68 cases met criteria for inclusion. The average gestational age for patients with the diagnosis of subpial hemorrhage was 39.5 weeks, with the majority of patients being full term. The most common location for subpial hemorrhage was the temporal lobe (32/68) with up to 24% of patients having multi-focal subpial hemorrhage. Associated radiographic findings included infarct (59%), medullary vein thrombosis (27%) and parenchymal hemorrhage (45%).Associations with previously identified possible risk factors in our population were trauma (14%), coagulopathy (14%), and history of congenital cardiac disease (10%). Of the patients, 60 patients had follow-up with 41% having neurologic symptoms at follow-up ranging from hypotonia to hemiparesis.
Conclusion(s): This study involving the largest series of patients with subpial hemorrhage to date indicates that this is not an uncommon entity in the neonatal population. Most common etiologies include trauma and coagulopathy, but rates of coagulopathy in our populationwere lower than previously reported. Long term neurologic sequelae are variable and further investigation should be focused on correlating size and location of subpial hemorrhage to clinical outcomes
EMBASE:636152464
ISSN: 1432-1998
CID: 5024992

Multisystem inflammatory syndrome in children (MIS-C) and retropharyngeal edema: A case series

Daube, Ariel; Rickert, Scott; Madan, Rebecca Pellett; Kahn, Philip; Rispoli, Joanne; Dapul, Heda
Multisystem inflammatory syndrome in children (MIS-C) is thought to follow SARS-CoV-2 infection and presents with fever and multisystem dysfunction. We report three children with suspected MIS-C found to have retropharyngeal edema without evidence of a bacterial etiology. We raise the possibility that an association between MIS-C and retropharyngeal edema exists.
PMCID:7931672
PMID: 33752089
ISSN: 1872-8464
CID: 4822422

Assessing the impact of an orientation week on acclimation to radiology residency

Prabhu, Vinay; Rispoli, Joanne M; Chhor, Chloe M; Mercado, Cecilia L; Fefferman, Nancy R
PURPOSE/OBJECTIVE:Acclimating residents to radiology residency requires attention to new responsibilities, educational material, and social cohesion. To this end, we instituted a structured orientation week for incoming residents and assessed its impact. PROCEDURES/METHODS:During the first weeks of July 2016 and 2017, first year residents attended a five day orientation free of clinical duties, consisting of didactics, hands-on training sessions, and social events. After two orientation cohorts, residents who completed orientation week, and two cohorts who had not, were given a voluntary, anonymous survey using Likert scale questions (1 [worst] to 5 [best]) regarding preparedness for responsibilities, learning, and social cohesion. Residents were asked which components were or would have been helpful. Independent samples t-tests were performed to evaluate differences between the two groups (two-tailed p < 0.05). FINDINGS/RESULTS:21/37 (57%) residents participated. Higher percentages of residents who participated in the orientation week gave scores ≥4 when asked about preparedness for rotations (70% vs. 36%), learning new material (80% vs. 36%), and class cohesiveness (90% vs. 70%). Mean scores on these questions were also higher for these residents with regards to: preparedness for new responsibilities (3.7 vs. 2.9), learning new material (3.8 vs. 2.9), and class cohesiveness (4.5 vs. 3.8), with differences approaching significance (p = 0.09-0.15). Individual components receiving most votes of ≥4 were social outings, resident lunches, didactic lectures, and PACS training. CONCLUSION/CONCLUSIONS:A weeklong orientation program free of clinical duties was valued by residents and contributed to acclimation to new responsibilities, education, and social cohesion.
PMID: 32387799
ISSN: 1873-4499
CID: 4430812

Clinical and Diffusion Tensor MRI Findings in Congenital Homonymous Hemianopia

Rispoli, Joanne; Seay, Meagan; Sum, Melissa; Rucker, Janet C; Shepherd, Timothy M
PMID: 30865055
ISSN: 1536-5166
CID: 3733222

The Use of an Emergency Department Expeditor to Improve Emergency Department CT Workflow: Initial Experiences

Gyftopoulos, Soterios; Jamin, Catherine; Wu, Tina S; Rispoli, Joanne; Fixsen, Eric; Rybak, Leon; Recht, Michael P
PMID: 30600159
ISSN: 1558-349x
CID: 3563382

Clinical utility for diffusion MRI sequence in emergency and inpatient spine protocols

Hoch, Michael J; Rispoli, Joanne; Bruno, Mary; Wauchope, Mervin; Lui, Yvonne W; Shepherd, Timothy M
Diffusion imaging of the spine has the potential to change clinical management, but is challenging due to the small size of the cord and susceptibility artifacts from adjacent structures. Reduced field-of-view (rFOV) diffusion can improve image quality by decreasing the echo train length. Over the past 2 years, we have acquired a rFOV diffusion sequence for MRI spine protocols on most inpatients and emergency room patients. We provide selected imaging diagnoses to illustrate the utility of including diffusion spine MRI in clinical practice. Our experiences support using diffusion MRI to improve diagnostic certainty and facilitate prompt treatment or clinical management.
PMID: 28601735
ISSN: 1873-4499
CID: 2594972

Mutant IDH1 and thrombosis in gliomas

Unruh, Dusten; Schwarze, Steven R; Khoury, Laith; Thomas, Cheddhi; Wu, Meijing; Chen, Li; Chen, Rui; Liu, Yinxing; Schwartz, Margaret A; Amidei, Christina; Kumthekar, Priya; Benjamin, Carolina G; Song, Kristine; Dawson, Caleb; Rispoli, Joanne M; Fatterpekar, Girish; Golfinos, John G; Kondziolka, Douglas; Karajannis, Matthias; Pacione, Donato; Zagzag, David; McIntyre, Thomas; Snuderl, Matija; Horbinski, Craig
Mutant isocitrate dehydrogenase 1 (IDH1) is common in gliomas, and produces D-2-hydroxyglutarate (D-2-HG). The full effects of IDH1 mutations on glioma biology and tumor microenvironment are unknown. We analyzed a discovery cohort of 169 World Health Organization (WHO) grade II-IV gliomas, followed by a validation cohort of 148 cases, for IDH1 mutations, intratumoral microthrombi, and venous thromboemboli (VTE). 430 gliomas from The Cancer Genome Atlas were analyzed for mRNAs associated with coagulation, and 95 gliomas in a tissue microarray were assessed for tissue factor (TF) protein. In vitro and in vivo assays evaluated platelet aggregation and clotting time in the presence of mutant IDH1 or D-2-HG. VTE occurred in 26-30 % of patients with wild-type IDH1 gliomas, but not in patients with mutant IDH1 gliomas (0 %). IDH1 mutation status was the most powerful predictive marker for VTE, independent of variables such as GBM diagnosis and prolonged hospital stay. Microthrombi were far less common within mutant IDH1 gliomas regardless of WHO grade (85-90 % in wild-type versus 2-6 % in mutant), and were an independent predictor of IDH1 wild-type status. Among all 35 coagulation-associated genes, F3 mRNA, encoding TF, showed the strongest inverse relationship with IDH1 mutations. Mutant IDH1 gliomas had F3 gene promoter hypermethylation, with lower TF protein expression. D-2-HG rapidly inhibited platelet aggregation and blood clotting via a novel calcium-dependent, methylation-independent mechanism. Mutant IDH1 glioma engraftment in mice significantly prolonged bleeding time. Our data suggest that mutant IDH1 has potent antithrombotic activity within gliomas and throughout the peripheral circulation. These findings have implications for the pathologic evaluation of gliomas, the effect of altered isocitrate metabolism on tumor microenvironment, and risk assessment of glioma patients for VTE.
PMCID:5640980
PMID: 27664011
ISSN: 1432-0533
CID: 2374852

Coordinate regulation of mature dopaminergic axon morphology by macroautophagy and the PTEN signaling pathway

Inoue, Keiichi; Rispoli, Joanne; Yang, Lichuan; Macleod, David; Beal, M Flint; Klann, Eric; Abeliovich, Asa
Macroautophagy is a conserved mechanism for the bulk degradation of proteins and organelles. Pathological studies have implicated defective macroautophagy in neurodegeneration, but physiological functions of macroautophagy in adult neurons remain unclear. Here we show that Atg7, an essential macroautophagy component, regulates dopaminergic axon terminal morphology. Mature Atg7-deficient midbrain dopamine (DA) neurons harbored selectively enlarged axonal terminals. This contrasted with the phenotype of DA neurons deficient in Pten - a key negative regulator of the mTOR kinase signaling pathway and neuron size - that displayed enlarged soma but unaltered axon terminals. Surprisingly, concomitant deficiency of both Atg7 and Pten led to a dramatic enhancement of axon terminal enlargement relative to Atg7 deletion alone. Similar genetic interactions between Atg7 and Pten were observed in the context of DA turnover and DA-dependent locomotor behaviors. These data suggest a model for morphological regulation of mature dopaminergic axon terminals whereby the impact of mTOR pathway is suppressed by macroautophagy.
PMCID:3789823
PMID: 24098148
ISSN: 1553-7390
CID: 1055742

Macroautophagy deficiency mediates age-dependent neurodegeneration through a phospho-tau pathway

Inoue, Keiichi; Rispoli, Joanne; Kaphzan, Hanoch; Klann, Eric; Chen, Emily I; Kim, Jongpil; Komatsu, Masaaki; Abeliovich, Asa
BACKGROUND: Macroautophagy is an evolutionarily conserved mechanism for bulk intracellular degradation of proteins and organelles. Pathological studies have implicated macroautophagy defects in human neurodegenerative disorders of aging including Alzheimer's disease and tauopathies. Neuronal deficiency of macroautophagy throughout mouse embryonic development results in neurodevelopmental defects and early postnatal mortality. However, the role of macroautophagy in mature CNS neurons, and the relationship with human disease neuropathology, remains unclear. Here we describe mice deficient in an essential macroautophagy component, Atg7, specifically within postnatal CNS neurons. RESULTS: Postnatal forebrain-specific Atg7 conditional knockout (cKO) mice displayed age-dependent neurodegeneration and ubiquitin- and p62-positive inclusions. Phosphorylated tau was significantly accumulated in Atg7 cKO brains, but neurofibrillary tangles that typify end-stage human tauopathy were not apparent. A major tau kinase, glycogen synthase kinase 3beta (GSK3beta), was also accumulated in Atg7 cKO brains. Chronic pharmacological inhibition of tau phosphorylation, or genetic deletion of tau, significantly rescued Atg7-deficiency-mediated neurodegeneration, but did not suppress inclusion formation. CONCLUSIONS: These data elucidate a role for macroautophagy in the long-term survival and physiological function of adult CNS neurons. Neurodegeneration in the context of macroautophagy deficiency is mediated through a phospho-tau pathway.
PMCID:3544596
PMID: 22998728
ISSN: 1750-1326
CID: 1055752