ArticleAnnals of neurology2022
Septin-5 and -7-IgGs: Neurologic, Serologic, and Pathophysiologic Characteristics.
Article in Annals of neurology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
What it found
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it, 39 citations in OpenAlex.
- Evidence-based consensus guidelines for the management of catatonia: Recommendations from the British Association for Psychopharmacology.Journal of psychopharmacology (Oxford, England) · 2023Guideline
- Septin multimer autoantibodies in severe motor neuropathy mimicking lower motor neuron disease.Brain : a journal of neurology · 2026Article
- Novel methods for neural IgG characterization and validation in autoimmune neurologic disorders.Journal of immunological methods · 2026Review
- Insights Into the Antigenic Repertoire of Unclassified Synaptic Antibodies.Annals of clinical and translational neurology · 2026Article
- Seeing elephants and drinking water from a porcelain vase - A case of septin-7 antibody-associated encephalitis presenting as a severe disorganized psychosis.Translational psychiatry · 2026Article
- Septins in the nervous system: from cytoskeletal dynamics to neurological disorders.Cell communication and signaling : CCS · 2025Review
- Acute neuronal cell death and neuroinflammation per se do not trigger secondary autoimmune encephalitis in mice.Scientific reports · 2025Article
- Anhidrosis in septin-7 autoimmunity.Clinical autonomic research : official journal of the Clinical Autonomic Research Society · 2025Article
- Anti-septin complex positive autoimmune encephalitis after severe falciparum malaria: a case report.Malaria journal · 2024Article
- Long-term outcomes in antibody-negative autoimmune encephalitis: a retrospective study.Journal of neurology · 2024Article
- Human-derived monoclonal autoantibodies as interrogators of cellular proteotypes in the brain.Trends in neurosciences · 2024Review
- Case Presentation of Autoimmune Septin-5 Cerebellar Ataxia.Movement disorders clinical practice · 2024Article
- Autoimmune encephalitis: what the radiologist needs to know.Neuroradiology · 2024Review
- Utility of Protein Microarrays for Detection of Classified and Novel Antibodies in Autoimmune Neurologic Disease.Neurology(R) neuroimmunology & neuroinflammation · 2023Article
- Septin-3 autoimmunity in patients with paraneoplastic cerebellar ataxia.Journal of neuroinflammation · 2023Article
- Paraneoplastic Neurologic Disorders.Current neurology and neuroscience reports · 2023Review
- Case report: Anti septin-5-encephalitis as a treatable cause of cerebellar ataxia and psychiatric symptoms.Frontiers in neurology · 2023Article
Corrections and comments
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Authors and funding
21 authors at 9 institutions in 2 countries.
Funding
Abstract
BACKGROUND AND
objectivesWe sought to determine clinical significance of neuronal septin autoimmunity and evaluate for potential IgG effects.
methodsSeptin-IgGs were detected by indirect immunofluorescence assays (IFAs; mouse tissue and cell based) or Western blot. IgG binding to (and internalization of) extracellular septin epitopes were evaluated for by live rat hippocampal neuron assay. The impact of purified patient IgGs on murine cortical neuron function was determined by recording extracellular field potentials in a multielectrode array platform.
resultsSeptin-IgGs were identified in 23 patients. All 8 patients with septin-5-IgG detected had cerebellar ataxia, and 7 had prominent eye movement disorders. One of 2 patients with co-existing septin-7-IgG had additional psychiatric phenotype (apathy, emotional blunting, and poor insight). Fifteen patients had septin-7 autoimmunity, without septin-5-IgG detected. Disorders included encephalopathy (11; 2 patients with accompanying myelopathy, and 2 were relapsing), myelopathy (3), and episodic ataxia (1). Psychiatric symptoms (≥1 of agitation, apathy, catatonia, disorganized thinking, and paranoia) were prominent in 6 of 11 patients with encephalopathic symptoms. Eight of 10 patients with data available (from 23 total) improved after immunotherapy, and a further 2 patients improved spontaneously. Staining of plasma membranes of live hippocampal neurons produced by patient IgGs (subclasses 1 and 2) colocalized with pre- and post-synaptic markers. Decreased spiking and bursting behavior in mixed cultures of murine glutamatergic and GABAergic cortical neurons produced by patient IgGs were attributable to neither antigenic crosslinking and internalization nor complement activation.
interpretationSeptin-IgGs are predictive of distinct treatment-responsive autoimmune central nervous system (CNS) disorders. Live neuron binding and induced electrophysiologic effects by patient IgGs may support septin-specific pathophysiology. ANN NEUROL 2022;92:1090-1101.
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