Life-threatening secondary hemophagocytic lymphohistiocytosis following vagal nerve stimulator infection in a child with CHD2 myoclonic encephalopathy: a case report

2020 ◽  
Vol 36 (11) ◽  
pp. 2851-2856 ◽  
Author(s):  
Cristiano Parisi ◽  
Santiago Candela-Cantó ◽  
Mercedes Serrano ◽  
Albert Catala ◽  
Javier Aparicio ◽  
...  
Author(s):  
Josia Fauser ◽  
Stefan Köck ◽  
Eberhard Gunsilius ◽  
Andreas Chott ◽  
Andreas Peer ◽  
...  

SummaryHLH is a life-threatening disease, which is characterized by a dysregulated immune response with uncontrolled T cell and macrophage activation. The often fulminant course of the disease needs a fast diagnostic work-up to initiate as soon as possible the appropriate therapy. We present herein the case of a 71-year-old patient with rapidly progressive hyperinflammatory syndrome, which post mortem resulted in the diagnosis of EBV-associated HLH. With this case report, we intend to highlight the relevance of the HScore in the diagnosis of HLH, to create a greater awareness for EBV as a trigger of HLH, and to demonstrate the importance of treating EBV-associated HLH as early as possible.


Author(s):  
Chiara Pizzanelli ◽  
Chiara Milano ◽  
Paolo Perrini ◽  
Davide Di Carlo ◽  
Paola Anna Erba ◽  
...  

2010 ◽  
Vol 67 (suppl_2) ◽  
pp. ons457-ons460
Author(s):  
Brent R. O'Neill ◽  
James E. Wilberger

ABSTRACT BACK GROUND: We describe an approach to vagal nerve stimulator (VNS) lead replacement through the posterior cervical triangle. Scar around the structures of the carotid sheath is avoided and new leads are placed on a pristine section of the vagus nerve proximal to the original site. CLINICAL PRESENTATION: Skin incision from the implantation surgery is incorporated and extended to allow access to the posterior border of the sternocleidomastoid muscle (SCM). Dissection proceeds along the posterior border of the SCM. The SCM and jugular vein are retracted anterior to expose a fresh segment of the vagal nerve immediately superficial to the carotid artery and proximal to the original electrode site. Once the nerve is adequately exposed, electrode placement proceeds in the standard fashion. Dysfunctional electrodes are left in place, and the lead wire is cut as near the electrodes as can be easily accessed. Three patients have undergone lead revision with this approach. Lead placement was successful and free from complications in all cases. CONCLUSION: The posterior cervical triangle approach provides a virgin dissection plane for VNS revision.


2021 ◽  
pp. 194187442110446
Author(s):  
Matthew R. Woodward ◽  
Margaret S. Ferris ◽  
Guillermo Rivell ◽  
Laura Malone ◽  
Tara M. Dutta ◽  
...  

We are writing to present an interesting and novel case from our practice of a patient who presented with altered mental status and a rapidly progressive paraplegia as well as high fevers and pancytopenia. A bone marrow biopsy was diagnostic of hemophagocytic lymphohistiocytosis (HLH) and MRI showed hemorrhagic encephalitis and spinal subarachnoid hemorrhage. This case demonstrates the diverse neurological symptoms with which HLH presents, including spinal cord pathology. The astute neurologist should consider this diagnosis in the appropriate clinical context and diagnosis may require imaging to the complete neuraxis.


Author(s):  
Nolan Winslow ◽  
Sonia Pulido ◽  
Jonathan Garst ◽  
Andres Maldonado

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