A 69-year-old man with anti-Ma2 paraneoplastic encephalitis presented with subacute onset of severe hypersomnia, memory loss, parkinsonism, and gaze palsy. A brain magnetic resonance imaging study showed bilateral damage in the dorsolateral midbrain, amygdala, and paramedian thalami. Videopolysomnography disclosed rapid eye movement (REM) sleep behavior disorder, and a Multiple Sleep Latency Test showed a mean sleep latency of 7 minutes and 4 sleep-onset REM periods.
View Article and Find Full Text PDFThe pathophysiology of restless legs syndrome (RLS) is associated with central dopaminergic system dysfunction leading to speculations that RLS may be common in those neurodegenerative diseases with dopaminergic cell loss. However, since RLS is a very common condition, the co-occurrence with less frequent disorders such as the neurodegenerative diseases might be a matter of chance. Currently, no data suggests that patients with sporadic and familial RLS are at increased risk for developing a neurodegenerative disease.
View Article and Find Full Text PDFDuring a viewing of Disney's animated film Cinderella (1950), one author (AI) noticed a dog having nightmares with dream-enactment that strongly resembled RBD. This prompted a study in which all Disney classic full-length animated films and shorts were analyzed for other examples of RBD. Three additional dogs were found with presumed RBD in the classic films Lady and the Tramp (1955) and The Fox and the Hound (1981), and in the short Pluto's Judgment Day (1935).
View Article and Find Full Text PDFNasal continuous positive airway pressure (CPAP) is an effective treatment for most patients with obstructive sleep apnea syndrome (OSAS), improving sleepiness, cognitive function and mood. A number of patients, however, complain about persistent sleepiness after CPAP. In these cases another clinical history should be carried out to confirm the diagnosis of OSAS, to check CPAP compliance and to exclude associated conditions such as poor sleep hygiene, depression, narcolepsy or idiopathic hypersomnia.
View Article and Find Full Text PDFBackground And Objective: To evaluate the distribution of clinical, electrophysiological and biological variables, and their relationship with the CSF hypocretin-1 levels, in patients with central hypersomnias diagnosed as narcolepsy-cataplexy (NC), narcolepsy without cataplexy (NnC) and idiopathic hypersomnia (IH) based on the ICSD-2 criteria.
Patients And Method: We performed in all patients a clinical interview, a nocturnal polysomnogram and a multiple sleep latency test (MSLT), HLA analysis and measurement of CSF Hcrt-1 levels (low < or = 110 pg/mL).
Results: Out of 51 patients, 31 were classified as NC, 11 as NnC and 8 as IH.
An autoimmune-mediated mechanism is considered the most probable etiology for narcolepsy. However, this hypothesis remains unproven. Since narcolepsy is characterized by dysfunction of the hypothalamic hypocretinergic (orexinergic) system, we evaluated the presence of hypothalamic-specific antibodies in sera and CSF of 25 hypocretin-deficient and 6 non-deficient narcoleptic patients by immunohistochemistry and analyzing a screening of a rat cDNA expression hypothalamic library.
View Article and Find Full Text PDFTo study dream content in patients with severe obstructive sleep apnea syndrome (OSAS) and its modification with Continuous Positive Airway Pressure (CPAP) therapy. We assessed twenty consecutive patients with severe OSAS and 17 healthy controls. Polysomnograms were recorded at baseline in patients and controls and during the CPAP titration night, 3 months after effective treatment and 2 years later in patients.
View Article and Find Full Text PDFBackground: Rapid-eye-movement (REM) sleep behaviour disorder (RBD) is a parasomnia characterised by dream-enacting behaviours related to unpleasant dreams and loss of muscle atonia during REM sleep. RBD may be idiopathic or associated with neurological disease. Available data suggest that in some cases RBD might be the initial manifestation of a neurodegenerative disease.
View Article and Find Full Text PDFalpha(4)beta(1) and alpha(4)beta(7) integrins are preferentially expressed on eosinophils and mononuclear leukocytes and play critical roles in their recruitment to inflammatory sites. We investigated the effects of TR14035, a small molecule, alpha(4)beta(1)/alpha(4)beta(7) dual antagonist, in a rat model of allergic asthma. Actively sensitized rats were challenged with aerosol antigen or saline on day 21, and the responses evaluated 24 and 48-h later.
View Article and Find Full Text PDFOf six patients registered in our center with nonparaneoplastic limbic encephalitis associated with antibodies to voltage-gated potassium channels, the five men had rapid eye movement sleep behavior disorder (RBD) coincident with voltage-gated potassium channel antibody-associated limbic encephalitis onset. In three patients, immunosuppression resulted in resolution of RBD in parallel with remission of the limbic syndrome. RBD persisted in two patients with partial resolution of the limbic syndrome.
View Article and Find Full Text PDFObjective: To describe the clinical and video-polysomnographic (VPSG) features of a group of subjects with severe obstructive sleep apnea/hypopnea (OSAH) mimicking the symptoms of REM sleep behavior disorder (RBD).
Design: Evaluation of clinical and VPSG data.
Setting: University hospital sleep laboratory unit.
Objective: To compare the clinical and video-polysomnographic (VPSG) characteristics of idiopathic REM sleep behavior disorder (RBD) vs the RBD seen in multiple system atrophy (MSA) and Parkinson disease (PD).
Methods: Clinical features and VPSG measures were evaluated in 110 consecutive nondemented subjects (26 MSA, 45 PD, and 39 idiopathic RBD) free of psychoactive medications referred for suspected RBD to our sleep unit over a 5-year period, with extended follow-up (mean 26.9 +/- 21.
Several neurodegenerative disorders have a profound metabolic and structural impact on the brainstem. MR spectroscopy provides metabolic information non-invasively and has the potential to characterize the changes associated with normal aging and differentiate them from neurodegenerative alterations. The present work was aimed at studying the upper brainstem tegmentum at the midbrain and pontine levels in 57 adult normal volunteers, aged 23-79 years, with long-echo time proton MR spectroscopy to evaluate possible regional differences and the effect of age.
View Article and Find Full Text PDFA large number of patients with Parkinson's disease (PD) experience nocturnal problems that impair their sleep quality. Among them, motor disorders such as tremor, rigidity, akinesia, akathisia, periodic leg movements, painful dystonia, dyskinesias, restless legs syndrome, and rapid eye movement sleep behavior disorder are common. This article reviews the clinical characteristics of some nocturnal motor problems that often induce sleep disruption in PD patients.
View Article and Find Full Text PDFRapid eye movement sleep behavior disorder (RBD) in the setting of parkinsonism or dementia often reflects an underlying synucleinopathy. Lewy bodies, intraneuronal aggregates containing abnormal alpha-synuclein, are absent in most cases of parkinsonism with parkin mutations (Park2). We performed clinical history and video-polysomnography in 10 Park2 patients (seven men; age, 51.
View Article and Find Full Text PDFThe authors found that continuous positive airway pressure (CPAP) eliminated video-polysomnographic documented stridor in 13 multiple system atrophy (MSA) subjects with vocal cord abnormalities. Long-term follow-up showed high CPAP tolerance, no recurrence of stridor, no major side effects, subjective improvement in sleep quality, and that median survival time was similar to a group of 26 MSA patients without stridor. This study shows that in MSA, CPAP is an effective noninvasive long-term therapy for nocturnal stridor.
View Article and Find Full Text PDFSleep disorders are so common in multiple system atrophy that they should be considered an integral part of the disease. Sleep fragmentation occurs in more than half of the patients, with sleep onset and sleep maintenance insomnia. Periodic leg movements of sleep are also common, although they are generally asymptomatic.
View Article and Find Full Text PDFIn Don Quijote de la Mancha, Miguel de Cervantes presents Don Quixote as an amazing character of the 17th century who suffers from delusions and illusions, believing himself to be a medieval knight errant. Besides this neuropsychiatric condition, Cervantes included masterful descriptions of several sleep disorders such as insomnia, sleep deprivation, disruptive loud snoring and rapid eye movement sleep behaviour disorder. In addition, he described the occurrence of physiological, vivid dreams and habitual, post-prandial sleepiness--the siesta.
View Article and Find Full Text PDFPeriodic leg movements in sleep (PLMS) unrelated to restless legs syndrome (RLS) are a polysomnographic finding with a controversial clinical value. We describe a patient with isolated periodic leg movements in sleep (without any awake or sleep complaints), who developed severe diurnal RLS symptoms a few months after starting dopaminergic treatment, a phenomenon mimicking augmentation. The diurnal RLS symptoms disappeared after withdrawal of the dopaminergic drugs.
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