REM Sleep Behavior Disorder: A disorder characterized by episodes of vigorous and often violent motor activity during REM sleep (SLEEP, REM). The affected individual may inflict self injury or harm others, and is difficult to awaken from this condition. Episodes are usually followed by a vivid recollection of a dream that is consistent with the aggressive behavior. This condition primarily affects adult males. (From Adams et al., Principles of Neurology, 6th ed, p393)Sleep, REM: A stage of sleep characterized by rapid movements of the eye and low voltage fast pattern EEG. It is usually associated with dreaming.Sleep-Wake Transition Disorders: Parasomnias characterized by behavioral abnormalities that occur during the transition between wakefulness and sleep (or between sleep and wakefulness).Clonazepam: An anticonvulsant used for several types of seizures, including myotonic or atonic seizures, photosensitive epilepsy, and absence seizures, although tolerance may develop. It is seldom effective in generalized tonic-clonic or partial seizures. The mechanism of action appears to involve the enhancement of GAMMA-AMINOBUTYRIC ACID receptor responses.Dreams: A series of thoughts, images, or emotions occurring during sleep which are dissociated from the usual stream of consciousness of the waking state.Polysomnography: Simultaneous and continuous monitoring of several parameters during sleep to study normal and abnormal sleep. The study includes monitoring of brain waves, to assess sleep stages, and other physiological variables such as breathing, eye movements, and blood oxygen levels which exhibit a disrupted pattern with sleep disturbances.Narcolepsy: A condition characterized by recurrent episodes of daytime somnolence and lapses in consciousness (microsomnias) that may be associated with automatic behaviors and AMNESIA. CATAPLEXY; SLEEP PARALYSIS, and hypnagogic HALLUCINATIONS frequently accompany narcolepsy. The pathophysiology of this disorder includes sleep-onset rapid eye movement (REM) sleep, which normally follows stage III or IV sleep. (From Neurology 1998 Feb;50(2 Suppl 1):S2-S7)Multiple System Atrophy: A syndrome complex composed of three conditions which represent clinical variants of the same disease process: STRIATONIGRAL DEGENERATION; SHY-DRAGER SYNDROME; and the sporadic form of OLIVOPONTOCEREBELLAR ATROPHIES. Clinical features include autonomic, cerebellar, and basal ganglia dysfunction. Pathologic examination reveals atrophy of the basal ganglia, cerebellum, pons, and medulla, with prominent loss of autonomic neurons in the brain stem and spinal cord. (From Adams et al., Principles of Neurology, 6th ed, p1076; Baillieres Clin Neurol 1997 Apr;6(1):187-204; Med Clin North Am 1999 Mar;83(2):381-92)Lewy Body Disease: A neurodegenerative disease characterized by dementia, mild parkinsonism, and fluctuations in attention and alertness. The neuropsychiatric manifestations tend to precede the onset of bradykinesia, MUSCLE RIGIDITY, and other extrapyramidal signs. DELUSIONS and visual HALLUCINATIONS are relatively frequent in this condition. Histologic examination reveals LEWY BODIES in the CEREBRAL CORTEX and BRAIN STEM. SENILE PLAQUES and other pathologic features characteristic of ALZHEIMER DISEASE may also be present. (From Neurology 1997;48:376-380; Neurology 1996;47:1113-1124)Acetogenins: Polyketides of up to a few dozen carbons in length, formed by chain extension of multiple PROPIONATES and oxygenated to form tetrahydrofuran and lactone rings along the length of the chain. They are found in ANNONACEAE and other PLANTS. Related compounds cyclize to MACROLIDES.Parkinson Disease: A progressive, degenerative neurologic disease characterized by a TREMOR that is maximal at rest, retropulsion (i.e. a tendency to fall backwards), rigidity, stooped posture, slowness of voluntary movements, and a masklike facial expression. Pathologic features include loss of melanin containing neurons in the substantia nigra and other pigmented nuclei of the brainstem. LEWY BODIES are present in the substantia nigra and locus coeruleus but may also be found in a related condition (LEWY BODY DISEASE, DIFFUSE) characterized by dementia in combination with varying degrees of parkinsonism. (Adams et al., Principles of Neurology, 6th ed, p1059, pp1067-75)REM Sleep Parasomnias: Abnormal behavioral or physiologic events that are associated with REM sleep, including REM SLEEP BEHAVIOR DISORDER.Sleep: A readily reversible suspension of sensorimotor interaction with the environment, usually associated with recumbency and immobility.Video Recording: The storing or preserving of video signals for television to be played back later via a transmitter or receiver. Recordings may be made on magnetic tape or discs (VIDEODISC RECORDING).Electromyography: Recording of the changes in electric potential of muscle by means of surface or needle electrodes.Parasomnias: Movements or behaviors associated with sleep, sleep stages, or partial arousals from sleep that may impair sleep maintenance. Parasomnias are generally divided into four groups: arousal disorders, sleep-wake transition disorders, parasomnias of REM sleep, and nonspecific parasomnias. (From Thorpy, Sleep Disorders Medicine, 1994, p191)Neurodegenerative Diseases: Hereditary and sporadic conditions which are characterized by progressive nervous system dysfunction. These disorders are often associated with atrophy of the affected central or peripheral nervous system structures.Cataplexy: A condition characterized by transient weakness or paralysis of somatic musculature triggered by an emotional stimulus or physical exertion. Cataplexy is frequently associated with NARCOLEPSY. During a cataplectic attack, there is a marked reduction in muscle tone similar to the normal physiologic hypotonia that accompanies rapid eye movement sleep (SLEEP, REM). (From Adams et al., Principles of Neurology, 6th ed, p396)Attention Deficit and Disruptive Behavior Disorders: Includes two similar disorders: oppositional defiant disorder and CONDUCT DISORDERS. Symptoms occurring in children with these disorders include: defiance of authority figures, angry outbursts, and other antisocial behaviors.Olfaction Disorders: Loss of or impaired ability to smell. This may be caused by OLFACTORY NERVE DISEASES; PARANASAL SINUS DISEASES; viral RESPIRATORY TRACT INFECTIONS; CRANIOCEREBRAL TRAUMA; SMOKING; and other conditions.Sleep Disorders: Conditions characterized by disturbances of usual sleep patterns or behaviors. Sleep disorders may be divided into three major categories: DYSSOMNIAS (i.e. disorders characterized by insomnia or hypersomnia), PARASOMNIAS (abnormal sleep behaviors), and sleep disorders secondary to medical or psychiatric disorders. (From Thorpy, Sleep Disorders Medicine, 1994, p187)Severity of Illness Index: Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder.Sleep Stages: Periods of sleep manifested by changes in EEG activity and certain behavioral correlates; includes Stage 1: sleep onset, drowsy sleep; Stage 2: light sleep; Stages 3 and 4: delta sleep, light sleep, deep sleep, telencephalic sleep.Motor Activity: The physical activity of a human or an animal as a behavioral phenomenon.Guadeloupe: The name of two islands of the West Indies, separated by a narrow channel. Their capital is Basse-Terre. They were discovered by Columbus in 1493, occupied by the French in 1635, held by the British at various times between 1759 and 1813, transferred to Sweden in 1813, and restored to France in 1816. Its status was changed from colony to a French overseas department in 1946. Columbus named it in honor of the monastery of Santa Maria de Guadalupe in Spain. (From Webster's New Geographical Dictionary, 1988, p470 & Room, Brewer's Dictionary of Names, 1992, p221)Autonomic Nervous System Diseases: Diseases of the parasympathetic or sympathetic divisions of the AUTONOMIC NERVOUS SYSTEM; which has components located in the CENTRAL NERVOUS SYSTEM and PERIPHERAL NERVOUS SYSTEM. Autonomic dysfunction may be associated with HYPOTHALAMIC DISEASES; BRAIN STEM disorders; SPINAL CORD DISEASES; and PERIPHERAL NERVOUS SYSTEM DISEASES. Manifestations include impairments of vegetative functions including the maintenance of BLOOD PRESSURE; HEART RATE; pupil function; SWEATING; REPRODUCTIVE AND URINARY PHYSIOLOGY; and DIGESTION.Sleep Disorders, Intrinsic: Dyssomnias (i.e., insomnias or hypersomnias) associated with dysfunction of internal sleep mechanisms or secondary to a sleep-related medical disorder (e.g., sleep apnea, post-traumatic sleep disorders, etc.). (From Thorpy, Sleep Disorders Medicine, 1994, p187)Electroencephalography: Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain.Wakefulness: A state in which there is an enhanced potential for sensitivity and an efficient responsiveness to external stimuli.Neurologic Examination: Assessment of sensory and motor responses and reflexes that is used to determine impairment of the nervous system.Eye Movement Measurements: Methods and procedures for recording EYE MOVEMENTS.Brain Stem: The part of the brain that connects the CEREBRAL HEMISPHERES with the SPINAL CORD. It consists of the MESENCEPHALON; PONS; and MEDULLA OBLONGATA.
Exploding head syndrome: Exploding head syndrome (EHS) is a condition in which a person hears loud noises (such as a bomb exploding, a gunshot, or a cymbal crash) or experiences an explosive feeling when falling asleep or waking up. These noises have a sudden onset, are typically brief in duration, and are often jarring for the sufferer.ClonazepamNightmare: A nightmare is an unpleasant dream that can cause a strong emotional response from the mind, typically fear but also despair, anxiety and great sadness. The dream may contain situations of discomfort, psychological or physical terror.PolysomnographyNarcolepsyGregor Wenning: Gregor K. Wenning (* born 21st March1964 in Horstmar, Westfalia) is a German Neurologist best known for his clinical and scientific work in Parkinson's disease and atypical Parkinsonian disorders, particularly multiple system atrophy (MSA).Dennis Walsh: Dennis Walsh (12 June 1933 – 1 June 2005) was an English astronomer, born into a poor family in Manchester. He was best known for his discovery in 1979 of the first example of a gravitational lens which he made while studying quasars found in the Jodrell Bank 966MHz survey.Jerry L. McLaughlin: Jerry L. McLaughlin is a researcher who has conducted research for 28 years studying plants looking for molecules that fight cancer at Purdue University's School of Pharmacy.Causes of Parkinson's disease: Parkinson's disease (PD) is a degenerative disorder of the central nervous system. Most people with PD have idiopathic Parkinson's disease (having no specific known cause).Non-rapid eye movement sleepProfessional DiscAging movement control: Normal aging movement control in humans is about the changes on the muscles, motor neurons, nerves, sensory functions, gait, fatigue, visual and manual responses, in men and women as they get older but who do not have neurological, muscular (atrophy, dystrophy...) or neuromuscular disorder.Benign neonatal sleep myoclonus: Benign neonatal sleep myoclonus (BNSM) is the occurrence of myoclonus (jerky movements) during sleep. It is not associated with seizures.Centre for Research in Neurodegenerative DiseasesCataplexyOlfactory sulcus: The medial orbital gyrus presents a well-marked antero-posterior sulcus, the olfactory sulcus, for the olfactory tract.Invasion of Guadeloupe (1794): The Invasion of Guadeloupe was a British attempt in 1794 to take and hold the island of Guadeloupe in the West Indies during the French Revolutionary Wars. The British had negotiated with the French planters, Ignace-Joseph-Philippe de Perpignan and Louis de Curt, who wished to gain British protection, as the French Constitutional Assembly was passing a law abolishing slavery.Quantitative electroencephalography: Quantitative electroencephalography (QEEG) is a field concerned with the numerical analysis of electroencephalography data and associated behavioral correlates.Central tegmental tract: The central tegmental tractKamali A, Kramer LA, Butler IJ, Hasan KM. Diffusion tensor tractography of the somatosensory system in the human brainstem: initial findings using high isotropic spatial resolution at 3.
(1/116) Rapid eye movement sleep behaviour disorder: demographic, clinical and laboratory findings in 93 cases.
We describe demographic, clinical, laboratory and aetiological findings in 93 consecutive patients with rapid eye movement (REM) sleep behaviour disorder (RBD), which consists of excessive motor activity during dreaming in association with loss of skeletal muscle atonia of REM sleep. The patients were seen at the Mayo Sleep Disorders Center between January 1, 1991 and July 31, 1995. Eighty-one patients (87%) were male. The mean age of RBD onset was 60.9 years (range 36-84 years) and the mean age at presentation was 64.4 years (37-85 years). Thirty-two per cent of patients had injured themselves and 64% had assaulted their spouses. Subdural haematomas occurred in two patients. Dream content was altered and involved defence of the sleeper against attack in 87%. The frequency of nocturnal events decreased with time in seven untreated patients with neurodegenerative disease. MRI or CT head scans were performed in 56% of patients. Although four scans showed brainstem pathology, all of these patients had apparently unrelated neurodegenerative diseases known to be associated with RBD. Neurological disorders were present in 57% of patients; Parkinson's disease, dementia without parkinsonism and multiple system atrophy accounted for all but 14% of these. RBD developed before parkinsonism in 52% of the patients with Parkinson's disease. Five of the 14 patients with multiple system atrophy were female, and thus the strong male predominance in RBD is less evident in this condition. Psychiatric disorders, drug use or drug withdrawal were rarely causally related to RBD. Clonazepam treatment of RBD was completely or partially successful in 87% of the patients who used the drug. We conclude that RBD is a well-defined condition and that descriptions from different centres are fairly consistent. It is commonest in elderly males and may result in serious morbidity to patients and bed partners. There is a strong relationship to neurodegenerative disease, especially Parkinson's disease, multiple system atrophy and dementia, and neurologists should explore the possibility of RBD in patients with these conditions. RBD symptoms may be the first manifestations of these disorders and careful follow-up is needed. Neuroimaging is unlikely to reveal underlying disorders not suspected clinically. We confirm the effectiveness of clonazepam, but note that attention to the safety of the bed environment may be sufficient for patients with contraindications to the drug. (+info)
(2/116) Rapid eye movement sleep behaviour disorder, depression and cognitive impairment. Case study.
BACKGROUND: Rapid eye movement (REM) sleep behaviour disorder is a relatively new diagnostic category. It has never before been associated with a treatable depressive condition. AIMS: To report on a 74-year-old man with a history of depression and REM sleep behaviour disorder, associated with mild cognitive impairment. METHOD: Assessment using brain CT, MRI, PET, electroencephalography, neuropsychological testing and nocturnal polysomnography. RESULTS: Depression was treated with sertraline. Sleep laboratory studies supported a diagnosis of REM sleep behaviour disorder, which was treated with clonazepam. Sleep apnoea, revealed later, was treated with nasal continuous positive airways pressure. Brain MRI showed mild atrophy, but neuropsychological testing indicated no progressive cognitive deterioration. CONCLUSIONS: This case draws attention to REM sleep behaviour disorder and its potential interaction with depression and cognitive impairment, producing symptoms which can be mistaken for early dementia. The diagnosis of REM sleep behaviour disorder is easily missed, and it requires careful history-taking and sleep investigation in all suspected sufferers. Associated neurological, sleep and psychiatric conditions (including depression and cognitive impairment) may confound the diagnosis. (+info)
(3/116) Interobserver reliability of ICSD-R criteria for REM sleep behaviour disorder.
We estimated the interobserver reliability (IR) of the diagnosis of rapid eye movement (REM) Sleep Behaviour Disorder (RBD) among trained neurologists, with the application of International Classification of Sleep Disorders Revised (ICSD-R), by means of videotaped interviews of people with motor sleep behaviour disorders of different nature. IR of clinical judgement for the diagnosis of RBD was "substantial" (Kappa 0.65); nevertheless, some criteria ('limb or body movement associated with dream mentation', criterion B, and 'sleep behaviours (that) disrupt sleep continuity', criterion C3) showed a 'moderate' IR, resulting from the intrinsic limitations of the patient report and terminological ambiguity. Further clarification of terminology of the ICSD-R criteria would be useful to improve the reliability. (+info)
(4/116) Falling asleep.
We describe a 74-year-old woman who presented with a history of falling from bed in association with vivid dreams and physical violence towards her spouse. A clinical diagnosis of rapid eye movement sleep behaviour disorder was made and complete resolution of her symptoms was achieved with first line treatment. (+info)
(5/116) Combination of 'idiopathic' REM sleep behaviour disorder and olfactory dysfunction as possible indicator for alpha-synucleinopathy demonstrated by dopamine transporter FP-CIT-SPECT.
REM sleep behaviour disorder (RBD) and olfactory dysfunction are common and very early features of alpha-synucleinopathies, in particular Parkinson's disease. To investigate the hypothesis that these two clinical features in combination are an indicator of evolving alpha-synucleinopathy, olfactory function was assessed in RBD. We studied 30 patients (18 male, 12 female; mean age 48 +/- 14 years, range 19-78 years) with clinical (idiopathic, n = 6; symptomatic, n = 13, mostly associated with narcolepsy) or subclinical (n = 11, associated with narcolepsy) RBD according to standard criteria and 30 age- and gender-matched healthy control subjects using standardized 'Sniffin' Sticks'. RBD patients had a significantly higher olfactory threshold (P = 0.0001), lower discrimination score (P = 0.003), and lower identification score (P = 0.001). Compared with normative data, 97% of the RBD patients had a pathologically increased olfactory threshold, 63% an impaired odour discrimination score, and 63% a decreased identification score. On neurological examination, signs of parkinsonism were newly found in five patients with clinical RBD (not associated with narcolepsy), who usually had a long history of 'idiopathic' RBD. Four of the five patients fulfilled the UK Brain Bank criteria for the clinical diagnosis of Parkinson's disease. The underlying nigrostriatal degeneration of clinical Parkinson's disease was confirmed by I-123-FP-CIT SPECT in one patient and early nigrostriatal degeneration was identified by SPECT in a further two patients with 'idiopathic' clinical RBD out of 11 RBD patients who agreed to undergo SPECT studies. Our study shows that RBD patients have a profound impairment of olfactory function. Five patients with clinical RBD not associated with narcolepsy had clinical or imaging signs of nigrostriatal degeneration. This new clinical finding correlates with the neuropathological staging of Parkinson's disease (stages 1-3) as proposed by Braak. In stage 1, the anterior olfactory nucleus or the olfactory bulb is affected (along with the dorsal motor nucleus of the glossopharyngeal and vagal nerves). In stage 2, additional lesions consistently remain confined to the medulla oblongata and pontine tegmentum, which are critical areas for RBD. Midbrain lesions are found only in stage 3, in particular degeneration of dopaminergic neurons in the substantia nigra pars compacta. Thus, 'idiopathic' RBD patients with olfactory impairment might present with stage 2 preclinical alpha-synucleinopathy. Since narcoleptic patients are not known to have an increased risk of developing parkinsonism, the pathophysiology and clinical relevance of hyposmia in RBD/narcolepsy patients requires further research. (+info)
(6/116) Impaired rapid eye movement sleep in the Tg2576 APP murine model of Alzheimer's disease with injury to pedunculopontine cholinergic neurons.
Impaired rapid eye movement sleep (REMS) is commonly observed in Alzheimer's disease, suggesting injury to mesopontine cholinergic neurons. We sought to determine whether abnormal beta-amyloid peptides impair REMS and injure mesopontine cholinergic neurons in transgenic (hAPP695.SWE) mice (Tg2576) that model brain amyloid pathologies. Tg2576 mice and wild-type littermates were studied at 2, 6, and 12 months by using sleep recordings, contextual fear conditioning, and immunohistochemistry. At 2 months of age, REMS was indistinguishable by genotype but was reduced in Tg2576 mice at 6 and 12 months. Choline acetyltransferase-positive neurons in the pedunculopontine tegmentum of Tg2576 mice at 2 months evidenced activated caspase-3 immunoreactivity, and at 6 and 12 months the numbers of pedunculopontine tegmentum choline acetyltransferase-positive neurons were reduced in the Tg2576 mice. Other cholinergic groups involved in REMS were unperturbed. At 12 months, Tg2576 mice demonstrated increased 3-nitrotyrosine immunoreactivity in cholinergic projection sites but not in cholinergic soma. We have identified a population of selectively compromised cholinergic neurons in young Tg2576 mice that manifest early onset REMS impairment. The differential vulnerability of these cholinergic neurons to Abeta injury provides an invaluable tool with which to understand mechanisms of sleep/wake perturbations in Alzheimer's disease. (+info)
(7/116) Visual hallucinations in posterior cortical atrophy.
BACKGROUND: Visual hallucinations have been reported to occur in up to 25% of patients who meet the criteria for posterior cortical atrophy (PCA). It is not known, however, whether patients who meet the criteria for PCA and have hallucinations are different from those who meet the criteria and do not have hallucinations. OBJECTIVE: To compare the clinical and imaging features of patients with PCA with and without well-formed visual hallucinations. DESIGN: Case-control study. SETTING: Tertiary care medical center. PATIENTS: Fifty-nine patients fulfilling the criteria for PCA were retrospectively identified and divided into 2 groups based on the presence (n = 13) or absence (n = 46) of visual hallucinations. MAIN OUTCOME MEASURES: Statistically significant clinical differences and imaging differences using voxel-based morphometry between the 2 groups. RESULTS: In patients with PCA and hallucinations, parkinsonism and rapid eye movement sleep behavior disorder occurred more frequently, as did myoclonic jerks (P<.001 for both). Voxel-based morphometry showed greater atrophy in a network of structures, including the primary visual cortex, lentiform nuclei, thalamus, basal forebrain, and midbrain, in patients with hallucinations. CONCLUSIONS: Hallucinations in patients with PCA are associated with parkinsonism, rapid eye movement sleep behavior disorder, and myoclonic jerks. The voxel-based morphometry results suggest that hallucinations in PCA cannot be exclusively attributed to atrophy of the posterior association cortices and may involve a circuit of thalamocortical connections. (+info)
(8/116) Olfactory dysfunction in patients with narcolepsy with and without REM sleep behaviour disorder.
Patients with idiopathic rapid eye movement sleep behaviour disorder (RBD) frequently develop Parkinson's disease and the majority present with hyposmia, which is a potential preclinical non-motor sign of Parkinson's disease. Accordingly, it has been proposed that the clinical symptoms of hyposmia and RBD in combination have to be considered as very early symptoms of Parkinson's disease. Since not only patients with idiopathic RBD but also patients in whom RBD is associated with narcolepsy present with an olfactory dysfunction we investigated if hyposmia in RBD patients with concomitant narcolepsy is RBD specific or if narcolepsy per se is associated with olfactory dysfunction. We studied olfactory function in 20 narcoleptic patients each with RBD (9 male and 11 female; mean age 45.4 +/- 14.0 years, range 20-75 years) and without associated RBD (8 male and 12 female; mean age 44.4 +/- 13.40 years, range 20-70 years) and 40 age- and gender-matched healthy control subjects using standardized 'Sniffin' Sticks'. Both, narcoleptics with (Narc/+RBD) and without RBD (Narc/-RBD) had a significantly higher olfactory threshold (Narc/+RBD, P = 0.0001; Narc/-RBD, P = 0.0001), lower discrimination scores (P = 0.001; P = 0.014) and lower identification scores (P = 0.057; P = 0.003) than controls. There were no symptoms or signs for early parkinsonism in both patient groups. Our results show for the first time that narcolepsy per se is associated with olfactory dysfunction. In contrast to patients with idiopathic RBD, hyposmia in patients with RBD associated with narcolepsy is unlikely to be a predictor for developing parkinsonism. (+info)
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