and was called “ambiguous sleep” because of its “low phasic atonia with an extreme abundance of twitches and muscular discharges.” The prevalence of RBD in narcolepsy with cataplexy (NC) is high, being clinically evident in 45–61% of patients and polysomnographically detectable in 36–43% of them. RBD was observed in narcoleptic patients even before its first recognition as a clinical entity by Schenck et al. The time lag between the occurrence of RBD and synucleinopathy can span from one to many years or decades. Thus, RBD often heralds synucleinopathies, such as Parkinson disease, Lewy body dementia, and multiple system atrophy.
Patients initially diagnosed with idiopathic RBD often later develop other neurological signs including parkinsonism (most frequently), orthostatic hypotension, anosmia, and cognitive impairment. RBD may be idiopathic or symptomatic and both forms are strongly associated with neurodegerative diseases finally, RBD can be iatrogenic.
Introductionįirst described in 1986, rapid eye movement (REM) sleep behavior disorder (RBD) is a parasomnia characterized by repeated episodes of dream enactment behavior and REM sleep without atonia (RWA), evident during polysomnographic recording and manifested as increased phasic or tonic muscle activity on electromyogram channels. Patients predisposed to RBD and later parkinsonism might be susceptible to a variety of triggers that, in our patient, might have been represented by a possible latent autoimmune process leading to the development of narcolepsy with cataplexy and rheumatoid arthritis, later. Moreover, a late development of parkinsonism and the occurrence of rheumatoid arthritis were detected and clinically and instrumentally characterized. The patient was found to present for almost two decades, in addition to RBD, also narcolepsy. A 78-year-old male patient in whom RBD was first diagnosed was followed-up by clinical examination, video-polysomnography, multiple sleep latency test, cerebral magnetic resonance imaging, and dopamine transporter imaging by single-photon emission computerized tomography. Our peer support groups are popular events where around 10 children and families in particular age bands come together to ask questions, problem solve, and meet others experiencing the same challenges.A patient is reported in whom signs and symptoms of REM sleep behavior disorder (RBD) and narcolepsy have been associated for almost two decades with a late development of parkinsonism and rheumatoid arthritis. Narcolepsy is rare and many children and families might feel they are on their own. This becomes crucial around exam times when our specialist sleep psychology service liaises with schools, to provide tailored information Support groups ManagementĪfter accurately diagnosing the condition, we work with parents and schools to increase an understanding about narcolepsy and its symptoms. We have extensive diagnostic equipment and tests, including inpatient polysomnography and multiple sleep latency testing, as well as HLA and hypocretin measures. We share care with local services, and then transition these young people at 16 years to our adult sleep services. We're a busy service, running clinics for over 120 children with narcolepsy and cataplexy. Typically, the person’s knees buckle and may give way upon laughing, surprise or anger. In other typical cataplectic attacks the head may drop or the jaw may become slack.Ĭataplexy in children is slightly different and a variety of symptoms and movements including tongue protrusion and involuntary arm movements have been described.Īs these symptoms look similar to other neurological movement disorders, it's common to explore a range of other conditions before the diagnosis of narcolepsy with cataplexy becomes more certain. This is a rare sleep disorder with irresistible and unpredictable daytime sleep attacks and a number of other symptoms.Ĭataplexy, unique to narcolepsy, is a sudden episode of muscle weakness triggered by emotions.