Chorea Minor Associated with Anti-Neurochondrin Autoantibodies

Rommel, Frank Risto; Miske, Ramona; Stöcker, W.; Arneth, Borros; Neubauer, Bernd A.; Hahn, Andreas

doi:10.1055/s-0037-1606371

Cited by 15 publications

(13 citation statements)

References 4 publications

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“…4,5 Neurochondrin autoimmunity was previously described in 3 adult patients with cerebellar degeneration and in a child with chorea. 6,7 All but 1 of the 7 patients with clinical information available in our report had predominance of rhombencephalitis (cerebellar and brainstem localization). Myelopathy and acute psychosis (accompanying ataxia) and neuropathy (without other features) were each encountered in single patients.…”

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confidence: 58%

“…We confirm the principal clinical accompaniments originally described, namely, severe cerebellar ataxia and other features of rhombencephalitis, and usually without evidence of systemic cancer. Other phenotypes include chorea 7 and small fiber neuropathy (reported herein). Two of our patients with rhombencephalitis had additional neuropsychiatric findings (psychosis or myelopathy).…”

Section: Discussionmentioning

confidence: 80%

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Neurochondrin neurological autoimmunity

Shelly

Kryzer

Komorowski

et al. 2019

Neurol Neuroimmunol Neuroinflamm

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ObjectivesTo describe the neurologic spectrum and treatment outcomes for neurochondrin-IgG positive cases identified serologically in the Mayo Clinic Neuroimmunology Laboratory.MethodsArchived serum and CSF specimens previously scored positive for IgGs that stained mouse hippocampal tissue in a nonuniform synaptic pattern by immunofluorescence assay (89 among 616,025 screened, 1993–2019) were reevaluated. Antibody characterization experiments revealed specificity for neurochondrin, confirmed by recombinant protein assays.ResultsIgG in serum (9) or CSF (4) from 8 patients yielded identical neuron-restricted CNS patterns, most pronounced in hippocampus (stratum lucidum in particular), cerebellum (Purkinje cells and molecular layer), and amygdala. All were neurochondrin-IgG positive. Five were women; median symptom onset age was 43 years (range, 30–69). Of 7 with clinical data, 6 presented with rapidly progressive cerebellar ataxia, brainstem signs, or both; 1 had isolated unexplained psychosis 1 year prior. Five of 6 had cerebellar signs, 4 with additional brainstem symptoms or signs (eye movement abnormalities, 3; dysphagia, 2; nausea and vomiting, 1). One patient with brainstem signs (vocal cord paralysis and VII nerve palsy) had accompanying myelopathy (longitudinally extensive abnormality on MRI; aquaporin-4-IgG and myelin oligodendrocyte glycoprotein-IgG negative). The 7th patient had small fiber neuropathy only. Just 1 of 7 had contemporaneous cancer (uterine). Six patients with ataxia or brainstem signs received immunotherapy, but just 1 remained ambulatory. At last follow-up, 5 had MRI evidence of severe cerebellar atrophy.ConclusionIn our series, neurochondrin autoimmunity was usually accompanied by a nonparaneoplastic rapidly progressive rhombencephalitis with poor neurologic outcomes. Other phenotypes and occasional paraneoplastic causes may occur.

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confidence: 58%

Section: Discussionmentioning

confidence: 80%

Neurochondrin neurological autoimmunity

Shelly

Kryzer

Komorowski

et al. 2019

Neurol Neuroimmunol Neuroinflamm

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“…So far, only 18 cases (together with the presented case 19 cases) with autoimmunity associated with anti‐neurochondrin antibodies have been described, most often with clinically evident cerebellar or brainstem syndromes [3, 4, 15–19]. Anti‐neurochondrin antibodies belong to those antibodies supporting the diagnosis of PACA [2].…”

Section: Discussionmentioning

confidence: 99%

“…Five MRIs were described as normal [4,15] (5/18, 27.8%); four of them were neuropaediatric patients and the last one was the patient with the small fibre neuropathy. A summary of the diagnostic findings of all published cases with anti-neurochondrin associated autoimmunity is listed in clinical improvement (including our patient; 52.6%) [3,4,15,17,18].…”

Section: Liter Ature Re Vie Wmentioning

confidence: 95%

“…Eighteen of 19 patients with autoimmunity and detection of anti‐neurochondrin antibodies received an immunotherapy after diagnosis (94.7%); only one patient remained untreated [4]. Fourteen cases were treated (amongst others) with steroids [4, 15, 16, 18, 19] (14/18, 77.8%), 11 with intravenous immunoglobulins (IVIG) [3, 4, 17, 18] (11/18, 61.1%), five (including our case) with cyclophosphamide [3, 4, 19] (5/18, 27.8%), four with azathioprine [3, 4, 15] (4/18, 22.2%), four with rituximab [4, 18] (4/18, 22.2%), two with mycophenolate [16, 19] (2/18, 11.1%) and two with plasmapheresis [4, 19] (2/18, 11.1%). All forms of therapies are listed in Table 6.…”

Section: Literature Reviewmentioning

confidence: 99%

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Anti‐neurochondrin antibody as a biomarker in primary autoimmune cerebellar ataxia—a case report and review of the literature

Schwarzwald

Salmen

Betancourt

et al. 2022

Euro J of Neurology

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Background and purpose: Neuronal autoantibodies can support the diagnosis of primary autoimmune cerebellar ataxia (PACA). Knowledge of PACA is still sparce. This article aims to highlight the relevance of anti-neurochondrin antibodies and possible therapeutical consequences in people with PACA.Methods: This is a case presentation and literature review of PACA associated with antineurochondrin antibodies. Results: A 33-year-old man noticed reduced control of the right leg in May 2020. During his first clinic appointment at our institution in September 2021, he complained about gait imbalance, fine motor disorders, tremor, intermittent diplopia and slurred speech. He presented a pancerebellar syndrome with stance, gait and limb ataxia, scanning speech and oculomotor dysfunction. Within 3 months the symptoms progressed. An initial cerebral magnetic resonance imaging, June 2020, was normal, but follow-up imaging in October 2021 and July 2022 revealed marked cerebellar atrophy (29% volume loss). Cerebrospinal How to cite this article: Schwarzwald A, Salmen A, León Betancourt AX, et al. Anti-neurochondrin antibody as a biomarker in primary autoimmune cerebellar ataxia-a case report and review of the literature.

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Treatments and Outcomes Among Patients with Sydenham Chorea

Eyre,

Thomas,

Ferrarin

et al. 2024

JAMA Netw Open

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ImportanceSydenham chorea is the most common acquired chorea of childhood worldwide; however, treatment is limited by a lack of high-quality evidence.ObjectivesTo evaluate historical changes in the clinical characteristics of Sydenham chorea and identify clinical and treatment factors at disease onset associated with chorea duration, relapsing disease course, and functional outcome.Data SourcesThe systematic search for this meta-analysis was conducted in PubMed, Embase, CINAHL, Cochrane Library, and LILACS databases and registers of clinical trials from inception to November 1, 2022 (search terms: [Sydenham OR Sydenham’s OR rheumatic OR minor] AND chorea).Study SelectionPublished articles that included patients with a final diagnosis of Sydenham chorea (in selected languages).Data Extraction and SynthesisThis study followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline. Individual patient data on clinical characteristics, treatments, chorea duration, relapse, and final outcome were extracted. Data from patients in the modern era (1945 through 2022) were entered into multivariable models and stratified by corticosteroid duration for survival analysis of chorea duration.Main Outcomes and MeasuresThe planned study outcomes were chorea duration at onset, monophasic course (absence of relapse after ≥24 months), and functional outcome (poor: modified Rankin Scale score 2-6 or persisting chorea, psychiatric, or behavioral symptoms at final follow-up after ≥6 months; good: modified Rankin Scale score 0-1 and no chorea, psychiatric, or behavioral symptoms at final follow-up).ResultsIn total, 1479 patients were included (from 307 articles), 1325 since 1945 (median [IQR] age at onset, 10 [8-13] years; 875 of 1272 female [68.8%]). Immunotherapy was associated with shorter chorea duration (hazard ratio for chorea resolution, 1.51 [95% CI, 1.05-2.19]; P = .03). The median chorea duration in patients receiving 1 or more months of corticosteroids was 1.2 months (95% CI, 1.2-2.0) vs 2.8 months (95% CI, 2.0-3.0) for patients receiving none (P = .004). Treatment factors associated with monophasic disease course were antibiotics (odds ratio [OR] for relapse, 0.28 [95% CI, 0.09-0.85]; P = .02), corticosteroids (OR, 0.32 [95% CI, 0.15-0.67]; P = .003), and sodium valproate (OR, 0.33 [95% CI, 0.15-0.71]; P = .004). Patients receiving at least 1 month of corticosteroids had significantly lower odds of relapsing course (OR, 0.10 [95% CI, 0.04-0.25]; P &lt; .001). No treatment factor was associated with good functional outcome.Conclusions and RelevanceIn this meta-analysis of treatments and outcomes in patients with Sydenham chorea, immunotherapy, in particular corticosteroid treatment, was associated with faster resolution of chorea. Antibiotics, corticosteroids and sodium valproate were associated with a monophasic disease course. This synthesis of retrospective data should support the development of evidence-based treatment guidelines for patients with Sydenham chorea.

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Chorea Minor Associated with Anti-Neurochondrin Autoantibodies

Cited by 15 publications

References 4 publications

Neurochondrin neurological autoimmunity

Neurochondrin neurological autoimmunity

Anti‐neurochondrin antibody as a biomarker in primary autoimmune cerebellar ataxia—a case report and review of the literature

Treatments and Outcomes Among Patients with Sydenham Chorea

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