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crossing bers between respiratory nuclei (page 472) In the few autopsied cases of congenital central hypoventilation, Liu and colleagues found the external arcuate nuclei of the medulla to be absent and the neuron population in the medullary respiratory areas to be depleted Patients with primary hypoventilation syndromes are usually of normal body habitus Awakenings during the night are frequent, usually after an apneic period, and insomnia is a common complaint Snoring is mild and intermittent Apnea of the obstructive type is more common than the purely central variety Obstructive apnea is often associated with obesity and less frequently with acromegaly, myxedema, micrognathia, and myotonic dystrophy In children far more than in adults, adenotonsillar hypertrophy may be a factor Other instances occur in neuromuscular diseases that weaken the posterior pharyngeal musculature; motor neuron disease is the most common example of this group Obstructive sleep apnea is characterized by noisy snoring of a special type After a period of regular albeit noisy breathing, there occurs a waning of breathing efforts; then, despite repeated inspiratory efforts, air ow ceases After a prolonged period of apnea (10 to 30 s or even longer), the patient makes a series of progressively greater breathing efforts until breathing resumes, accompanied by very loud snorting sounds and a brief arousal Obstructive sleep apnea occurs during both REM and NREM sleep The upper respiratory muscles (genioglossus, geniohyoid, tensor veli palatini, and medial pterygoid) normally contract just before the diaphragm contracts, resisting the collapse of the oropharynx If the airway is obstructed or the muscles are weakened and then go slack, the negative intrathoracic pressure causes narrowing of this passage Sedative medications, excessive tiredness, a recent stroke, head trauma or other acute neurologic disease, and primary pulmonary disease may all exaggerate obstructive sleep apnea, particularly in the obese patient with a tendency to snore The occurrence of a prolonged period of apnea, from whatever cause, is accompanied by progressive hemoglobin oxygen desaturation, hypercapnia and hypoxia, a transient increase in systemic and pulmonary arterial pressures, and sinus bradycardia or other arrhythmias The blood gas changes or perhaps other stimuli induce an arousal response, either a lightening of sleep or a very brief awakening, which is followed by an immediate resumption of breathing The patient quickly falls asleep again and the sequence is repeated, several hundred times a night in severe cases, greatly disrupting the sleep pattern and reducing the total sleep time Paradoxically, these patients are very dif cult to rouse at all times during the night Sleep apnea syndromes occur in persons of all ages In adults, obstructive sleep apnea is predominantly a disorder of overweight, middle-aged men and usually presents as excessive daytime sleepiness, a complaint that is often mistaken for narcolepsy (see below) Other patients, usually those with the much less common central form of apnea, complain mainly of a disturbance of sleep at night, or insomnia, which may be incorrectly attributed to anxiety or depression Morning headache, inattentiveness, and decline in school or work performance are other symptoms attributable to sleep apnea Ultimately, systemic and pulmonary arterial hypertension, cor pulmonale, polycythemia, and heart failure may develop, particularly in patients with obstructive apnea When combined with obesity, these symptoms are frequently referred to as the pickwickian syndrome, so named by Burwell and coworkers (1956), who identi ed this clinical syndrome with that of the extraordinarily sleepy, red-faced fat boy described by Dickens in The Pickwick Papers The term may no longer be apt, since it fails to take note of the facilitatory role of reduced respiratory drive of central type in the.

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genesis of obstructive apnea Furthermore, obesity need not be present in patients with sleep apnea; conversely, sleep apnea occurs in only a small proportion of obese persons In infants with delayed maturation of the respiratory centers, sleep apnea is not infrequent and not without danger, accounting for a certain number of crib deaths [sudden infant death syndrome (SIDS)] In approximately half of the observed infants with this condition, the apnea represents a respiratory arrest during a seizure This can be demonstrated by EEG The full-blown syndrome of obstructive sleep apnea is readily recognized by the features of daytime sleepiness, loud snoring, and the typical habitus of affected individuals However, in patients who complain only of excessive daytime sleepiness or insomnia, the diagnosis may be elusive and require special tests of respiratory function in addition to all-night polygraphic sleep monitoring Treatment The approach is governed by the severity of symptoms and the predominant type of apnea, central or obstructive In central apnea, any underlying abnormality, such as congestive heart failure or nasal obstruction, should, of course, be treated insofar as possible Trazodone (50 mg at bedtime) has reportedly been successful in reducing sleep apnea associated with olivopontocerebellar degeneration (Salazar-Grueso et al) Where no underlying cause can be found, one of several medications acetazolamide, medroxyprogesterone, protriptyline, and particularly clomipramine may be helpful in the short run (Brownell et al) Low- ow oxygen may also be useful in reducing central sleep apnea In the treatment of obstructive apnea, continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BIPAP) is the most useful measure This is delivered by a tight- tting nasal mask that is worn at night and connected to a pressure-cycled ventilator circuit triggered by the patient s breath The pressure ostensibly maintains patency of the naso- and oropharynx, thereby reducing the obstructive component Patients also bene t from losing weight, lateral positioning during sleep, avoidance of alcohol and other sedative drugs, and the use of the medications mentioned above Surgical correction of an upper airway defect may be helpful, but it is dif cult to predict which patients will bene t and there are no clear guidelines for procedures such as uvulectomy or tonsillectomy Those few patients with the most severe hypersomnia and cardiopulmonary failure may require tracheostomy and nocturnal respirator care (See Parkes for a full account of therapeutic measures) Some patients with nonobstructive apnea may also bene t from nighttime treatment with CPAP, but the results are far less consistent than with the obstructive type.

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