Narcolepsy type 1 and Sydenham chorea - Report of 3 cases and review of the literature.
Case series
Dyskinesia
Narcolepsy type 1
Streptococcal disease
Sydenham chorea
Journal
Sleep medicine
ISSN: 1878-5506
Titre abrégé: Sleep Med
Pays: Netherlands
ID NLM: 100898759
Informations de publication
Date de publication:
Dec 2023
Dec 2023
Historique:
received:
26
06
2023
revised:
26
10
2023
accepted:
27
10
2023
medline:
30
11
2023
pubmed:
6
11
2023
entrez:
5
11
2023
Statut:
ppublish
Résumé
Narcolepsy type 1 (NT1) is an immune-mediated disorder characterized by excessive daytime sleepiness, cataplexy, low levels of hypocretin-1 in the cerebrospinal fluid, and a strong association with the HLA DQB1*06:02 allele. There is evidence for streptococcal infections as one pathogenic factor that may lead to NT1 as part of a multifactorial pathogenesis. Elevated titers of Antistreptolysin-O antibodies and increased inflammatory activity in response to streptococci antigens have been described in patients with NT1. Sydenham chorea (SC) results from a post-streptococcal autoimmune process targeting basal ganglia neurons. Despite this common trigger, SC has been interpreted as a misdiagnosis in a few described cases of patients who were first diagnosed with SC and later with NT1. Our goal was to analyze the association between SC and NT1. We reviewed the literature and report three patients from three European sleep centers who were diagnosed with both SC and NT1 within a few months. We describe the cases of one male (age 10) and two female (age 22 and 10) patients. We argue that in those cases both diagnoses are justified, unlike reports of previous cases in which SC was considered a misdiagnosis in patients with NT1. It remains, however, unclear if the conditions occur independently or if there is an overlap disorder- an SC-like subtype of narcolepsy with a particular sequence of symptoms. Further studies need to clarify the causality of the relationship and the pathophysiology of the reported rare association.
Sections du résumé
OBJECTIVES/BACKGROUND
OBJECTIVE
Narcolepsy type 1 (NT1) is an immune-mediated disorder characterized by excessive daytime sleepiness, cataplexy, low levels of hypocretin-1 in the cerebrospinal fluid, and a strong association with the HLA DQB1*06:02 allele. There is evidence for streptococcal infections as one pathogenic factor that may lead to NT1 as part of a multifactorial pathogenesis. Elevated titers of Antistreptolysin-O antibodies and increased inflammatory activity in response to streptococci antigens have been described in patients with NT1. Sydenham chorea (SC) results from a post-streptococcal autoimmune process targeting basal ganglia neurons. Despite this common trigger, SC has been interpreted as a misdiagnosis in a few described cases of patients who were first diagnosed with SC and later with NT1. Our goal was to analyze the association between SC and NT1.
PATIENTS/METHODS
METHODS
We reviewed the literature and report three patients from three European sleep centers who were diagnosed with both SC and NT1 within a few months.
RESULTS
RESULTS
We describe the cases of one male (age 10) and two female (age 22 and 10) patients.
CONCLUSIONS
CONCLUSIONS
We argue that in those cases both diagnoses are justified, unlike reports of previous cases in which SC was considered a misdiagnosis in patients with NT1. It remains, however, unclear if the conditions occur independently or if there is an overlap disorder- an SC-like subtype of narcolepsy with a particular sequence of symptoms. Further studies need to clarify the causality of the relationship and the pathophysiology of the reported rare association.
Identifiants
pubmed: 37925849
pii: S1389-9457(23)00406-9
doi: 10.1016/j.sleep.2023.10.028
pii:
doi:
Substances chimiques
Orexins
0
Types de publication
Review
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
234-238Informations de copyright
Copyright © 2023. Published by Elsevier B.V.
Déclaration de conflit d'intérêts
Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Elena S. Wenz reports a relationship with University of Bern that includes: funding grants. Ulf Kallweit reports a relationship with Bioprojet, Jazz Pharmaceuticals, Takeda Pharmaceuticals that includes: consulting or advisory and speaking and lecture fees. Rolf Fronczek reports a relationship with Takeda Pharmaceuticals, Bioprojet that includes: consulting or advisory. Rolf Fronczek reports a relationship with Jazz Pharmaceuticals that includes: funding grants. Gert Jan Lammers reports a relationship with Bioprojet, Jazz Pharmaceuticals, Takeda Pharmaceuticals, NLS Pharmaceutics that includes: consulting or advisory and speaking and lecture fees.