Cases reported "Narcolepsy"

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1/3. narcolepsy in the older adult: epidemiology, diagnosis and management.

    narcolepsy is a disorder of impaired expression of wakefulness and rapid-eye-movement (REM) sleep. This manifests as excessive daytime sleepiness and expression of individual physiological correlates of REM sleep that include cataplexy and sleep paralysis (REM sleep atonia intruding into wakefulness), impaired maintenance of REM sleep atonia (e.g. REM sleep behaviour disorder [RBD]), and dream imagery intruding into wakefulness (e.g. hypnagogic and hypnopompic hallucinations). Excessive sleepiness typically begins in the second or third decade followed by expression of auxiliary symptoms. Only cataplexy exhibits a high specificity for diagnosis of narcolepsy. While the natural history is poorly defined, narcolepsy appears to be lifelong but not progressive. Mild disease severity, misdiagnoses or long delays in cataplexy expression often cause long intervals between symptom onset, presentation and diagnosis. Only 15-30% of narcoleptic individuals are ever diagnosed or treated, and nearly half first present for diagnosis after the age of 40 years. attention to periodic leg movements (PLM), sleep apnoea and RBD is particularly important in the management of the older narcoleptic patient, in whom these conditions are more likely to occur. diagnosis requires nocturnal polysomnography (NPSG) followed by multiple sleep latency testing (MSLT). The NPSG of a narcoleptic patient may be totally normal, or demonstrate the patient has a short nocturnal REM sleep latency, exhibits unexplained arousals or PLM. The MSLT diagnostic criteria for narcolepsy include short sleep latencies (<8 minutes) and at least two naps with sleep-onset REM sleep. Treatment includes counselling as to the chronic nature of narcolepsy, the potential for developing further symptoms reflective of REM sleep dyscontrol, and the hazards associated with driving and operating machinery. Elderly narcoleptic patients, despite age-related decrements in sleep quality, are generally less sleepy and less likely to evidence REM sleep dyscontrol. Nonpharmacological management also includes maintenance of a strict wake-sleep schedule, good sleep hygiene, the benefits of afternoon naps and a programme of regular exercise. Thereafter, treatment is highly individualised, depending on the severity of daytime sleepiness, cataplexy and sleep disruption. Wake-promoting agents include the traditional psychostimulants. More recently, treatment with the 'activating' antidepressants and the novel wake-promoting agent modafinil has been advocated. cataplexy is especially responsive to antidepressants which enhance synaptic levels of noradrenaline (norepinephrine) and/or serotonin. Obstructive sleep apnoea and PLMs are more common in narcolepsy and should be suspected when previously well controlled older narcolepsy patients exhibit a worsening of symptoms. The discovery that narcolepsy/cataplexy results from the absence of neuroexcitatory properties of the hypothalamic hypocretin-peptidergic system will significantly advance understanding and treatment of the symptom complex in the future.
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2/3. Diagnostic ambiguities in a case of post-traumatic narcolepsy with cataplexy.

    narcolepsy arising from trauma can present particular problems of differential diagnosis. In this case study presentation the patient suffered a head trauma, without unconsciousness, and began to experience unusual episodic behaviours. Symptom presentation differed from the typical clinical manifestations of idiopathic narcolepsy leading to an 8-year search for a definitive diagnosis. Key relevant aspects that led to diagnostic ambiguities were the order of symptom development, negative for the antigen HLA DR2, significance of the Multiple Sleep Latency Test (MSLT) mean sleep latency versus number of sleep onset rapid eye movement periods, the somewhat atypical features of cataplexy, the coexistence of sleep apnoea, and the mildness of the original head injury. It is argued that cases of post-traumatic narcolepsy should be considered in the context of their clinical development over time and that practitioners should be aware that this form of narcolepsy can differ from the typical clinical history of idiopathic narcolepsy.
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3/3. narcolepsy in the custodial setting.

    A case of a detained person who suffered from narcolepsy is presented. The management of the condition in general and with particular reference to the custodial setting is discussed. The management of obstructive sleep apnoea, the commonest sleep disorder, is contrasted. Forensic physicians are often confronted with rare or unusual medical problems in the custodial setting, often having to make immediate medical decisions without access to specialist medical advice. Twenty-four hour access to internet medical resources in the custodial setting may be helpful.
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