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Immune Myopathy With Perimysial Pathology Associated With Interstitial Lung Disease and Anti-EJ Antibodies

Kwon, Patrick M; Zhou, Lan; Motiwala, Rajeev; Kerr, Leslie D; Shin, Susan C
OBJECTIVES/OBJECTIVE:We report a case of immune myopathy with perimysial pathology associated with anti-glycyl-transfer RNA synthetase (anti-EJ) antibody and an excellent treatment response. METHODS:Chart review. RESULTS:A 36-year-old woman presented with 3 months of fatigue, weight loss, progressive weakness in a scapuloperoneal distribution, and dysphagia. Nerve conduction studies, electromyography, and ultrasound suggested an irritable myopathy. She had marked elevations of creatine kinase and positive anti-glycyl-transfer RNA synthetase (anti-EJ) antibodies. A left biceps muscle biopsy revealed inflammation of the perimysium and surrounding perimysial blood vessels with focal fragmentation of the perimysium. Further evaluation revealed interstitial lung disease. Treatment with prednisone and mycophenolate mofetil led to marked clinical improvement of her symptoms. CONCLUSIONS:Our case adds to the growing spectrum of inflammatory myopathies and highlights the importance of performing a comprehensive, multisystem workup.
PMID: 28538253
ISSN: 1537-1611
CID: 2956852

Hypertrophic Olivary Degeneration and Movement Disorder in a Patient with Familial Creutzfeldt-Jakob Disease [Case Report]

Granger, Andre; Agarwal, Shashank; Andino, Andres; Kwon, Patrick; Zakin, Elina
A 38-year-old male presented with a three-week history of bilateral lower extremity choreiform movements. History included sleep abnormalities, rushed and unintelligible speech, with delusions two to six months prior to presentation. He also developed mild dysphagia, staring spells, and anterograde amnesia. On examination, he had pressured speech, asynchronous cycling movements of the bilateral lower extremities persisting during sleep, occasional ballistic movements of the upper extremities, and ataxia. Magnetic resonance imaging (MRI) of the brain showed high cortical signal change in bilateral parieto-occipital cortices with evidence of medullary olive hypertrophy bilaterally. Electroencephalography showed generalized slowing without periodic spikes. Cerebrospinal fluid was positive for protein 14-3-3 and real-time quaking-induced conversion. Genetic testing was positive for autosomal dominant prion protein gene (PRNP) genetic mutation. The patient passed away three months after discharge. This case provides previously undescribed imaging and movement abnormalities in a patient with familial Creutzfeldt-Jakob disease (CJD), and suggests that CJD should not be removed from the differential in patients with these atypical findings.
PMID: 33178508
ISSN: 2168-8184
CID: 4665352

Keeping the team together: Transformation of an inpatient neurology service at an urban, multi-ethnic, safety net hospital in New York City during COVID-19

Lord, Aaron S; Lombardi, Nicole; Evans, Katherine; Deveaux, Dewi; Douglas, Elizabeth; Mansfield, Laura; Zakin, Elina; Jakubowska-Sadowska, Katarzyna; Grayson, Kammi; Omari, Mirza; Yaghi, Shadi; Humbert, Kelley; Sanger, Matt; Kim, Sun; Boffa, Michael; Szuchumacher, Mariana; Jongeling, Amy; Vazquez, Blanca; Berberi, Nisida; Kwon, Patrick; Locascio, Gianna; Chervinsky, Alexander; Frontera, Jennifer; Zhou, Ting; Kahn, D Ethan; Abou-Fayssal, Nada
The COVID-19 pandemic dramatically affected the operations of New York City hospitals during March and April of 2020. This article describes the transformation of a neurology division at a 450-bed tertiary care hospital in a multi-ethnic community in Brooklyn during this initial wave of COVID-19. In lieu of a mass redeployment of staff to internal medicine teams, we report a novel method for a neurology division to participate in a hospital's expansion of care for patients with COVID-19 while maintaining existing team structures and their inherent supervisory and interpersonal support mechanisms.
PMID: 32877768
ISSN: 1872-6968
CID: 4583362

Special considerations in the assessment of catastrophic brain injury and determination of brain death in patients with SARS-CoV-2

Valdes, Eduard; Agarwal, Shashank; Carroll, Elizabeth; Kvernland, Alexandra; Bondi, Steven; Snyder, Thomas; Kwon, Patrick; Frontera, Jennifer; Gurin, Lindsey; Czeisler, Barry; Lewis, Ariane
INTRODUCTION/BACKGROUND:The coronavirus disease 2019 (Covid-19) pandemic has led to challenges in provision of care, clinical assessment and communication with families. The unique considerations associated with evaluation of catastrophic brain injury and death by neurologic criteria in patients with Covid-19 infection have not been examined. METHODS:We describe the evaluation of six patients hospitalized at a health network in New York City in April 2020 who had Covid-19, were comatose and had absent brainstem reflexes. RESULTS:Four males and two females with a median age of 58.5 (IQR 47-68) were evaluated for catastrophic brain injury due to stroke and/or global anoxic injury at a median of 14 days (IQR 13-18) after admission for acute respiratory failure due to Covid-19. All patients had hypotension requiring vasopressors and had been treated with sedative/narcotic drips for ventilator dyssynchrony. Among these patients, 5 had received paralytics. Apnea testing was performed for 1 patient due to the decision to withdraw treatment (n = 2), concern for inability to tolerate testing (n = 2) and observation of spontaneous respirations (n = 1). The apnea test was aborted due to hypoxia and hypotension. After ancillary testing, death was declared in three patients based on neurologic criteria and in three patients based on cardiopulmonary criteria (after withdrawal of support (n = 2) or cardiopulmonary arrest (n = 1)). A family member was able to visit 5/6 patients prior to cardiopulmonary arrest/discontinuation of organ support. CONCLUSION/CONCLUSIONS:It is feasible to evaluate patients with catastrophic brain injury and declare brain death despite the Covid-19 pandemic, but this requires unique considerations.
PMID: 32798855
ISSN: 1878-5883
CID: 4572952

Subclavian steal syndrome due to dialysis fistula corrected with subclavian artery stenting

Agarwal, Shashank; Schwartz, Lisa; Kwon, Patrick; Selas, George; Farkas, Jeffrey; Arcot, Karthikeyan; Tiwari, Ambooj
PMID: 30564504
ISSN: 2163-0402
CID: 3556582

Subclavian Steal Syndrome secondary to Dialysis AVF treated with Balloon Mounted Stent [Meeting Abstract]

Agarwal, Shashank; Kwon, Patrick; Selas, George; Farkas, Jeffrey; Arcot, Karthikeyan; Schwartz, Lisa; Tiwari, Ambooj
ISSN: 0028-3878
CID: 3561412

Subclavian steal syndrome secondary to dialysis AVF treated with covered Biliary Stent [Meeting Abstract]

Agarwal, S; Kwon, P; Farkas, J; Selas, G; Arcot, K; Tiwari, A; Schwartz, L
Introduction: Subclavian steal syndrome (SSS) is usually due to stenosis of the Subclavian Artery (SA) proximal to the origin of the vertebral artery (VA). SSS with intact SA in patients with dialysis arterio-venous fistulas (AVF) has been occasionally reported[1-4]. We present a unique case of the same successfully treated with a covered stent. Methods: A 65-year-old male with DM, HTN, ESRD and left brachial artery- basilic vein fistula (2.27 L/min flow) had multiple admissions with vertebrobasilar symptoms in the setting of elevated BP. Repeated evaluations with CT/CTA/MRI were negative for steno-occlusive disease or infarction. Therefore, symptoms were attributed to hypertensive urgency. On his third presentation in 4 months, he had additional symptoms of left arm pain, weakness and numbness. Signs included left arm hyperemia, warmth and mild motor-sensory deficits. Results: MRA demonstrated reverse flow of blood in left VA with focal stenosis of proximal left SA. Angiogram showed a kinked LSA and rapid/ early shunting to the subcalvian vein. There was no ante-grade visualization of the LVA with reverse flow from the RVA via VB junction. However on compression of the shunt with a BP cuff, antegrade flow in LVA reappeared with disappearance of RVA-LVA steal. BP transduction revealed a 50-mmHg point difference across the kinked segment. Subsequently, proximal left SA was stented with a covered biliary stent resulting in disappearance in RVALVA & LSA-LSV shunts, reappearance of antegrade LVA flow and resolution of symptoms. Conclusions: High-flow AVF is an underdiagnosed cause of symptomatic SSS. We suggest determining AVF flow speeds in any hemodialysis patient who presents with symptoms of posterior circulation insufficiency and obtaining noninvasive vascular studies if flow exceeds 2 L/min or if there has been a recent increase in measured flow during hemodialysis. Obtaining vascular studies with and without fistula compression could be of additional diagnostic utility
ISSN: 1664-5545
CID: 2862382

Acute dystonia in the setting of recanalized left MCA with no residual neurological deficits [Meeting Abstract]

Lalchan, R; Bo, R; M, Liff J; Frontera, J; Kwon, P; Tiwari, A
Introduction: Adult onset focal dystonia often affects the upper extremities and cervical region but less often the lower extremities. Dystonia is the second most reported movement disorder post-stroke and often has a delayed presentation ranging from weeks to months. Most reports are in cases where there is permanent and substantial tissue injury. The clinical significance of Basal ganglia infarction or petechial hemorrhage following endovascular therapy for MCA occlusion is not well understood. The development of dystonia in the setting of a recanalized LMCA has never been reported before. Methods: A 39-year-old female presented with Left MCA occlusion. She had no other medical history except for an idiopathic left basal ganglia hemorrhagic stroke 6 months ago with residual mild forearm weakness. She underwent urgent mechanical thrombectomy with successful sequential recanalization of her inferior followed by superior division. The latter was complicated by a mild self-limiting subarachnoid hemorrhage in the left sylvian fissure. She had small petechial hemorrhages in the left basal ganglia on MRI. However, she recovered completely in 5 days and was discharged with a NIHSS of 1 (similar to baseline) as well as mRS of 2. Ten months later, she developed a painful, fixed right lower extremity dystonia where her ankle was inverted and plantarflexed with her toes curled. This was treated with multiple anticholinergic & GABAergic medications as well as Botox to only achieve partial success. Currently
ISSN: 1664-5545
CID: 2862352

Cerebral Vascular Malformations and Headache

Kwon, Patrick M; Evans, Randolph W; Grosberg, Brian M
PMID: 26255812
ISSN: 1526-4610
CID: 2222342

Adult stem cells from the hyaluronic acid-rich node and duct system differentiate into neuronal cells and repair brain injury

Lee, Seung J; Park, Sang H; Kim, Yu I; Hwang, Sunhee; Kwon, Patrick M; Han, In S; Kwon, Byoung S
The existence of a hyaluronic acid-rich node and duct system (HAR-NDS) within the lymphatic and blood vessels was demonstrated previously. The HAR-NDS was enriched with small (3.0-5.0 mum in diameter), adult stem cells with properties similar to those of the very small embryonic-like stem cells (VSELs). Sca-1(+)Lin(-)CD45(-) cells were enriched approximately 100-fold in the intravascular HAR-NDS compared with the bone marrow. We named these adult stem cells "node and duct stem cells (NDSCs)." NDSCs formed colonies on C2C12 feeder layers, were positive for fetal alkaline phosphatase, and could be subcultured on the feeder layers. NDSCs were Oct4(+)Nanog(+)SSEA-1(+)Sox2(+), while VSELs were Oct4(+)Nanog(+)SSEA-1(+)Sox2(-). NDSCs had higher sphere-forming efficiency and proliferative potential than VSELs, and they were found to differentiate into neuronal cells in vitro. Injection of NDSCs into mice partially repaired ischemic brain damage. Thus, we report the discovery of potential adult stem cells that may be involved in tissue regeneration. The intravascular HAR-NDS may serve as a route that delivers these stem cells to their target tissues.
PMID: 25027245
ISSN: 1557-8534
CID: 2222332