Abstract

Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, and sleep-onset rapid eye movement (REM) sleep periods. It is now identified as a neurodegenerative disease because there is a massive loss of specific neurons in the brain. These neurons contain the neuropeptides hypocretin-1 and hypocretin-2, which are also known as orexin-A and orexin-B. Cerebrospinal fluid hypocretin-1 measurements are diagnostic for primary narcolepsy. The cause of neural loss could be autoimmune since most patients have the HLA DQB1 0602 allele that predisposes to the disorders. The discovery of hypocretin deficiency is redefining the clinical entity of narcolepsy and offering novel diagnostic procedures. This article reviews the current understanding of narcolepsy and discusses the implications of hypocretin discovery.

Highlights

  • a sleep disorder characterized by excessive daytime sleepiness

  • It is now identified as a neurodegenerative disease because there is a massive loss

  • which are also known as orexin-A and orexin-B. Cerebrospinal fluid hypocretin-1 measurements are diagnostic for primary narcolepsy

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Summary

Hypocretin system and narcolepsy

Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, and sleep-onset rapid eye movement (REM) sleep periods It is identified as a neurodegenerative disease because there is a massive loss of specific neurons in the brain. La narcolepsia (Tabla 1), también conocida como síndrome de Gelineau o epilepsia del sueño, se define como una enfermedad neurológica caracterizada por excesiva somnolencia diurna, transiciones prematuras de la vigila al sueño de movimientos oculares rápidos (MOR; parálisis de sueño), cataplejia (períodos en los cuales el individuo presenta pérdida bilateral del tono muscular sin pérdida de la conciencia), alucinaciones hipnagógicas (visiones fugaces en la transición vigilia-sueño) o hipnopómpicas (transición sueño-vigilia), y sueño nocturno fragmentado[1]. B. Una historia de cataplejia definida como episodios repentinos y transitorios de pérdida del tono muscular desencadenado por emociones. Adicionalmente, la determinación de hipocretina en líquido cerebroespinal se ha añadido como prueba de utilidad diagnóstica dada su alta sensibilidad y especificidad para la narcolepsia[8]

FISIOPATOLOGÍA DE LA NARCOLEPSIA
NARCOLEPSIA Y AUTOINMUNIDAD
Findings
SISTEMA HIPOCRETINÉRGICO
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