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Schwalmstadt, Germany

Mayer G.,Hephata Klinik
Nervenarzt | Year: 2016

Knowledge of the physiology of sleep-wake regulation can contribute to an understanding of the pathophysiology and symptoms of neurological diseases and is helpful for initiating specific therapies for sleep-wake cycle stabilization. Based on historically important observations on the close relationship between sleep and neurological diseases, new insights and developments in selected neurological entities are presented in this review article. © 2016, Springer-Verlag Berlin Heidelberg. Source

Mayer G.,Hephata Klinik
Expert Review of Neurotherapeutics | Year: 2012

Narcolepsy is a life-long neurodegenerative disorder that causes considerable impairment to quality of life. Until the 1970s, the treatment for one of the main symptoms, excessive daytime sleepiness, was restricted to stimulants, whereas the second core symptom, cataplexy, was treated with antidepressants, and the resultant fragmented night-time sleep with hypnotics. Sodium oxybate (Xyrem®, UCB Pharma, Brussels, Belgium) is an efficacious drug for all three symptoms which improves the quality of life of narcoleptic patients. Owing to its metabolic pathway, there is very little pharmacokinetic interaction with other drugs. In combination with modafinil, some of its therapeutic benefits are enhanced. Adverse events and side effects are moderate when taken according to indication and as recommended. Essential limitations have to be considered before starting the treatment (sleep-related breathing disorders, alcohol intake, hypnotic and sedative comedication, and epilepsy). This article gives an overview of sodium oxybate, which has been US FDA approved for the treatment of cataplexy and excessive daytime sleepiness in patients with narcolepsy, and EMA approved for the treatment of narcolepsy-cataplexy. © 2012 Expert Reviews Ltd. Source

Mayer G.,Hephata Klinik
Nervenarzt | Year: 2014

Narcolepsy is a rare sleep disorder. The classical presentation includes the four symptoms excessive daytime sleepiness, cataplexy, sleep paralysis and hypnagogic hallucinations. As a model disease with all the transitions from awake to sleeping conditions, non-rapid eye movement (NREM) and rapid eye movement (REM), it plays an important role in neurology and sleep medicine. Patients with narcolepsy possess a reduced number of hypocretin-producing neurons in the hypothalamus and accordingly the hypocretin level in the cerebro spinal fluid is low. The neuropeptide hypocretin (orexin) has functions, such as the regulation of the sleep-wake cycle, the autonomous nerve system, motor system and metabolic processes. The delay in diagnosing narcolepsy is difficult to comprehend in modern medicine. The frequent association with other sleep-wake disorders may be responsible for the delay. Genomewide association studies have subsequently been able to prove that autoimmune mechanisms are responsible for the manifestation of narcolepsy with the HLA association being the most important for susceptibility and protection. Imaging studies have revealed changes, making a multifactorial etiopathogenesis probable. The frequent occurrence of metabolic disorders has not yet been clarified. Early diagnosis of narcolepsy has the possibility to offer affected persons an adequate medication to lead an almost normal life and the future possibility to cure narcolepsy through immunomodulation therapy. © Springer-Verlag 2014. Source

Mayer G.,Hephata Klinik
Somnologie | Year: 2013

Kleine-Levin syndrome (KLS) is a rare sleep disorder. Most of the published studies have included only small numbers of patients. Therapeutic and diagnostic studies have the lowest evidence level. This study is a retrospective study including 15 KLS patients of the past 19 years. The patients had first symptoms in their early teens triggered by sleep deprivation, infections, and alcohol intake. Frequency ranged from 2-16/year, duration from 1-6 weeks. Liver enzymes, blood count, inflammatory markers, and magnetic resonance images were normal in all patients. Cerebral spinal fluid (CSF) hypocretin-1 was assessed in 6 patients. It was at the lower normal range in 2 patients and < 130 pg/ml in 2 other patients during an asymptomatic phase, while it was low in 2 patients (96-123 pg/ml) and normal in another patient during a symptomatic phase. Treatment with lithium was established in almost all patients and resulted in marked reduction or cessation of symptomatic episodes. The longest observation was 19 years with a relapse after cessation of lithium. Assessment of CSF hypocretin-1 during and outside of a symptomatic phase is recommended in the diagnostic workup of KLS; however, so far only the clinical course is helpful in establishing the diagnosis. Early treatment is recommended and has been found to be effective in the long course of the disease. © 2013 Springer-Verlag Berlin Heidelberg. Source

Mayer G.,Hephata Klinik | Jennum P.,Copenhagen University | Riemann D.,Albert Ludwigs University of Freiburg | Dauvilliers Y.,French Institute of Health and Medical Research
Sleep Medicine Reviews | Year: 2011

The objective of this review is to highlight the impact of insomnia in central neurological disorders by providing information on its prevalence and give recommendations for diagnosis and treatment. Insomnia in neurological disorders is a frequent, but underestimated symptom. Its occurrence may be a direct consequence of the disease itself or may be secondary to pain, depression, other sleep disorders or the effects of medications. Insomnia can have a significant impact on the patient's cognitive and physical function and may be associated with psychological distress and depression. Diagnosis of insomnia is primarily based on medical history and validated questionnaires. Actigraphy is a helpful diagnostic tool for assessing the circadian sleep-wake rhythm. For differential diagnosis and to measure the duration of sleep full polysomnography may be recommended. Prior to initiating treatment the cause of insomnia must be clearly identified. First line treatment aims at the underlying neurologic disease. The few high quality treatment studies show that short term treatment with hypnotics may be recommended in most disorders after having ruled out high risk for adverse effects. Sedating antidepressants may be an effective treatment for insomnia in stroke and Parkinson's disease (PD) patients. Melatonin and light treatment can stabilize the sleep-wake circadian rhythm and shorten sleep latency in dementias and PD. Cognitive behavioral therapy (CBT) can be effective in treating insomnia symptoms associated with most of the central neurological diseases. The prevalence and treatment of insomnia in neurological diseases still need to be studied in larger patient groups with randomized clinical trials to a) better understand their impact and causal relationship and b) to develop and improve specific evidence-based treatment strategies. © 2011 Elsevier Ltd. Source

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