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A nine-month-old boy with regression of milestones and severe constipation: an unusual case of a large spinal NTRK1 fusion pilocytic astrocytoma

Offenbacher, Rachel; Kobets, Andrew; Dalvi, Nagma; Hsu, Kevin; Chin, Steven; Snuderl, Matija; Levy, Adam; Martin, Allison
INTRODUCTION/BACKGROUND:Pilocytic astrocytoma, a World Health Organization grade 1 tumor, is the most common brain tumor in children between 5 and 14 years of age and the second most common in children younger than 5 and older than 14. Although classical to the cerebellum and hypothalamic regions, it can also arise in the spinal cord. Larotrectinib, a selective inhibitor of tropomyosin receptor kinase, has been effective in pediatric tumors with NTRK fusion mutations in children as young as 1-month-old. CASE/METHODS:We share the case of a 9-month-old boy who presented with a 4-month history of regression of his milestones and severe constipation who was found to have a large spinal pilocytic astrocytoma with multiple intracranial periventricular lesions.
PMID: 36107222
ISSN: 1433-0350
CID: 5336352

An Unexpected Cause of Right-Sided Facial and Periorbital Edema

Tauber, Jenna; Joiner, Devon; Hsu, Kevin; Barmettler, Anne
PMID: 35420574
ISSN: 1537-2677
CID: 5443222

Impact of MRA Echo Time on Stroke Prevention Therapy in Pediatric Patients with Sickle Cell Disease [PrePrint]

Dhillon, Parmpreet; Morrone, Kerry; Hsu, Kevin; Gomes, William; Silver, Ellen; Lax, Daniel; Peng, Qi; Lee, Seon Kyu; Manwani, Deepa; Mitchell, William
ISSN: n/a
CID: 5295672

Bilateral cranial nerve 6 palsy in a patient with multiple sclerosis and vitamin D-dependent rickets [Case Report]

Sriram, Aishwarya; Joiner, Devon; Hsu, Kevin; Zhang, Cheng
The development of multiple sclerosis (MS) is multifactorial. Elevated levels of vitamin D may lower the risk and reduce relapses by immunomodulatory mechanisms. Conversely, vitamin D-dependent rickets (VDDR), an inheritable form of rickets secondary to impairment in vitamin D synthesis or action, may increase MS risk. This has been described in three patients with VDDR type 1A. Here, we present a patient with VDDR type 2 - unclear if type 2A or 2B based on historical genetic testing - who subsequently developed MS. She presented with 8 weeks of binocular horizontal diplopia and was found to have 8 prism dioptres of esotropia in primary gaze and a mild limitation of abduction in both eyes. Radiological workup was consistent with MS demyelination. She was started on solumedrol infusions, with full resolution of the esotropia and abduction deficits. She has since been transitioned to ocrelizumab with vitamin D supplementation and has not had a relapse to date. It is important to consider MS in patients genetically predisposed to low vitamin D levels or functional impairment, as with VDDR. Vitamin D supplementation can achieve remission in some forms of VDDR, and its role in MS prevention in these patients should be considered. In patients with type 2A or 2B VDDR, who have impairment in receptor function, additional treatment modalities require investigation. Lastly, demyelination is a rare cause of bilateral cranial nerve 6 palsy. This case illustrates the importance of considering MS in cranial nerve palsies, particularly in patients with vitamin D deficiencies or functional impairment.
PMID: 36544586
ISSN: 0165-8107
CID: 5443212

Lyme Neuroborreliosis Presenting as Multiple Cranial Neuropathies [Case Report]

Sriram, Aishwarya; Lessen, Samantha; Hsu, Kevin; Zhang, Cheng
Neuroborreliosis can manifest with cranial nerve (CN) palsies, commonly CN VII. Rarely have isolated or multiple palsies been reported. We describe a case of a young female from a Lyme endemic region who presented with bilateral CN VI palsies and a dilated right pupil, possibly a partial CN III palsy. She later developed CN VII palsy and bilateral enhancement of multiple cranial nerves on neuroimaging. She was diagnosed with Lyme disease by serological testing, with gradual improvement on antibiotics. Our case illustrates that neuroborreliosis can present as any or multiple CN palsies, and should be considered particularly in endemic areas.
PMID: 35273419
ISSN: 0165-8107
CID: 5443202

Review of COVID-19, part 1: Abdominal manifestations in adults and multisystem inflammatory syndrome in children

Kanmaniraja, Devaraju; Kurian, Jessica; Holder, Justin; Gunther, Molly Somberg; Chernyak, Victoria; Hsu, Kevin; Lee, Jimmy; Mcclelland, Andrew; Slasky, Shira E; Le, Jenna; Ricci, Zina J
The coronavirus disease 2019 (COVID -19) pandemic caused by the novel severe acute respiratory syndrome coronavirus (SARS-CoV-2) has affected almost every country in the world, resulting in severe morbidity, mortality and economic hardship, and altering the landscape of healthcare forever. Although primarily a pulmonary illness, it can affect multiple organ systems throughout the body, sometimes with devastating complications and long-term sequelae. As we move into the second year of this pandemic, a better understanding of the pathophysiology of the virus and the varied imaging findings of COVID-19 in the involved organs is crucial to better manage this complex multi-organ disease and to help improve overall survival. This manuscript provides a comprehensive overview of the pathophysiology of the virus along with a detailed and systematic imaging review of the extra-thoracic manifestation of COVID-19 with the exception of unique cardiothoracic features associated with multisystem inflammatory syndrome in children (MIS-C). In Part I, extra-thoracic manifestations of COVID-19 in the abdomen in adults and features of MIS-C will be reviewed. In Part II, manifestations of COVID-19 in the musculoskeletal, central nervous and vascular systems will be reviewed.
PMID: 34298343
ISSN: 1873-4499
CID: 5244912

Review of COVID-19, part 2: Musculoskeletal and neuroimaging manifestations including vascular involvement of the aorta and extremities

Kanmaniraja, Devaraju; Le, Jenna; Hsu, Kevin; Lee, Jimmy S; Mcclelland, Andrew; Slasky, Shira E; Kurian, Jessica; Holder, Justin; Gunther, Molly Somberg; Chernyak, Victoria; Ricci, Zina J
The coronavirus disease 2019 (COVID-19) pandemic caused by the novel severe acute respiratory syndrome coronavirus (SARS-CoV-2) has affected almost every country in the world resulting in severe morbidity, mortality and economic hardship, altering the landscape of healthcare forever. Its devastating and most frequent thoracic and cardiac manifestations have been well reported since the start of the pandemic. Its extra-thoracic manifestations are myriad and understanding them is critical in diagnosis and disease management. The role of radiology is growing in the second wave and second year of the pandemic as the multiorgan manifestations of COVID-19 continue to unfold. Musculoskeletal, neurologic and vascular disease processes account for a significant number of COVID-19 complications and understanding their frequency, clinical sequelae and imaging manifestations is vital in guiding management and improving overall survival. The authors aim to provide a comprehensive overview of the pathophysiology of the virus along with a detailed and systematic imaging review of the extra-thoracic manifestation of COVID-19. In Part I, abdominal manifestations of COVID-19 in adults and multisystem inflammatory syndrome in children will be reviewed. In Part II, manifestations of COVID-19 in the musculoskeletal, central nervous and vascular systems will be reviewed.
PMID: 34388683
ISSN: 1873-4499
CID: 5244922

Arterial Spin Labeling Applications in Neuroimaging: Emerging Use in Head and Neck Imaging

Hsu, Kevin
ISSN: 2589-8701
CID: 5443252

Neurologic Syndromes Predict Higher In-Hospital Mortality in COVID-19

Eskandar, Emad Nader; Altschul, David J; de La Garza Ramos, Rafael; Cezayirli, Phillip; Unda, Santiago R; Benton, Joshua; Dardick, Joseph; Toma, Aureliana; Patel, Nikunj; Malaviya, Avinash; Flomenbaum, David; Fernandez-Torres, Jenelys; Lu, Jenny; Holland, Ryan; Burchi, Elisabetta; Zampolin, Richard; Hsu, Kevin; McClelland, Andrew; Burns, Judah; Erdfarb, Amichai; Malhotra, Rishi; Gong, Michelle; Semczuk, Peter; Ferastraoaru, Victor; Rosengard, Jillian; Antoniello, Daniel; Labovitz, Daniel; Esenwa, Charles; Milstein, Mark; Boro, Alexis; Mehler, Mark F
OBJECTIVE:The SARS-Cov2 virus is protean in its manifestations, affecting nearly every organ system. However, nervous system involvement and its impact on disease outcome are poorly characterized. The objective of the study is to determine if neurological syndromes are associated with increased risk of inpatient mortality. METHODS:581 hospitalized patients with confirmed SARS-Cov2 infection, neurological involvement and brain-imaging were compared to hospitalized non-neurological COVID-19 patients. Four patterns of neurological manifestations were identified -acute stroke, new or recrudescent seizures, altered mentation with normal imaging, and neuro-COVID-19 complex. Factors present on admission were analyzed as potential predictors of in-hospital mortality, including sociodemographic variables, pre-existing comorbidities, vital-signs, laboratory values, and pattern of neurological manifestations. Significant predictors were incorporated into a disease-severity score. Patients with neurological manifestations were matched with patients of the same age and disease severity to assess the risk of death. RESULTS:4711 patients with confirmed SARS-Cov2 infection were admitted to one medical system in New York City during a 6-week period. Of these, 581 (12%) had neurological issues of sufficient concern to warrant neuro-imaging. These patients were compared to 1743 non-neurological COVID-19 patients matched for age and disease-severity admitted during the same period. Patients with altered mentation (n=258, p =0.04, OR 1.39, CI 1.04 - 1.86) or radiologically confirmed stroke (n=55, p = 0.001, OR 3.1, CI 1.65-5.92) had a higher risk of mortality than age and severity-matched controls. CONCLUSIONS:The incidence of altered mentation or stroke on admission predicts a modest but significantly higher risk of in-hospital mortality independent of disease severity. While other biomarker factors also predict mortality, measures to identify and treat such patients may be important in reducing overall mortality of COVID-19.
PMID: 33443111
ISSN: 1526-632x
CID: 4776982

Utility of Apical Lung Assessment on Computed Tomography Angiography as a COVID-19 Screen in Acute Stroke

Esenwa, Charles; Lee, Ji-Ae; Nisar, Taha; Shmukler, Anna; Goldman, Inessa; Zampolin, Richard; Hsu, Kevin; Labovitz, Daniel; Altschul, David; Haramati, Linda B
BACKGROUND AND PURPOSE:Evaluation of the lung apices using computed tomography angiography of the head and neck during acute ischemic stroke (AIS) can provide the first objective opportunity to screen for coronavirus disease 2019 (COVID-19). METHODS:We performed an analysis assessing the utility of apical lung exam on computed tomography angiography for COVID-19-specific lung findings in 57 patients presenting with AIS. We measured the diagnostic accuracy of apical lung assessment alone and in combination with patient-reported symptoms and incorporate both to propose a COVID-19 era AIS algorithm. RESULTS:Apical lung assessment when used in isolation, yielded a sensitivity of 0.67, specificity of 0.93, positive predictive value of 0.19, negative predictive value of 0.99, and accuracy of 0.92 for the diagnosis of COVID-19, in patients presenting to the hospital for AIS. When combined with self-reported clinical symptoms of cough or shortness of breath, sensitivity of apical lung assessment improved to 0.83. CONCLUSIONS:Apical lung assessment on computed tomography angiography is an accurate screening tool for COVID-19 and can serve as part of a combined screening approach in AIS.
PMID: 33115325
ISSN: 1524-4628
CID: 5443192