ArticleNeurology(R) neuroimmunology & neuroinflammation2014
Massive CNS monocytic infiltration at autopsy in an alemtuzumab-treated patient with NMO.
Article in Neurology(R) neuroimmunology & neuroinflammation, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers, 1 of them a synthesis that pooled it.
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Who cites it
33 citing papers in PubMed, 1 synthesis or guideline pooled it, 60 citations in OpenAlex.
- Long-term disability in neuromyelitis optica spectrum disorder with a history of myelitis is associated with age at onset, delay in diagnosis/preventive treatment, MRI lesion length and presence of symptomatic brain lesions.Multiple sclerosis and related disorders · 2019Pooled it
- Successful Natalizumab Treatment of Two Female Individuals With Susac Syndrome.European journal of neurology · 2025Article
- Case Report: Severe rebound after withdrawal of fingolimod in a patient with neuromyelitis optica spectrum disorder.Frontiers in immunology · 2023Article
- A single relapse induces worsening of disability and health-related quality of life in patients with neuromyelitis optica spectrum disorder.Frontiers in neurology · 2023Article
- Excessive MALAT1 promotes the immunologic process of neuromyelitis optica spectrum disorder by upregulating BAFF expression.Translational neuroscience · 2023Article
- An Autopsy Confirmed Neuromyelitis Optica Spectrum Disorder with Extensive Brain White Matter Lesion and Optic Neuritis but Intact Spinal Cord, Clinically Mimicking a Secondary Progressive Multiple Sclerosis-like Course.Internal medicine (Tokyo, Japan) · 2022Article
- Immunopathology of Tumefactive Demyelinating Lesions-From Idiopathic to Drug-Related Cases.Frontiers in neurology · 2022Review
- Th17-Related Cytokines as Potential Discriminatory Markers between Neuromyelitis Optica (Devic's Disease) and Multiple Sclerosis-A Review.International journal of molecular sciences · 2021Review
- Review of approved NMO therapies based on mechanism of action, efficacy and long-term effects.Multiple sclerosis and related disorders · 2020Review
- Differential Effects of MS Therapeutics on B Cells-Implications for Their Use and Failure in AQP4-Positive NMOSD Patients.International journal of molecular sciences · 2020Review
- Immune reconstitution therapies: concepts for durable remission in multiple sclerosis.Nature reviews. Neurology · 2020Review
- Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature.Frontiers in neurology · 2020Article
- Differential Diagnosis of Pediatric Multiple Sclerosis.Children (Basel, Switzerland) · 2019Review
- Sex differences in autoimmune disorders of the central nervous system.Seminars in immunopathology · 2019Review
- Cognitive Impairment in Neuromyelitis Optica Spectrum Disorders: A Review of Clinical and Neuroradiological Features.Frontiers in neurology · 2019Review
- Alemtuzumab as Treatment for Multiple Sclerosis.Cold Spring Harbor perspectives in medicine · 2018Review
- Functional characterization of reappearing B cells after anti-CD20 treatment of CNS autoimmune disease.Proceedings of the National Academy of Sciences of the United States of America · 2018Article
- Acute Management of Optic Neuritis: An Evolving Paradigm.Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society · 2018Review
- Optical coherence tomography in neuromyelitis optica spectrum disorders: potential advantages for individualized monitoring of progression and therapy.The EPMA journal · 2018Review
- Diagnosis and Treatment of NMO Spectrum Disorder and MOG-Encephalomyelitis.Frontiers in neurology · 2018Review
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesTo describe the clinical course and neuropathology at autopsy of a patient with neuromyelitis optica (NMO) treated with alemtuzumab.
methodsCase report.
resultsA 61-year-old woman with aquaporin-4 immunoglobulin G antibody seropositive NMO had 10 clinical relapses in 4 years despite treatment with multiple immunosuppressive therapies. Alemtuzumab was administered and was redosed 15 months later. For the first 19 months after the initial alemtuzumab infusion, the patient did not experience discrete clinical relapses or have evidence of abnormally enhancing lesions on brain or spinal cord MRI. However, she experienced insidiously progressive nausea, vomiting, and vision loss, and her brain MRI revealed marked extension of cortical, subcortical, and brainstem T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities. She died 20 months after the initial alemtuzumab infusion. Acute, subacute, and chronic demyelinating lesions were found at autopsy. Many of the lesions showed marked macrophage infiltration with a paucity of lymphocytes.
conclusionsFollowing alemtuzumab treatment, there appeared to be ongoing innate immune activation associated with tissue destruction that correlated with nonenhancing T2/FLAIR hyperintensities on MRI. We interpret the cessation of clinical relapses, absence of contrast-enhancing lesions, and scarcity of lymphocytes at autopsy to be indicative of suppression of adaptive immunity by alemtuzumab. This case illustrates that progressive worsening in NMO can occur as a consequence of tissue injury associated with monocytic infiltration. This observation may be relevant to multiple sclerosis (MS) as well as NMO and might explain why in previous studies of secondary progressive MS alemtuzumab did not seem to inhibit disability progression despite a dramatic decline in contrast-enhancing lesions.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.