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Anti-IgLON5 Disease: A New Bulbar-Onset Motor Neuron Mimic Syndrome.
Werner, Jana; Jelcic, Ilijas; Schwarz, Esther Irene; Probst-Müller, Elisabeth; Nilsson, Jakob; Schwizer, Bernhard; Bloch, Konrad Ernst; Lutterotti, Andreas; Jung, Hans-Heinrich; Schreiner, Bettina.
Afiliación
  • Werner J; From the Department of Neurology (J.W., I.J., A.L., H.-H.J.), Department of Respiratory Medicine and Sleep Disorders Center (E.I.S., K.E.B.), Department of Immunology (E.P.-M., J.N.), University Hospital Zurich; Lindenhofspital (B. Schwizer), Bern; and Department of Neurology and University Zurich I
  • Jelcic I; From the Department of Neurology (J.W., I.J., A.L., H.-H.J.), Department of Respiratory Medicine and Sleep Disorders Center (E.I.S., K.E.B.), Department of Immunology (E.P.-M., J.N.), University Hospital Zurich; Lindenhofspital (B. Schwizer), Bern; and Department of Neurology and University Zurich I
  • Schwarz EI; From the Department of Neurology (J.W., I.J., A.L., H.-H.J.), Department of Respiratory Medicine and Sleep Disorders Center (E.I.S., K.E.B.), Department of Immunology (E.P.-M., J.N.), University Hospital Zurich; Lindenhofspital (B. Schwizer), Bern; and Department of Neurology and University Zurich I
  • Probst-Müller E; From the Department of Neurology (J.W., I.J., A.L., H.-H.J.), Department of Respiratory Medicine and Sleep Disorders Center (E.I.S., K.E.B.), Department of Immunology (E.P.-M., J.N.), University Hospital Zurich; Lindenhofspital (B. Schwizer), Bern; and Department of Neurology and University Zurich I
  • Nilsson J; From the Department of Neurology (J.W., I.J., A.L., H.-H.J.), Department of Respiratory Medicine and Sleep Disorders Center (E.I.S., K.E.B.), Department of Immunology (E.P.-M., J.N.), University Hospital Zurich; Lindenhofspital (B. Schwizer), Bern; and Department of Neurology and University Zurich I
  • Schwizer B; From the Department of Neurology (J.W., I.J., A.L., H.-H.J.), Department of Respiratory Medicine and Sleep Disorders Center (E.I.S., K.E.B.), Department of Immunology (E.P.-M., J.N.), University Hospital Zurich; Lindenhofspital (B. Schwizer), Bern; and Department of Neurology and University Zurich I
  • Bloch KE; From the Department of Neurology (J.W., I.J., A.L., H.-H.J.), Department of Respiratory Medicine and Sleep Disorders Center (E.I.S., K.E.B.), Department of Immunology (E.P.-M., J.N.), University Hospital Zurich; Lindenhofspital (B. Schwizer), Bern; and Department of Neurology and University Zurich I
  • Lutterotti A; From the Department of Neurology (J.W., I.J., A.L., H.-H.J.), Department of Respiratory Medicine and Sleep Disorders Center (E.I.S., K.E.B.), Department of Immunology (E.P.-M., J.N.), University Hospital Zurich; Lindenhofspital (B. Schwizer), Bern; and Department of Neurology and University Zurich I
  • Jung HH; From the Department of Neurology (J.W., I.J., A.L., H.-H.J.), Department of Respiratory Medicine and Sleep Disorders Center (E.I.S., K.E.B.), Department of Immunology (E.P.-M., J.N.), University Hospital Zurich; Lindenhofspital (B. Schwizer), Bern; and Department of Neurology and University Zurich I
  • Schreiner B; From the Department of Neurology (J.W., I.J., A.L., H.-H.J.), Department of Respiratory Medicine and Sleep Disorders Center (E.I.S., K.E.B.), Department of Immunology (E.P.-M., J.N.), University Hospital Zurich; Lindenhofspital (B. Schwizer), Bern; and Department of Neurology and University Zurich I
Neurol Neuroimmunol Neuroinflamm ; 8(2)2021 03 04.
Article en En | MEDLINE | ID: mdl-33531378
ABSTRACT

OBJECTIVE:

To expand the spectrum of anti-IgLON5 disease by adding 5 novel anti-IgLON5-seropositive cases with bulbar motor neuron disease-like phenotype.

METHODS:

We characterized the clinical course, brain MRI and laboratory findings, and therapy response in these 5 patients.

RESULTS:

Patients were severely affected by bulbar impairment and its respiratory consequences. Sleep-related breathing disorders and parasomnias were common. All patients showed clinical or electrophysiologic signs of motor neuron disease without fulfilling the diagnostic criteria for amyotrophic lateral sclerosis. One patient regained autonomy in swallowing and eating, possibly related to immunotherapy.

CONCLUSION:

IgLON5 disease is an important differential diagnosis to evaluate in patients with bulbar motor neuron disease-like phenotype and sleep disorders. There is need for a deeper understanding of the underlying pathobiology to determine whether IgLON5 disease is an immunotherapy-responsive condition.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Autoanticuerpos / Moléculas de Adhesión Celular Neuronal / Enfermedad de la Neurona Motora Tipo de estudio: Diagnostic_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Neurol Neuroimmunol Neuroinflamm Año: 2021 Tipo del documento: Article Pais de publicación: EEUU / ESTADOS UNIDOS / ESTADOS UNIDOS DA AMERICA / EUA / UNITED STATES / UNITED STATES OF AMERICA / US / USA
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Autoanticuerpos / Moléculas de Adhesión Celular Neuronal / Enfermedad de la Neurona Motora Tipo de estudio: Diagnostic_studies / Observational_studies / Prognostic_studies / Risk_factors_studies Límite: Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Neurol Neuroimmunol Neuroinflamm Año: 2021 Tipo del documento: Article Pais de publicación: EEUU / ESTADOS UNIDOS / ESTADOS UNIDOS DA AMERICA / EUA / UNITED STATES / UNITED STATES OF AMERICA / US / USA