Central Alveolar Hypoventilation and Sleep

central Alveolar Hypoventilation and Sleep Treatment by Intermittent PositivePressure Ventilation through Nasal Mask in an Adult

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IPPV = intermittent positive-pressure ventilation; MRI magnetic resonance imaging; PVC premature ventricular complexes; CPAP continuous positive-airway pressure

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diopath ic central alveolar hypoventilation syndrome is associated with sleep-related hypoventilation and central sleep apnea. We report on a patient who presented with significant cardiac arrhythmias and hemodynamic changes during sleep with this syndrome and the successful results of IPPV through a nose mask. CASE REPORT

A 48-year-old machine operator from Turkey, with no significant past medical history other than a 25-year habit of six packs of cigarettes per week, presented in 1983 with daytime fatigue, intermittent headaches, and unexplained hypercapnia. His untreated condition worsened. In 1987 the patient appeared excessively sleepy, not obese (height, 164 em: weight, 62 kg), with a complaint of severe morning headaches that partially cleared during the day. He reported disturbed nocturnal sleep but no history of snoring. He was neither confused nor disoriented, and he appropriately answered questions through an interpreter. A systematic Holter ECG showed unexplained nighttime bradycardiac episodes, and sleep apnea was observed clinically The patient's neurologic evaluation was within the normal range, and there was no muscle impairment. An EEG demonstrated no focal lesions or signs of encephalopathy. Magnetic resonance imaging (MRI) of cortical regions and brain stem areas was also negative. The patient had ten 24-h Holter monitor ECGs, a pulmonary evaluation including chest x-ray films, pulmonary function tests, and daytime effort testing. He underwent Swan-Ganz catheterization with investigation of right circulation while awake and asleep, as well as polygraphic monitoring during sleep. The investigation demonstrated for the first time the presence of daytime hypoxia and hypercapnia (Fig 1, A and B) unexplained by normal pulmonary evaluations. Arterial blood gas values while the patient was awake were easily identified from supine rest to effort while supine (Table 1). This indicates the possibility of fast normalization of blood gas abnormalities during effort despite a supine position. Pulmonary function tests, repeated *Stanford University School of Medicine, Palo Alto, CA. tMarburg University Medical School, Marburg, Federal Republic of Germany. This work was supported by the German Research Council (Deutsche Forschungsgemeinschaft) (D. F.G.).

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Christian Guilleminault, M.D.;* Riccardo Stoohs, M.D.;t Hartmut Schneider, M.D.;t Thomas Podszus, M.D.;t J Herman Peter, M.D.;t and Peter von Wichert, M.D.t

Idiopathic central alveolar hypoventilation, uncommonly seen in middle-aged adults, has often been treated by tracheostomy and assisted ventilation during sleep or by implantation of a diaphragmatic pacemaker with or without tracheostomy. We report the successful treatment of a middle-aged man by the easy application of intermittent positive-pressure ventilation through a nose mask. (Chest 1989; 96:1210-12)

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FIGURE 1 (A, upper) Hypercapnic (rebreathing technique) and (B,

lower) isocapnic hypoxic responses on an awake patient (closed

circles) and on 15 normal controls (open circles); all subjects were middle-aged men who were relaxed, seated, and monitored ident}cally. The patient's daytime responses were clearly abnormal. VE= minute ventilation; BSA = body surface area.

several times, eliminated restrictive or obstructive lung problems. Thoracoabdominal neuromuscular disorders were also eliminated (Table 1). Polygraphic monitoring during sleep included EEG, electrooculogram (EOG), chin electromyogram (EMG), and ECG. Respiration was monitored by respiratory inductive plethysmography, Central AlveolarHypoventilation and Sleep (Guillaminault at 8/)

Table 1- Variables Recorded Before Any Treatment Trial

Arterial blood gases Awake seated

75 59

65.8 75.3

Supine at rest Supine during 100-W effort Pulmonary function tests VC* FEV. FEV1NC Peak flow Residual volume TLC IVC Baseline polysomnography TST, min %NREM sleep %REM sleep No. of central apneas No. of central hypopneas %T<90% Sa02 Lowest Sa02 , %

Peo2 , mm Hg

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47 63 57.5 47.5

7.34 7.33 7.34 7.34

3,651 ml (87%) 3,123 ml (95%) 85% (109%)

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*Numbers in parentheses indicate normal values or percentage from the norms. airflow (thermistors), and ear oximetry (Biox-Ohmeda). Holter ECG and Swan-Ganz catheterization with monitoring of pulmonary arterial pressure (PAP) and intermittent investigation of wedge pressure (PawP) were performed during one night. The patient presented a disrupted nocturnal sleep. During sleep, there were repeated falls in oxygen saturation (SaOJ associated with repeated central apnea and hypopnea, with the lowest Sa02 monitored during rapid eye movement (REM) sleep (Table 1). Transcutaneous Pco, measurements over two nights indicated a progressive increase in CO 2 from a mean PtcC02 baseline of 51 mm Hg to 70 to 75 mm Hg during sleep. Two analyses of Holter ECG monitoring performed

in association with polygraphic monitoring during sleep indicated presence of significant cardiac arrhythmias in association with Sa02 falls. Moreover, the type of arrhythmia noted was strongly in8uenced by sleep state. During the first monitoring, 14 premature ventricular complexes (PVCs) were seen during nonrapid eye movement (NREM) sleep, while 44 sinus arrests were monitored during REM sleep. The sinus arrests oscillated between 3 and 11.6 s in duration and were entirely distributed during two REM sleep periods in the second half of the night (11 and 33 sinus arrests, respectively). During NREM sleep, 872 PVCs were monitored, with 49 couplets, 48 bigeminy-trigeminy, and 6 idioventricular rhythms, in association

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OmmHg FIGURE 2. Monitoring of cardiorespiratory variables during REM sleep. From top to bottom: airflow (monitored by thermistors); RC-Resp, inductive respiratory plethysmography, rib cage; Abd Resp, abdomen; Sa02 , ear oximetry; ECG; PPA, pulmonary arterial pressure. Note the II-s sinus arrest with simultaneous fall in PAP despite significant hypoxia.

CHEST I 96 I 5 I NOVEMBER, 1989

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with Sa02 falls during the second polygraphic recording, while 12 sinus pauses lasting between 3 and 11.3 s were noted during REM sleep. Investigation of the right circulation indicated near-normal wake pulmonary pressures (S/D PAP = 25110mm Hg; mean PAP = 15 mm Hg). During sleep there were fluctuations depending on changes in Sa02 , with occasional increases up to 60130 mm Hg. The most dramatic change was during the sinus arrest of 11 s, where mean PAP fell to 8 mm Hg during the entire asystole (Fig 2). Treatment Our investigation supported the diagnosis of primary central alveolar hypoventilation syndrome. The absence of neurologic findings, including negative findings at MRI, did not allow further definition of the syndrome. Nasal continuous positive-airway pressure (CPAP), with pressure up to 15 em H 20, administered during the first half of the night while simultaneously monitoring PA~ did not improve breathing and hemodynamic measurements. We decided to try nasal IPP\z The Respironics nose mask was used and IPPV administered using a MONNAL-D-Air Liquide Co. ventilator (13 breaths/min). The patient was followed up for seven nights in the hospital, was monitored for several nights with Holter ECG, and had a new polygraphic evaluation that included inductive respiratory plethysmography and measurement of Sa02 • Clinical complaints decreased drastically within days of the initiation of treatment. Cardiac arrhythmias were not found at Holter ECG recordings, but sinus arrest was noted during an early morning REM sleep at a time when the patient had pulled off his mask. Central apnea and hypopnea had completely disappeared during nocturnal monitoring, and IPPV continuously maintained Sa02 above 92 percent. Daytime arterial blood gas values registered Po 2 , 93; PCo2 , 39; and Sa02 • 95.2 percent. Within a week, the patient had complete control of his sleep-related syndrome and daytime symptoms and was discharged home. The maintenance of the favorable response was confirmed at a new polygraphic monitoring performed one month later. DISCUSSION

Central alveolar hypoventilation in adults can be seen secondary to neurologic lesions and impairment of central chemosensitivity, independent of its cause. 1-5 Our patient did not present any neurologic symptoms or positive anatomic findings at MRI to support any of these etiologies. He also did not fit the patterns noted in very obese patients, with or without associated obstructive sleep apnea, where a progressive blunting of hypoxic and hypercapnic responses in association with sleep disturbances combined with massive obesity is strongly suspected. The cause of the central alveolar hypoventilation remained unclear. The distribution and types of cardiac arrhythmias presented by our patient were of interest. Cardiac arrhythmias are often noted in association with sleep-related disordered breathing, but sinus arrest is predominantly associated with obstructive sleep apnea. Here, we observed a strong dichotomy between sinus arrest seen only in association with REM sleep central sleep apnea and other arrhythmias during NREM sleep central sleep apneas. There is a vagal dominance during tonic REM sleep, compared with wake and NRE M sleep, that is linked to the active disinhibition of brain stem structures associated with RE M sleep. This may explain the dichotomy noted. Also, to our knowledge, this was the first monitoring ever made of abrupt drops in PAP in association with an II-s sinus arrest during a central sleep apnea. This very significant fall was seen despite the 1212

associated hypoxia and hypercapnia. The mean PAP of 8 mm Hg during the sinus arrest is closely related to the PAP measured in dogs at the time of death (6 mm Hg). Whatever the underlying mechanisms, the frequency and duration of REM sleep-related sinus arrest were undoubtedly lifethreatening. Finally, our case report emphasizes a new therapeutic approach for central alveolar hypoventilation during sleep. IPPV through nasal mask has been widely used in Europe. Robert in Lyon (France) has over 100 patients with obstructive sleep apnea syndrome (OSAS), COPD and OSAS, muscle disorders, etc, currently being treated by this technique (personal communication March, 1988). Kerbyet al,6 and Carroll and Branthwaite," have also used IffV through a nose mask in patients with neuromuscular diseases and COPD, a protocol also used by Guilleminault, QueraSalva and Partinea in ten patients with progressive muscle diseases (unpublished data). IPPV via nasal mask has been reported only once, by Ellis et al," as a successful treatment of central alveolar hypoventilation syndrome in a 6-yea~old child. Our observations confirm the validity of this treatment in certain adult cases of primary central alveolar hypoventilation syndrome. Because of ease of application, this treatment should be considered before contemplating tracheostomy and assisted ventilation or diaphragmatic pacing. ACKNOWLEDGMENT: We thank Alison Grant for editing the manuscript. REFERENCES

1 Devereux M~ Kean JR, Davis RL. Autonomic respiratory failure associated with infarction of medulla. Arch Neurol 1973; 29:4652 2 Levin BE, Margolis G. Acute failure of automatic respiration secondary to unilateral brainstem infarct. Ann Neurol 1977; 1:583-86 3 Guilleminault C, Quera-Salva MA, Nino-Murcia G, Partinen M. Central sleep apnea and partial obstruction of the upper airway. Ann Neuroll987; 21:465-69 4 Dooling EC, Richardson E~ Ophthalmoplegia and Ondines curse. Arch Ophthalmol1977; 95:1790-93 5 Cummiskey J. Guilleminault C, Davis R, Duncan ~ Golden J. Automatic respiratory failure, sleep studies and Leigh's disease. Neurology 1987; 37:1876-78 6 Kerby GR, Mayer LS. Pingleton SK. Nocturnal positive pressure ventilation via nasal mask. Am Rev Respir Dis 1987; 135:738-40 7 Carroll N. Branthwaite MA. Control of nocturnal hypoventilation by nasal intermittent positive pressure ventilation. Thorax 1988; 43:349-53 8 Ellis ER, McCauley VB, Mellis C, Sullivan CEo Treatment of alveolar hypoventilation in a 6-year-old girl with intermittent positive pressure ventilation through a nose mask. Am Rev Respir Dis 1987; 136:188-91

Respiratory Failure due to Vocal Cord Dyskinesia in Olivo-PontoCerebellar Atrophy* Naoko Aragane, M.D.; Osamu Katoh, M.D., F.C.C.~; Hozumi Yamada, M.D., F.C.C.~; Yamo Kuroda, M.D.; and Tadatsugu Maeyama, M.D.t From the *Departments of Internal Medicine, and tOtology, Saga Medical School, Nabeshima, Saga, Japan. Reprint requests: Dr. Katoh, Department of Internal Medicine, Saga Medical School, Nabeshima, Saga, Japan 840-01 RespiratoryFailuredue to VocalCord Dyskinesia(Aragane at a/)