Esther Nie MD, PhD is currently a Neuroimmunology Fellow with a special focus on neuroimmuno-oncology. She completed Adult Neurology Residency at Stanford. Dr. Nie's clinical interests include neurological presentations of CAR-T neurotoxicity, BITE therapies, and CNS/PNS demyelinating diseases. Her research interests include: microglial response in CAR-T therapy, steroid effects on the CAR-T/neurological outcomes, and enhancing repair after brain injury.

Clinical Focus

  • Neuroimmunology
  • Neurology

Academic Appointments

Professional Education

  • Fellowship: Stanford University Dept of Neurology (2023) CA
  • Board Certification: American Board of Psychiatry and Neurology, Neurology (2022)
  • Residency: Stanford University Neurology Residency (2022) CA
  • Internship: Cedars Sinai Medical Center Internal Medicine Residency (2019) CA
  • Medical Education: UCLA David Geffen School Of Medicine Registrar (2018) CA
  • M.D., UCLA David Geffen School of Medicine, Medicine (2018)
  • Ph.D., UCLA David Geffen School of Medicine, Neuroscience (2016)
  • B.S., Yale University, Molecular Biophysics and Biochemistry (2009)

Clinical Trials

  • A Study of Anti-CD19 Chimeric Antigen Receptor T-Cell ( CAR-T) Therapy in Subjects With Non-relapsing and Progressive Forms of Multiple Sclerosis Recruiting

    A Study of Anti-CD19 Chimeric Antigen Receptor T Cell Therapy in Subjects with Non-relapsing and Progressive Forms of Multiple Sclerosis

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All Publications

  • Clinical Features of Neurotoxicity Following CD19 CAR T-cell Therapy in Mantle Cell Lymphoma. Blood advances Nie, E. H., Su, Y. J., Baird, J. H., Agarwal, N., Bharadwaj, S., Weng, W. K., Smith, M., Dahiya, S., Han, M. H., Dunn, J. E., Kipp, L. B., Miklos, D. B., Scott, B. J., Frank, M. J. 2024


    CD19 chimeric antigen receptor (CAR) T-cell therapy has proven highly effective for treating relapsed/refractory mantle cell lymphoma (MCL). However, immune effector cell-associated neurotoxicity syndrome (ICANS) remains a significant concern. This study aimed to evaluate the clinical, radiological, and laboratory correlatives associated with ICANS development following CD19 CAR T-cell therapy in patients with MCL. All patients (n = 26) who received standard of care brexucabtagene autoleucel until July 2022 at our institution were evaluated. Laboratory and radiographic correlatives including brain magnetic resonance imaging (MRI) and electroencephalogram (EEG) were evaluated to determine the clinical impact of ICANS. Seventeen (65%) patients experienced ICANS after treatment, with a median onset on day 6. Ten (38%) patients experienced severe (≥ grade 3) ICANS. All ICANS patients had antecedent cytokine release syndrome (CRS), but no correlation was observed between ICANS severity and CRS grade. 92% of EEGs revealed interictal changes; no patients experienced frank seizures due to ICANS. 86% of severe ICANS patients with post-infusion brain MRIs demonstrated acute neuroimaging findings not seen on pretreatment MRI. Severe ICANS was also associated with higher rates of cytopenia, coagulopathy, increased cumulative steroid exposure, and prolonged hospitalization. However, severe ICANS did not affect treatment outcomes of patients with MCL. Severe ICANS is frequently associated with a range of post-infusion brain MRI changes and abnormal EEG findings. Longer hospitalization was observed in severe ICANS patients, especially those with abnormal acute MRI or EEG findings, but there was no discernible impact on overall treatment response and survival.

    View details for DOI 10.1182/bloodadvances.2023011896

    View details for PubMedID 38295285

  • A rare non-gadolinium enhancing sarcoma brain metastasis with microenvironment dominated by tumor-associated macrophages. Acta neuropathologica communications Rogawski, D., Wheeler, J., Nie, E., Zhu, W., Villanueva, E., Coffey, G., Ma, Q., Ganjoo, K., Fischbein, N., Iv, M., Vogel, H., Nagpal, S. 2024; 12 (1): 15


    Brain metastases occur in 1% of sarcoma cases and are associated with a median overall survival of 6 months. We report a rare case of a brain metastasis with unique radiologic and histopathologic features in a patient with low grade fibromyxoid sarcoma (LGFMS) previously treated with immune checkpoint inhibitor (ICI) therapy. The lone metastasis progressed in the midbrain tegmentum over 15 months as a non-enhancing, T2-hyperintense lesion with peripheral diffusion restriction, mimicking a demyelinating lesion. Histopathology of the lesion at autopsy revealed a rich infiltrate of tumor-associated macrophages (TAMs) with highest density at the leading edge of the metastasis, whereas there was a paucity of lymphocytes, suggestive of an immunologically cold environment. Given the important immunosuppressive and tumor-promoting functions of TAMs in gliomas and carcinoma/melanoma brain metastases, this unusual case provides an interesting example of a dense TAM infiltrate in a much rarer sarcoma brain metastasis.

    View details for DOI 10.1186/s40478-023-01713-8

    View details for PubMedID 38254244

    View details for PubMedCentralID 5021195

  • Epidemiological and Clinical Outcome Determinants of Post-COVID-19 Myelopathy Sumera, J., Sarkar, T., McDonald, J., Sattarnezhad, N., Nie, E., Kipp, L., Dunn, J., Han, M., Joseph, Y., Tomczak, A., Lock, C. SAGE PUBLICATIONS LTD. 2023: 719
  • SARS-CoV-2 Vaccine Immune Response on Anti-Complement Therapy, Eculizumab Sattarnezhad, N., Sumera, J., McDonald, J., Nie, E., Tomczak, A., Joseph, Y., Kalle, S., Sarkar, T., Kipp, L., Lock, C., Dunn, J. E., Han, M. LIPPINCOTT WILLIAMS & WILKINS. 2023
  • Myelin Oligodendrocyte Glycoprotein Antibody Disease (MOGAD) in conjunction with Allogeneic Bone Marrow Transplantation and Autoimmune Neutropenia: A study of two companion cases and institutional review of the MOGAD clinical phenotype spectrum Nie, E., McDonald, J., Dunn, J., Han, M. LIPPINCOTT WILLIAMS & WILKINS. 2023
  • Multifocal demyelinating leukoencephalopathy and oligodendroglial lineage cell loss with immune effector cell-associated neurotoxicity syndrome (ICANS) following CD19 CAR T-cell therapy for mantle cell lymphoma. Journal of neuropathology and experimental neurology Nie, E. H., Ahmadian, S. S., Bharadwaj, S. N., Acosta-Alvarez, L., Threlkeld, Z. D., Frank, M. J., Miklos, D. B., Monje, M., Scott, B. J., Vogel, H. 2023


    Immune effector cell-associated neurotoxicity syndrome (ICANS) is a prevalent condition seen after treatment with chimeric antigen receptor T-cell (CAR T) therapy and other cancer cell therapies. The underlying pathophysiology and neuropathology of the clinical syndrome are incompletely understood due to the limited availability of brain tissue evaluation from patient cases, and a lack of high-fidelity preclinical animal models for translational research. Here, we present the cellular and tissue neuropathologic analysis of a patient who experienced grade 4 ICANS after treatment with anti-CD19 CAR T therapy for mantle cell lymphoma. Our pathologic evaluation reveals a pattern of multifocal demyelinating leukoencephalopathy associated with a clinical course of severe ICANS. A focused analysis of glial subtypes further suggests region-specific oligodendrocyte lineage cell loss as a potential cellular and pathophysiologic correlate in severe ICANS. We propose a framework for the continuum of neuropathologic changes thus far reported across ICANS cases. Future elucidation of the mechanistic processes underlying ICANS will be critical in minimizing neurotoxicity following CAR T-cell and related immunotherapy treatments across oncologic and autoimmune diseases.

    View details for DOI 10.1093/jnen/nlac121

    View details for PubMedID 36592076

  • A rare neuromyelitis optica mimic: Primary CNS histiocytic sarcoma. Multiple sclerosis (Houndmills, Basingstoke, England) Rogawski, D. S., Nirschl, J. J., McDonald, J., Nie, E., Schwartz, N. U., Vogel, H., Scott, B. J., Gold, C. A., Kipp, L. B. 2022; 28 (10): 1651-1654


    Primary central nervous system (CNS) histiocytic sarcoma is a rare hematolymphoid malignancy with features of mature histiocytes and carries a poor prognosis. We describe a unique case in which a 50-year-old woman presented with recurrent acute brainstem syndrome, area postrema syndrome, and myelitis with corresponding magnetic resonance imaging (MRI) lesions meeting diagnostic criteria for seronegative neuromyelitis optica spectrum disorder (NMOSD). Despite initial improvement with steroids and plasma exchange, she experienced recurrent symptoms over 10months referable to new and persistently enhancing lesions. At autopsy, neuropathology revealed a diffusely infiltrative primary CNS histiocytic sarcoma. This case represents a rare clinicoradiologic mimic of NMOSD, underscoring the importance of evaluation for infiltrative diseases in cases of atypical seronegative NMOSD.

    View details for DOI 10.1177/13524585221097564

    View details for PubMedID 35876468

  • Major tumor regressions in H3K27M-mutated diffuse midline glioma (DMG) following sequential intravenous (IV) and intracerebroventricular (ICV) delivery of GD2-CAR T cells Majzner, R. G., Mahdi, J., Ramakrishna, S., Patel, S., Chinnasamy, H., Yeom, K., Schultz, L., Barsan, V., Richards, R., Campen, C., Reschke, A., Toland, A., Baggott, C., Mavroukakis, S., Egeler, E., Moon, J., Jacobs, A., Yamabe-Kwong, K., Rasmussen, L., Nie, E., Green, S., Kunicki, M., Fujimoto, M., Ehlinger, Z., Reynolds, W., Prabhu, S., Warren, K. E., Cornell, T., Partap, S., Fisher, P., Grant, G., Vogel, H., Sahaf, B., Davis, K., Feldman, S., Monje, M., Mackall, C. L. AMER ASSOC CANCER RESEARCH. 2022
  • MAJOR TUMOR REGRESSIONS IN H3K27M-MUTATED DIFFUSE MIDLINE GLIOMA (DMG) FOLLOWING SEQUENTIAL INTRAVENOUS (IV) AND INTRACEREBROVENTRICULAR (ICV) DELIVERY OF GD2-CAR T-CELLS Monje, M., Majzner, R., Mahdi, J., Ramakrishna, S., Patel, S., Chinnasamy, H., Yeom, K., Schultz, L., Barsan, V., Richards, R., Campen, C., Reschke, A., Toland, A., Baggott, C., Mavroukakis, S., Egeler, E., Moon, J., Jacobs, A., Yamabe-Kwong, K., Rasmussen, L., Nie, E., Green, S., Kunicki, M., Fujimoto, M., Ehlinger, Z., Reynolds, W., Prabhu, S., Warren, K. E., Cornell, T., Partap, S., Fisher, P., Grant, G., Vogel, H., Sahaf, B., Davis, K., Feldman, S., Mackall, C. OXFORD UNIV PRESS INC. 2022: 20-21
  • Multifocal demyelinating leukoencephalopathy and oligodendroglial lineage cell loss with CD19 CAR T-cell lymphoma therapy Nie, E., Ahmadian, S., Bharadwaj, S., Acosta-Alvarez, L., Threlkeld, Z., Frank, M., Miklos, D., Born, D., Scott, B., Monje, M., Vogel, H. OXFORD UNIV PRESS INC. 2022: 464
  • Primary central nervous system histiocytic sarcoma presenting as neuromyelitis optica Nirschl, J., Rogawski, D., McDonald, J., Nie, E., Schwartz, N., Scott, B., Gratzinger, D., Gold, C., Kipp, L., Vogel, H. OXFORD UNIV PRESS INC. 2022: 491
  • HLA-DR4-positive Vogt-Koyanagi-Harada (VKH) Syndrome: Review of pathophysiology and clinical correlation (P1-1.Virtual) Loeffler, J., Nie, E., Han, M. LIPPINCOTT WILLIAMS & WILKINS. 2022
  • GD2-CAR T cell therapy for H3K27M-mutated diffuse midline gliomas. Nature Majzner, R. G., Ramakrishna, S., Yeom, K. W., Patel, S., Chinnasamy, H., Schultz, L. M., Richards, R. M., Jiang, L., Barsan, V., Mancusi, R., Geraghty, A. C., Good, Z., Mochizuki, A. Y., Gillespie, S. M., Toland, A. M., Mahdi, J., Reschke, A., Nie, E., Chau, I. J., Rotiroti, M. C., Mount, C. W., Baggott, C., Mavroukakis, S., Egeler, E., Moon, J., Erickson, C., Green, S., Kunicki, M., Fujimoto, M., Ehlinger, Z., Reynolds, W., Kurra, S., Warren, K. E., Prabhu, S., Vogel, H., Rasmussen, L., Cornell, T. T., Partap, S., Fisher, P. G., Campen, C. J., Filbin, M. G., Grant, G., Sahaf, B., Davis, K. L., Feldman, S. A., Mackall, C. L., Monje, M. 2022


    Diffuse intrinsic pontine glioma (DIPG) and other H3K27M-mutated diffuse midline gliomas (DMG) are universally lethal paediatric central nervous system tumours1. We previously discovered that the disialoganglioside GD2 is highly expressed on H3K27M-mutant glioma cells and demonstrated promising preclinical efficacy of GD2-directed chimeric antigen receptor (CAR) T cells2, providing the rationale for a first-in-human Phase 1 clinical trial (NCT04196413). Because CAR T-cell-induced brainstem inflammation can result in obstructive hydrocephalus, increased intracranial pressure, and dangerous tissue shifts, neurocritical care precautions were incorporated. Here we present the clinical experience from the first four patients with H3K27M-mutant DIPG/DMG treated with GD2-CAR T cells (GD2-CART) at dose level 1 (1e6 GD2-CAR T cells/kg administered intravenously). Patients who exhibited clinical benefit were eligible for subsequent GD2-CAR T infusions administered intracerebroventricularly3. Toxicity was largely related to tumor location and reversible with intensive supportive care. On-target, off-tumor toxicity was not observed. Three of four patients exhibited clinical and radiographic improvement. Proinflammatory cytokines were increased in plasma and cerebrospinal fluid (CSF). Transcriptomic analyses of 65,598 single cells from CAR T cell products and CSF elucidate heterogeneity in response between subjects and administration routes. These early results underscore the promise of this approach for H3K27M+ DIPG/DMG therapy.

    View details for DOI 10.1038/s41586-022-04489-4

    View details for PubMedID 35130560

  • Molecular, cellular and functional events in axonal sprouting after stroke. Experimental neurology Carmichael, S. T., Kathirvelu, B., Schweppe, C. A., Nie, E. H. 2017; 287 (Pt 3): 384-394


    Stroke is the leading cause of adult disability. Yet there is a limited degree of recovery in this disease. One of the mechanisms of recovery is the formation of new connections in the brain and spinal cord after stroke: post-stroke axonal sprouting. Studies indicate that post-stroke axonal sprouting occurs in mice, rats, primates and humans. Inducing post-stroke axonal sprouting in specific connections enhances recovery; blocking axonal sprouting impairs recovery. Behavioral activity patterns after stroke modify the axonal sprouting response. A unique regenerative molecular program mediates this aspect of tissue repair in the CNS. The types of connections that are formed after stroke indicate three patterns of axonal sprouting after stroke: reactive, reparative and unbounded axonal sprouting. These differ in mechanism, location, relationship to behavioral recovery and, importantly, in their prospect for therapeutic manipulation to enhance tissue repair.

    View details for DOI 10.1016/j.expneurol.2016.02.007

    View details for PubMedID 26874223

    View details for PubMedCentralID PMC4980303

  • GDF10 is a signal for axonal sprouting and functional recovery after stroke NATURE NEUROSCIENCE Li, S., Nie, E. H., Yin, Y., Benowitz, L. I., Tung, S., Vinters, H. V., Bahjat, F. R., Stenzel-Poore, M. P., Kawaguchi, R., Coppola, G., Carmichael, S. T. 2015; 18 (12): 1737-1745

    View details for DOI 10.1038/nn.4146

    View details for PubMedID 26502261

  • αβγ-Synuclein triple knockout mice reveal age-dependent neuronal dysfunction. Proceedings of the National Academy of Sciences of the United States of America Greten-Harrison, B., Polydoro, M., Morimoto-Tomita, M., Diao, L., Williams, A. M., Nie, E. H., Makani, S., Tian, N., Castillo, P. E., Buchman, V. L., Chandra, S. S. 2010; 107 (45): 19573-8


    Synucleins are a vertebrate-specific family of abundant neuronal proteins. They comprise three closely related members, α-, β-, and γ-synuclein. α-Synuclein has been the focus of intense attention since mutations in it were identified as a cause for familial Parkinson's disease. Despite their disease relevance, the normal physiological function of synucleins has remained elusive. To address this, we generated and characterized αβγ-synuclein knockout mice, which lack all members of this protein family. Deletion of synucleins causes alterations in synaptic structure and transmission, age-dependent neuronal dysfunction, as well as diminished survival. Abrogation of synuclein expression decreased excitatory synapse size by ∼30% both in vivo and in vitro, revealing that synucleins are important determinants of presynaptic terminal size. Young synuclein null mice show improved basic transmission, whereas older mice show a pronounced decrement. The late onset phenotypes in synuclein null mice were not due to a loss of synapses or neurons but rather reflect specific changes in synaptic protein composition and axonal structure. Our results demonstrate that synucleins contribute importantly to the long-term operation of the nervous system and that alterations in their physiological function could contribute to the development of Parkinson's disease.

    View details for DOI 10.1073/pnas.1005005107

    View details for PubMedID 20974939

    View details for PubMedCentralID PMC2984188