Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013421 (dystonia)
 8,418 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Wakefulness and sleep are antagonistic states competing for the domain of brain activity. Non-REM sleep and REM sleep are different states of being, sustained by activity in brainstem nuclei, hypothalamus, basal forebrain, and thalamus. Such complex phenomenology is subject to many alterations grouped in the new International Classification of Sleep Disorders. The insomnias are the result of interacting psychosocial, psychophysiologic, neurodevelopmental, and medical factors. Proper perspective of each factor provides the clinical strategies to approach medically the symptom-complex of insomnia. The most common cause of daytime hypersomnia is chronic sleep deprivation. Obstructive sleep apnea responds to nasal CPAP, but the failure rate approaches 30%. In intolerant patients BiPAP and surgical remedies should be considered. Motor and behavioral abnormalities of sleep may be linked to REM sleep as in the REM sleep behavior disorder. Paroxysmal nocturnal dystonia and nocturnal wanderings may be associated with epilepsy. Intrusions of one state of being (wakefulness, non-REM sleep, and REM sleep) into another result in mixed, poorly defined, or only partially developed states. Dissociation of states may be responsible for confusional arousals, hallucinations, and cateplexy. Senile degeneration of the suprachiasmatic nuclei may underlie the circadian rhythm changes in old age and the "sundown" syndrome in demented patients. Misalignment of the hypothalamic pacemaker causes dysregulation of sleep-related physiologic and behavioral variables. Exposure to bright light retrains the pacemaker in night-shift workers, transmeridian travelers, and in patients with seasonal affective syndrome. Benzodiazepine compounds are very effective hypnotics, but should be used sparingly in the elderly to avoid falls, memory lapses, and aggravation of a preexisting sleep apnea syndrome. Sleep laboratory evaluations are indicated in patients with hypersomnia, suspected sleep apnea syndrome, motor-behavioral disorders of sleep, and in many individuals complaining of insomnia.
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PMID:Update on disorders of sleep and the sleep--wake cycle. 160 36

The features of sleep parameters in the Rett syndrome were compared with those in early infantile autism (EIA) and hereditary progressive dystonia with marked diurnal fluctuation (HPD). The sleep-wakefulness cycle and the tonic and phasic components of sleep were evaluated in each disorder, the former was estimated by the day-by-day plot method and the latter two by polysomnography (PSG) following our method. Abnormalities of the sleep-wakefulness cycle were observed in the Rett syndrome and EIA, but in the latter these abnormalities became inapparent with age and improved markedly by correcting the environmental condition and completely by 5-hydroxytriptophan. The latter, if treated early, was followed by improvement of behavior. In the Rett syndrome, however, the abnormalities continued into late childhood to adolescence. In HPD, PSG abnormalities were restricted to the phasic component, which improved completely after levodopa in accordance with the clinical improvement. On the other hand, in the Rett syndrome as well as in EIA both the phasic and tonic components were involved and also the leakage of the components of REM stage into NREM stage was observed. In the Rett syndrome, these abnormalities aggravated with age, with disturbances in % sleep stage, nocturnal variation of tonic and phasic components of sleep and REM-NREM cycles, while in EIA the results of PSGs revealed no such progressions but showed an increase in twitch movement and a lack of normal increase in the number of REMs occurring in short intervals.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Polysomnography in the Rett syndrome. 162 34

A nocturnal polygraphic study was performed on 10 patients with cranial dystonia (blepharospasm (BS) and oromandibular dystonia (OMD)). All patients showed impaired sleep efficiency and reduced slow and REM sleep, more marked in subjects with severe dystonia. Abnormal muscular activity decreased progressively with deeper sleep and during the first hours of the night, without disappearing. A disordered hypnic++ pattern and impaired motor control even during sleep are typical features in cranial dystonia.
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PMID:Sleep and cranial dystonia. 171 8

The epileptic or nonepileptic origin of nocturnal paroxysmal dystonia (NPD) has been debated. We studied three patients with frequent attacks during non-REM sleep. During prolonged video-EEG monitoring, two patients had a convulsive seizure after a typical NPD episode and on these occasions EEG showed epileptiform discharge. In the three patients, attacks occurred repeatedly with different intensity, representing "fragments" of the same seizure. These fragments of the attack could occur periodically every 20-40 s. We postulate that short NPD attacks are actually epileptic seizures originating from the frontal lobes. The rhythmicity of the episodes may be due to rhythmic oscillation of cortical function during non-REM sleep.
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PMID:Nocturnal paroxysmal dystonia with short-lasting attacks: three cases with evidence for an epileptic frontal lobe origin of seizures. 240 Dec 46

It is known that EEG findings reveal various abnormalities in patients with involuntary movement. But these findings are not specific. It has been reported to be related to myoclonus and spike. The other involuntary movement is unknown to the relation to EEG findings. The involuntary movement usually disappears during sleep, but a certain involuntary movement appears only during sleep. In the patient with Huntington's chorea and dystonia musculorum deformans, PSG reveals an increase in interspersed wakefulness, decrease of deep sleep and prolongation of REM latency. Periodic limb movement and nocturnal paroxysmal dystonia appear only during sleep. Nocturnal sleep studies are important for exploring the pathophysiology in involuntary movement.
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PMID:[EEG and polysomnography findings in patients with dyskinesia]. 827 65

Paroxysmal dyskinesias are intermittent attacks of involuntary hyperkinetics abnormal movements. Among paroxysmal dyskinesias were individualized three entities: paroxysmal kinesigenic choreoathetosis, paroxysmal choreoathetosis of Mount and Reback, hypnogenic paroxysmal dystonia. New classifications are based upon the circumstances of occurrence, the duration of attacks and their etiology. We report here two observations of idiopathic non familial paroxysmal dyskinesias in three-year-old children. Both were seen first in consultation for falls and nocturnal motor agitation. The attacks were paroxysmal jerky "puppet-like" movements lasting from 20 seconds to 15 minutes. They could occur during non REM sleep, during the day, at rest, after a sudden movement, or during prolonged exercise. Carbamazepine was inefficient. These cases were not classifiable according to the classical criteria and could constitute a new entity. Moreover, some sleep-EEG showed abnormal patterns (frontal rapid rhythms, central spikes in one case) and led us to discuss the pathophysiology of this episodic movements disorder, and its relation with frontal partial epilepsy.
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PMID:[Diurnal and nocturnal paroxysmal dyskinesia in young children: a new entity?]. 929 45

The nature of sleep is one of the major sources of dissatisfaction with the quality of life among patients with Parkinson's disease (PD). Difficult sleep maintenance (light and fragmented sleep) and difficulties with sleep initiation are the earliest and most frequent sleep disorders observed in these patients. Sleep disorders are also common in the normal elderly population, suggesting that normal aging may play a role in the etiology of sleep disorders in PD. Factor et al. examined the frequency of various sleep disorders in PD and compared them to those of normal elderly subjects. Sleep fragmentation and spontaneous daytime dozing occurred much more frequently in PD patients than in controls. Sleep fragmentation in PD may be due to an increased skeletal muscle activity, disturbed breathing and REM/non-REM variations of the dopaminergic receptor sensitivity. In parkinsonian patients who developed motor fluctuations (on-off phenomenon, wearing-off) during the day, other common sleep-related motor complaints including nocturnal akinesia, dystonia and painful cramps are observed. In a double-blind cross-over study, we compared the efficacy of a single dose of a chronic release formulation of levodopa/carbidopa (Sinemet CR) with that of a placebo in improving sleep-related motor disturbances in a group of 40 fluctuating PD patients. Sinemet CR significantly improved nocturnal akinesia and increased the hours of sleep in this group of patients. Initiation and maintenance of sleep are problems that may not be solved with antiparkinsonian treatment.
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PMID:Sleep disorders in Parkinson's disease. 961 17

High-frequency stimulation of the subthalamic nucleus (STN) was used to investigate the relationship of sleep disorders with motor handicap in PD. In 10 insomniac patients with PD, stimulation reduced nighttime akinesia by 60% and completely suppressed axial and early morning dystonia, but did not alleviate periodic leg movements (n = 3) or REM sleep behavior disorders (n = 5). Total sleep time increased by 47%; wakefulness after sleep onset decreased by 51 minutes. Insomnia in patients with PD may predominantly result from nighttime motor disability.
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PMID:Improvement of sleep architecture in PD with subthalamic nucleus stimulation. 1111 33

Nocturnal disturbances are common in Parkinson's disease (PD) patients, with almost 70% of these patients reporting nocturnal disturbances. The etiology of sleep disturbances in patients with PD is still controversial. They might be dependent on dopaminergic drugs, on disease progression, or on a combination of these two factors. Nocturnal disturbances can be categorized in four groups: 1) PD-related motor symptoms, including nocturnal akinesia, early-morning dystonia, painful cramps, tremor, and difficulty turning in bed; 2) treatment-related nocturnal disturbances; 3) psychiatric symptoms, including hallucinations, vivid dreams, depression, dementia, insomnia, psychosis, and panic attacks; 4) other sleep disorders, including insomnia, REM behavioral disorder (RBD), restless legs syndrome (RLS), periodic leg movements (PLMS), and excessive daytime sleepiness (EDS). Specific treatment options are supplied for every group. A global evaluation of nocturnal disturbances would provide clinicians with a valuable tool to establish an optimal regimen that could positively influence all nocturnal disturbance categories and thus improve PD management on. However, it is important to consider that management of some nocturnal disturbances in a group may worsen nocturnal symptoms of another group or may increase EDS. PD-related symptoms can be treated with long-acting DA agonists to obtain continuous DA receptor stimulation during the night. Both treatment-related nocturnal disturbances and psychiatric symptoms may be related to drug treatment, and therefore, in both cases, drug reduction or discontinuance should be considered. Some sleep disorders, such as RLS and PLMS, may be controlled by DA agents, and others, such as insomnia and EDS, may be improved by reducing dopaminergic stimulation.
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PMID:Treatment of nocturnal disturbances and excessive daytime sleepiness in Parkinson's disease. 1550 42

Sleep disorders often occur in patients with epilepsy. Every neurologist is familiar with the postictal drowsiness after tonicoclonic convulsions and likewise with the provocation of an attack after sleep deprivation that is often combined with alcohol consumption. It is more difficult to differentiate between motor disturbances and epileptic episodes during sleep. For example, nocturnal paroxysmal dystonia, which are frequently an expression of frontal lobe epilepsy, are long not recognized as non-epileptic, sleep-associated attacks. In addition, nocturnal episodes in context of a REM sleep behavioral disturbance can occasionally be differentiated from frontal lobe epileptic seizures only with great difficulty. A clear differential diagnostics between epileptic and non-epileptic attacks during sleep is, however, a requirement for a selective treatment.
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PMID:[Epilepsy and sleep disorders]. 1596 74


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