Low CD40L levels and relative lymphopenia in narcoleptic patients

Coelho, Fernando Morgadinho Santos; Pradella-Hallinan, Márcia; Pedrazzoli, Mário; Soares, Carlos Augusto Senne; Fernandes, Gustavo Bruniera Peres; Murray, Brian J.; Tufik, Sérgio; Bittencourt, Lia

doi:10.1016/j.humimm.2011.05.024

Cited by 6 publications

(3 citation statements)

References 34 publications

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“…Genetic associations [10], [11], [12], [13], [14], [15], [16], presence of anti-tribbles 2 antibodies [17], [18], [19] and other recent observations [20], [21] suggest that autoimmune mechanisms are involved in the etiopathogenesis of narcolepsy [22], [23], [24]. Seasonality of onset [25] and reports of an association to preceding streptococcal infections [26], [27] have suggested a link with upper respiratory tract infections.…”

Section: Introductionmentioning

confidence: 93%

Increased Incidence and Clinical Picture of Childhood Narcolepsy following the 2009 H1N1 Pandemic Vaccination Campaign in Finland

et al. 2012

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BackgroundNarcolepsy is a rare neurological sleep disorder especially in children who are younger than 10 years. In the beginning of 2010, an exceptionally large number of Finnish children suffered from an abrupt onset of excessive daytime sleepiness (EDS) and cataplexy. Therefore, we carried out a systematic analysis of the incidence of narcolepsy in Finland between the years 2002–2010.MethodsAll Finnish hospitals and sleep clinics were contacted to find out the incidence of narcolepsy in 2010. The national hospital discharge register from 2002 to 2009 was used as a reference.FindingsAltogether 335 cases (all ages) of narcolepsy were diagnosed in Finland during 2002–2009 giving an annual incidence of 0.79 per 100 000 inhabitants (95% confidence interval 0.62–0.96). The average annual incidence among subjects under 17 years of age was 0.31 (0.12–0.51) per 100 000 inhabitants. In 2010, 54 children under age 17 were diagnosed with narcolepsy (5.3/100 000; 17-fold increase). Among adults ≥20 years of age the incidence rate in 2010 was 0.87/100 000, which equals that in 2002–2009. Thirty-four of the 54 children were HLA-typed, and they were all positive for narcolepsy risk allele DQB1*0602/DRB1*15. 50/54 children had received Pandemrix vaccination 0 to 242 days (median 42) before onset. All 50 had EDS with abnormal multiple sleep latency test (sleep latency <8 min and ≥2 sleep onset REM periods). The symptoms started abruptly. Forty-seven (94%) had cataplexy, which started at the same time or soon after the onset of EDS. Psychiatric symptoms were common. Otherwise the clinical picture was similar to that described in childhood narcolepsy.InterpretationA sudden increase in the incidence of abrupt childhood narcolepsy was observed in Finland in 2010. We consider it likely that Pandemrix vaccination contributed, perhaps together with other environmental factors, to this increase in genetically susceptible children.

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Section: Introductionmentioning

confidence: 93%

Increased Incidence and Clinical Picture of Childhood Narcolepsy following the 2009 H1N1 Pandemic Vaccination Campaign in Finland

et al. 2012

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“…27,28 The association between narcolepsy and the TCR α locus, as well as the decrease in the concentration of soluble CD40 ligand (CD40L) in patients with narcolepsy, are strong indications of an autoimmune mechanism. 21,29 A recent publication reported the loss of specific immunological protection in the affected twin in a genome study of discordant twins. 30 The immunological theory would explain cell loss in the lateral hypothalamus in patients with narcolepsy due to self-harm due to an imbalance in the immune complex formed by TCR, HLA, and CD40L.…”

Section: Pathophysiological Hypotheses Of Narcolepsymentioning

confidence: 99%

“…30 The immunological theory would explain cell loss in the lateral hypothalamus in patients with narcolepsy due to self-harm due to an imbalance in the immune complex formed by TCR, HLA, and CD40L. 17,21,29 Furthermore, patients with narcolepsy with cataplexy have more specific tribbles homolog two antibodies, which, although present in all patients with narcolepsy, would be more prevalent in the initial phase of the disease. 31 The production of anti-hypocretin-2 receptor antibodies has also been described in patients who developed postvaccination narcolepsy.…”

Section: Pathophysiological Hypotheses Of Narcolepsymentioning

confidence: 99%

Narcolepsy: an interface among neurology, immunology, sleep, and genetics

Coelho

2024

Arq Neuropsiquiatr

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Narcolepsy is a primary disorder of the central nervous system resulting from genetic, environmental, and immunological interactions defined as excessive daytime sleepiness plus cataplexy, hallucinations, sleep paralysis, and sleep fragmentation. The pathophysiology is not entirely known, but the interaction among genetic predisposition, environmental exposition, and immune component with consequent hypocretin-1 deficiency is the model to explain narcolepsy type I. The mechanism of narcolepsy type II is less understood. There is a delay of over ten years for the diagnosis of narcolepsy around the world. Patients with narcolepsy have many comorbidities with a negative impact on quality of life. The treatment of narcolepsy must contain an educational approach for the family, coworkers, and patients. Scheduled naps and sleep hygiene are essential to minimize the dose of medications. Much progress has been seen in the pharmacological treatment of narcolepsy with new stimulants, different presentations of oxybate, and recent studies with orexin agonists. Narcolepsy is a rare disease that needs to be more understood and highlighted to avoid delayed diagnosis and severe disabilities in patients.

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Sleep Disorders: Identifying Biomarkers and Clinical Applications

Frange,

Coelho

2023

Biomarkers in Neuropsychiatry

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Low CD40L levels and relative lymphopenia in narcoleptic patients

Cited by 6 publications

References 34 publications

Increased Incidence and Clinical Picture of Childhood Narcolepsy following the 2009 H1N1 Pandemic Vaccination Campaign in Finland

Increased Incidence and Clinical Picture of Childhood Narcolepsy following the 2009 H1N1 Pandemic Vaccination Campaign in Finland

Narcolepsy: an interface among neurology, immunology, sleep, and genetics

Sleep Disorders: Identifying Biomarkers and Clinical Applications

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