Publications by authors named "L S Hirsch"

The time-resolved detection of mid- to far-infrared electric fields absorbed and emitted by molecules is among the most sensitive spectroscopic approaches and has the potential to transform sensing in fields such as security screening, quality control, and medical diagnostics. However, the sensitivity of the standard detection approach, which relies on encoding the far-infrared electric field into amplitude modulation of a visible or near-infrared probe laser pulse, is limited by the shot noise of the latter. This constraint cannot be overcome without using a quantum resource.

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We propose and prioritize important outcome domains that should be considered for future research investigating long-term outcomes (LTO) after new onset refractory status epilepticus (NORSE). The study was led by the international NORSE Institute LTO Working Group. First, literature describing the LTO of NORSE survivors was identified using a PubMed search and summarized to identify knowledge gaps.

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Febrile infection-related epilepsy syndrome (FIRES) is a rare clinical presentation of refractory status epilepticus following a febrile infection. This study analyzes data from the NORSE/FIRES Family Registry, an international web-based registry available in six languages with data entered by patients, families, and clinicians to explore clinical presentations, survivorship, and long-term outcomes in adult and pediatric FIRES patients. We characterize and examine differences in demographics, prodromal symptoms, seizure frequency, anti-seizure medications (ASMs), quality of life, cognition, mood, and anxiety in adults vs pediatric populations with FIRES.

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The N-Myc transcription factor, encoded by MYCN, is a mechanistically validated, yet challenging, target for neuroblastoma (NB) therapy development. In normal neuronal progenitors, N-Myc undergoes rapid degradation, while, in MYCN-amplified NB cells, Aurora kinase A (Aurora-A) binds to and stabilizes N-Myc, resulting in elevated protein levels. Here, we demonstrate that targeted protein degradation of Aurora-A decreases N-Myc levels.

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Objective: To describe the lived experience of patients with NORSE and explore quality of life (QOL) for patients and their caregivers.

Background: NORSE is a rare condition characterized by refractory status epilepticus, often of unknown cause, in a previously neurologically healthy individual. Febrile infection-related epilepsy syndrome (FIRES) is a subset of NORSE.

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