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[Vocal cord abductor paralysis in multiple system atrophy--paradoxical movement of vocal cords during sleep].

Bilateral vocal cord abductor paralysis (VCAP) is frequently associated with multiple system atrophy (MSA) and the early clinical manifestation of VCAP is nocturnal inspiratory stridor simulating heavy snoring observed in patients with obstructive sleep apnea syndrome. We examined six MSA patients with nocturnal stridor and four disease controls including sleep apnea syndrome. Vocal cord movements were analyzed by laryngofiberscopy during both wakefulness and sleep induced by intravenous administration of diazepam. The results were as follows: First, the stenotic portion in the upper airway tract was the larynx (the vocal cords) in MSA patients with stridor, while the soft palate or the pharynx in the disease controls. Second, in the MSA patients, while awake-laryngofiberscopy showed abduction restriction suggestive of VCAP in only one of the six patients, sleep-laryngofiberscopy showed obvious paradoxical movement of the vocal cord in all the rests, where the vocal cords abducted in expiration and adducted in inspiration. In addition, there were two patterns in the inspiratory vocal cord position during sleep: one pattern where vocal glottis was still opening at the posterior one-third area and the other pattern where vocal glottis was almost completely closed through total length of the cords. Tracheostomy should be considered in the latter stage of VCAP.

Aged↗

After phrenicotomy the rat alters the output of the remaining respiratory muscles without changing its sleep-waking pattern.

The respiratory activity of selected rib cage and abdominal wall muscles was studied in intact and bilaterally phrenicotomized (PNX) rats during non-rapid eye movement sleep (nREMS) and REMS. The polygraphic method was used to identify the animal's sleep-waking states before and after PNX. Electromyographic (EMG) recordings were made from the following muscles: the parasternals of the 1st, 3rd, and 5th interspaces; the external and internal intercostals of the 1st, 6th and 10th interspaces; the levator costae attaching to the 10th rib; the scalenus medius; and, the abdominal wall muscles, the external and internal obliques and rectus abdominis. After PNX, all rib cage muscles contracted exclusively during inspiration and all but one increased their activity. The exception was the internal intercostal muscle of the 10th interspace; its activity decreased. The external and internal oblique muscles, both of which were active during expiration in nREMS, also increased their output after PNX: rectus abdominis became an inspiratory muscle. The persistence of phasic activity of respiratory muscles during REMS varied not only from muscle to muscle but from one REMS epoch to another. The sleep-waking pattern of the PNX rat differed in only a minor way from that of the intact rat. Therefore, we conclude that the rat with total paralysis of its diaphragm uses mainly its neurometabolic mechanisms to achieve an adequate level of alveolar ventilation and not neurobehavioral mechanisms.

Abdominal Muscles↗

Parasomnias: epidemiology and management.

The category of common sleep disorders known as parasomnias includes disorders of arousal, rapid eye movement (REM) sleep behaviour disorder (RBD), nocturnal seizures, rhythmic movement disorder, and tooth grinding or 'bruxism'. Parasomnias are all characterised as undesirable physical or behavioural phenomena occurring during the sleep period. Although these conditions can be distressing and, in some cases, hazardous to the sleeper and his or her bed partner, it is important to recognise that parasomnias are diagnosable and treatable in the vast majority of patients. Evaluation begins with a careful clinical interview with the sleeper and a family member to elucidate the frequency, duration, description and timing after sleep onset of these behavioural events. Disorders of arousal are the most common type of parasomnia and cover a spectrum from calm sleepwalking to emotionally agitated or complex behaviours, such as dressing or driving, for which the patient usually has no memory upon awaking. 'Sleep terrors' are quite common in young children and are often outgrown. Disorders of arousal represent a partial, as opposed to a full, awakening from deep non-REM sleep, typically occurring within the first 60 to 90 minutes after sleep onset. RBD is characterised clinically by a history of dream-enacting behaviour, and the patient may recall dream content. REM sleep periods typically occur in the latter half of the night. Physiologically, RBD results from a lack of the normal muscle atonia that is associated with REM sleep. RBD has been linked to a number of other neurological conditions; thus, a careful review of systems and a physical examination are crucial. A formal laboratory sleep study or polysomnogram with an expanded electroencephalographic montage can help distinguish among non-REM and REM parasomnias and nocturnal seizures. The latter may manifest clinically as arousals from sleep associated with vocalisation and/or complex behaviours. Rhythmic movement disorder can include head banging or body rocking at sleep onset or during the night. Tooth grinding is a common sleep-related behaviour that, when severe, can result in dental injury. Hypnagogic hallucinations (experience of dream imagery at sleep onset) and sleep-onset paralysis (experience of muscle/body paralysis as one is falling asleep) are symptoms rather than diagnostic categories. These phenomena classically occur in many individuals with narcolepsy, but also may occur in healthy sleep-deprived individuals. Safety precautions and good general sleep hygiene measures are recommended for individuals with a parasomnia, as the disorder can be exacerbated by sleep deprivation and various other factors. When the events are frequent or particularly dramatic, medication with a long- or medium-acting benzodiazepine, such as clonazepam, at bedtime is effective therapy in most cases of non-REM disorders of arousal and RBD. A dental guard may be helpful in tooth grinders. Relaxation training and guided imagery may be helpful strategies for some patients, especially those with disorders of arousal or rhythm movement disorders. There is no evidence of any association between parasomnias and psychiatric illness. Demystification of these conditions and reassurance, particularly for parents of paediatric patients, is an important aspect of clinical intervention.

Age Factors↗

[Value of sleep polygraph examination in the etiological diagnosis of apparently inexplicable respiratory insufficiency].

This study describes the case of a 58 year old man who presented with an episode of acute respiratory failure and right heart decompensation. After recovery from the acute illness, hypoxaemia, hypercapnia and pulmonary arterial hypertension remained, the causes of which were not known. There was no airway obstruction, only a moderate restrictive ventilatory defect, a little weight increase and a unilateral diaphragmatic paralysis. Obstructive sleep apnoea was finally suspected and confirmed by sleep recording. The obstructive sleep apnoea probably explained the respiratory insufficiency and the pulmonary hypertension. Loss of weight was associated with the disappearance of hypercapnia and pulmonary hypertension. As a result of this study, the value of sleep recording is emphasized. When respiratory failure or pulmonary hypertension seem unexplained, think of obstructive sleep apnoea.

Humans↗

[Sleep in progressive supranuclear palsy].

Nocturnal sleep was poligraphycally recorded in three male patients aged 54-67, with progressive supranuclear palsy (PSP). All patients suffered from insomnia. In case 1 REM sleep was markedly reduced and spindles were less numerous than in normal subjects. In cases 2 and 3, EEG patterns were not distinguishable from those observed when the patients were awake. Sleep, therefore, was recognized only by constant observation of the patients. As seen in the literature EEG changes during sleep can be correlated to the severity of the clinical picture and the stage of evolution of the disease. EEG patterns of sleep in PSP are similar to those reported in patients with presenile dementia.

Aged↗

Narcolepsy-cataplexy. I. Clinical and electrophysiologic characteristics.

The development, clinical course, and electrophysiologic characteristics of narcolepsy were evaluated in 50 adults who had a current complaint of sleep attacks and cataplexy. In most of the patients, the first symptoms, usually excessive daytime sleepiness and sleep attacks, developed during childhood or adolescence. The condition was invariably chronic. Patients frequently had family histories of some disorder of excessive daytime sleepiness. In nocturnal sleep or daytime nap recordings, all but three of the patients demonstrated a rapid-eye-movement (REM) period at sleep onset. Sleep apnea was found in only one patient. Our findings indicate that sleep laboratory recordings to detect a sleep-on-set REM period are of little diagnostic value when the narcoleptic patient has cataplexy. Furthermore, narcoleptic patients require sleep laboratory evaluation for sleep apnea only when the presence of apnea is suggested by the sleep history.

Adolescent↗

Isolated cataplexy of more than 40 years' duration.

For over 40 years, a 55-year-old woman had suffered from cataplexy, which had been mistakenly identified as hysteria. Sleepiness, hypnagogic hallucinations and sleep-onset paralysis were not present. This is the longest duration of isolated cataplexy reported so far.

Cataplexy↗

Resistive inspiratory muscle training in sleep-disordered breathing of traumatic tetraplegia.

OBJECTIVE: To assess the effect of resistive inspiratory muscle training (RIMT) on the static pulmonary function and sleep-induced breathing disorder of individuals with chronic cervical cord injury. DESIGN: Before-after training. SETTING: Home-setting training program. PATIENTS: Fourteen complete traumatic tetraplegic patients (12 men, 2 women; mean age, 41.1 +/- 14y; range, 19-56y) injured for more than 6 months. INTERVENTION: Subjects participated in a 6-week RIMT program for 15 minutes twice daily at a training intensity of 60% of maximum inspiratory pressure (MIP). The participants were reevaluated at the end of 6-week training. MAIN OUTCOME MEASURES: Lung volume, peak expiratory flow (PEF), MIP, and maximum expiratory pressure (MEP) were measured by using a spirometry and inspiratory force meter, respectively. Capnography was used to monitor nocturnal pulse oxyhemoglobin saturation (SpO(2)) and end-tidal carbon dioxide tension level (ETCO(2)) of the patients. RESULTS: The maximum voluntary ventilation (MVV) and MIP of individuals with chronic cervical cord injury substantially improved after RIMT. MIP increased from -68.7 +/- 27.4cmH(2)O to -77.3 +/- 24.0cmH(2)O and MVV rose from 62.7 +/- 33.2L to 73.4 +/- 31.3L (P <.05). Despite increasing from 3.5 +/- 1.8L/s to 4.0 +/- 1.7L/s, PEF was statistically insignificant. For the individuals with improved MIP, the duration of ETCO(2) greater than 48mmHg reduced from 2.2% +/- 3.3% to 1.0% +/- 2.0% of total sleep time (P =.05) and that of SpO(2) less than 90% significantly declined from 1.8% +/- 2.8% to 1.3% +/- 2.4% of total sleep time (P <.05). CONCLUSION: These findings suggest that RIMT can enhance the respiratory muscle strength and endurance of chronic tetraplegia and further ameliorate the sleep-induced breathing disorder. Therefore, RIMT is suggested as a home program for patients with sleep-disordered breathing.

Adult↗

Progressive supranuclear palsy.

Progressive supranuclear palsy has been recognized as a distinct nosological entity for about three decades now. Typically, this progressive neurological disease manifests itself late in the sixth decade with a terminal course of approximately four to six years. Well over one hundred cases have been described in the literature and the heterogeneous nature of progressive supranuclear palsy includes the characteristic vertical ophthalmoplegia, frequent falling and a profound nuchal rigidity. Other features are similar in many respects to those found in Parkinson's disease. The present article reviews the literature on progressive supranuclear palsy with particular reference to its clinical manifestations including the ophthalmoplegia, characteristic sleep disturbances and unique dementia. Also addressed, are neuropathological and epidemiological findings. Finally, conclusions and recommendations for further investigation are offered especially with regard to the neuropsychological nature of this neurological disorder.

Brain↗

Static properties of the passive pharynx in sleep apnea.

Complete paralysis under general anesthesia allowed separating anatomic factors from neural factors which influence pharyngeal patency. We compared static mechanical properties of the passive pharynx in normals and sleep apneics. The passive pharynx was narrower and more collapsible in sleep apneics than normal controls indicating significance of anatomic factors in the pathogenesis of obstructive sleep apnea.

Electroencephalography↗

Respiratory muscle activity and oxygenation during sleep in patients with muscle weakness.

Patients with respiratory muscle weakness show nocturnal hypoventilation, with oxygen desaturation particularly during rapid eye movement (REM) sleep, but evidence in individuals with isolated bilateral diaphragmatic paresis (BDP) is conflicting. The effect of sleep on relative activity of the different respiratory muscles of such patients and, consequently, the precise mechanisms causing desaturation have not been clarified. We have studied eight patients, four with generalized muscle weakness and four with isolated BDP during nocturnal sleep with measurements including oxygen saturation and surface electromyographic (EMG) activity of various respiratory muscle groups. Nocturnal oxygenation correlated inversely with postural fall in vital capacity, an index of diaphragmatic strength. During REM sleep, hypopnoea and desaturation occurred particularly during periods of rapid eye movements (phasic REM sleep). In most subjects, such events were "central" in type and associated with marked suppression of intercostal muscle activity, but two subjects had recurrent desaturation due to "obstructive" hypopnoea and/or apnoea. Expiratory activity of the external oblique muscle was present whilst awake and during non-rapid eye movement (NREM) sleep in seven of the eight subjects in the semirecumbent posture. This probably represents an "accessory inspiratory" effect, which aids passive caudal diaphragmatic motion as the abdominal muscles relax at the onset of inspiration. Expiratory abdominal muscle activity was suppressed in phasic REM sleep, suggesting that loss of this "accessory inspiratory" effect may contribute to "central" hypopnoea. We conclude that, in patients with muscle weakness, nocturnal oxygenation correlates with diaphragmatic strength.(ABSTRACT TRUNCATED AT 250 WORDS)

Electromyography↗

Sleep disorders and diaphragmatic function in patients with amyotrophic lateral sclerosis.

In amyotrophic lateral sclerosis (ALS), the progressive loss of upper and lower motor neurons leads to respiratory failure, often with predominant diaphragm dysfunction, and death. Because the diaphragm is the only active inspiratory muscle during rapid eye movement (REM) sleep, there is a high theoretical risk of respiratory disorders during REM sleep in patients with ALS. To assess this hypothesis, we studied sleep characteristics (polysomnography) in 21 patients with ALS, stratified according to the presence or absence of diaphragmatic dysfunction. Diaphragmatic dysfunction was defined as an absent or delayed diaphragm response to cervical or cortical magnetic stimulation, abdominal paradox, or respiratory pulse (Group 1, 13 patients). These patients did not differ in age, clinical course, or form (bulbar or spinal) from the eight others, who did not have diaphragmatic dysfunction (Group 2). REM sleep was reduced in Group 1 (7 +/- 7% of total sleep time; mean +/- SD) and normal in Group 2 (18 +/- 6%, p = 0.004). Apneas or hypopneas were rare in both groups. In Group 1, REM sleep was absent or minimal (less than 3 min) in five patients. An unusual and remarkable preservation of phasic inspiratory sternomastoid activation during REM was associated with longer REM sleep duration in six of the other patients with diaphragmatic dysfunction. Median survival time was dramatically shorter (217 d) in Group 1 than in Group 2 (619 d, p = 0.015).

Adult↗