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Título

CONTRIBUTION FOR THE NEUROANATOMY OF NARCOLEPSY ASSOACIATED TO THE ATROPHY OF MIDBRAIN IN PROGRESSIVE SUPRANUCLEAR PALSY

Introdução

Narcolepsy type 1 (NT1) is a lifelong sleep disorder affecting 1:2000 individuals. The disease is characterized by excessive daytime sleepiness, cataplexy and other abnormal manifestations of REM sleep, such as sleep paralysis and hypnagogic hallucinations. Recently, it was discovered that the pathophysiology of (idiopathic) narcolepsy-cataplexy is linked to the loss of hypocretinergic neurons in the lateral hypothalamus.(Barateau et al., 2022).

Sleep disorders are important manifestations of neurodegenerative disorders and eventually are clinically evident before the onset of other neuropsychiatric features (Hauw et al., 2011). Symptomatic or secondary narcolepsy refers to the symptoms of narcolepsy occurring during the course of other brain disorders.

Progressive supranuclear palsy (PSP) is an atypical parkinsonism related to 4R tauopathy. Neuropsychiatric symptoms include apathy, depression, anxiety, disinhibition and impulsive behavior (Fabbrini et al., 2019). A wide range of sleep disorders that include insomnia, rapid eye movement sleep behavior disorder, periodic limb movement disorder, excessive daytime sleepiness, and sleep apneas, may complicate the course of the Parkinson plus disorders, but narcolepsy is rarely mentioned (Bhattacharya et al., 2020). Besides that, little is known about the neuropathology of narcolepsy (Hauw et al., 2011).

Objetivo

To describe a patient with narcolepsy associated to focal midbrain atrophy in progressive supranuclear palsy.

Métodos

Design: Case report.
Setting: The Colorado Neurological Institute Movement Disorders Center in Englewood, Colorado, and the Mayo Clinic in Rochester, Minnesota.
Patient: A 62-year-old woman with a neurological disorder characterized by Richardson’s syndrome presented with narcolepsy (excessive daytime sleepiness, cataplexy, and other abnormal manifestations of REM sleep), and Godot’s syndrome in the setting of midbrain atrophy.
Intervention: symptomatic treatment for parkinsonism and sleep disorders.
Main outcome measures: Polysomnography, multiple sleep latency test, and neurological examination and neuroimaging.

Resultados

Treatment with a modafinila improved daytime somnolence.

Conclusões

This is the first report associating narcolepsy with primary PSP (Richardson’s syndrome). Symptomatic treatment of narcolepsy is beneficial even in the presence of midbrain atrophy in PSP

Palavras -chave

Narcolepsy, Progressive Supranuclear Palsy, Hypocretin, midbrain atrophy

Área

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