Clinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies.
Neurol Neuroimmunol Neuroinflamm
; 7(3)2020 05.
Article
en En
| MEDLINE
| ID: mdl-32170042
ABSTRACT
OBJECTIVE:
To describe the main syndrome and clinical course in a large cohort of patients with anti-Ri-associated paraneoplastic neurologic syndrome (Ri-PNS).METHODS:
Twenty-year retrospective nationwide study and systematic review of the literature.RESULTS:
Thirty-six patients with complete clinical information were identified (median age 66 years, range 47-87 years). In this French cohort, the majority were women (78%). At onset, 4 main patterns were observed cerebellar syndrome (39%), isolated tremor (24%), oculomotor disturbances (17%), and other symptoms (19%). Course was multistep for 78% of cases. At the time the disease reached the plateau phase (median 12 weeks, range 1-64 weeks; 28% >3 months), 24 (67%) showed an overt cerebellar syndrome, which was isolated in 3 patients, and was most frequently (21/24 cases) part of a multisystem neurologic disease. Patients manifested a variety of movement disorders, including myoclonus (33%), dystonia (17%), either cervical or oromandibular, and parkinsonism (17%). Most patients had cancer (92%), mainly breast cancer (n = 22). Misdiagnoses concerned 22% of patients (n = 8) and included atypical parkinsonism (n = 2), MS (n = 2), Bickerstaff encephalitis (n = 1), hyperekplexia (n = 1), vestibular neuritis (n = 1), and functional neurologic disorder (n = 1). Survival at 12 months was 73% (95% CI [0.54-0.85]), at 24 months 62% (95% CI [0.41-0.78]), and at 36 months 47% (95% CI [0.25-0.65]). There was no major clinical difference between cases retrieved from the systematic review of the literature (n = 55) and the French cohort.CONCLUSIONS:
Ri-PNS is a multisystem neurologic syndrome with prominent cerebellum/brainstem involvement. Opsoclonus-myoclonus is less common than expected, and the disorder can mimic neurodegenerative diseases.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Proteínas de Unión al ARN
/
Síndromes Paraneoplásicos del Sistema Nervioso
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Trastornos del Movimiento
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Proteínas del Tejido Nervioso
Tipo de estudio:
Diagnostic_studies
/
Etiology_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
/
Systematic_reviews
Límite:
Aged
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Aged80
/
Female
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Humans
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Male
/
Middle aged
País/Región como asunto:
Europa
Idioma:
En
Revista:
Neurol Neuroimmunol Neuroinflamm
Año:
2020
Tipo del documento:
Article
País de afiliación:
India