Anti-IgLON5 Disease: A New Bulbar-Onset Motor Neuron Mimic Syndrome.
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.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Autoanticuerpos
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Moléculas de Adhesión Celular Neuronal
/
Enfermedad de la Neurona Motora
Tipo de estudio:
Diagnostic_studies
/
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Límite:
Aged
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Female
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Humans
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Male
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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
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UNITED STATES
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UNITED STATES OF AMERICA
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US
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USA