Augmentation may be managed through a combination of behavioral strategies (walking and other physical activities) and medication-timing strategies.7,102 ABT-378 narcolepsy Westphal described the first unequivocal case of narcolepsy in 1877, and Gelineau coined the term narcolepsy in 1880. The prevalence of narcolepsy in the United States is 1/2000.3,118 Narcolepsy is a neurological disorder that affects men and women equally, with usual age of onset between
15 and 30 years. It is characterized by the following tetrad of symptoms: excessive daytime somnolence (EDS), which can be a continuous feeling of sleepiness or “sleep attacks,” cataplexy, hypnagogic or hypnapompic hallucinations, and sleep paralysis.4,118-125 Inhibitors,research,lifescience,medical Guilleminault et al reported that EDS alone or in combination with sleep paralysis or hypnagogic hallucinations is the initial symptom in 90% of patients and that 5% to 8% of patients present with cataplexy.126,127 Inhibitors,research,lifescience,medical Only 10% of patients experience the full tetrad.119 After
onset, EDS persists daily, although it can fluctuate during the day in a stereotyped individual pattern. Attention fluctuates modulated by situational circumstances. The attack usually starts with drowsiness associated with blurry or double vision Inhibitors,research,lifescience,medical and usually lasts for less than 20 min. Sleepiness is often relieved by a sleep attack, but the relief lasts for only several hours. Cataplexy involves sudden bilateral atonia of striated muscles with partial or complete weakness that is brought on by emotion or excitement.
Laughter is the most typical trigger and, less frequently, anger or surprise. Other triggers include anticipation of something special or hilarious, attempts at bantering, feeling Inhibitors,research,lifescience,medical amused, or immobility in response to a call for immediate action. The patient’s “state” and circumstances also influence whether an attack occurs: sleep deprivation or strong feeling of sleepiness can lower the attack threshold. The attacks Inhibitors,research,lifescience,medical start abruptly, but take several seconds to reach their maximum, with most attacks lasting less than a minute. During partial attacks, the knees may give whatever way and there may be sagging of the jaw, inclination of the head, and weakness of the muscles responsible for speech so that the patient is either unable to speak or has slurred speech. Even with severe attacks, eye movements and respiration are spared. Neurological examination during the attack shows atonia, loss of tendon reflexes, and extensor plantar responses. Prolonged episodes may be associated with hallucinations and rarely, “status cataplexies.” Video-polygraphic analysis of cataplectic attacks demonstrate three phases: (i) initial phase, consisting of arrest of eye movements and phasic, massive, inhibitory muscular events; (ii) falling phase, characterized by a rhythmic pattern of suppressions and enhancements of muscular activity leading to the fall; and (iii) atonic phase, associated with complete muscle atonia.