Abstracts of 3rd International Congress of the Association of Sleep Medicine (WASM) / Sleep Medicine 10, Suppl. 2 (2009) S1–S83
Conclusions: The mean hours of sleep for this group was coincident with the mean hours of sleep needed for the Brazilian general population, supporting published literature. In addition to being under the effects of a rotating shift work system, these train drivers probably experience alterations in sleep architecture due to the aging process, as decreased REM proportion, sleep fragmentation and decreased sleep subjective perception were similar to that of the Brazilian population, in general. We suggest that more prospective studies should be conducted to avoid an increased risk of accidents in these workers.
NEUROCOGNITIVE FUNCTIONS IN CHILDREN WITH SLEEP DISORDER BREATHING (SDB) AND OBESITY
M.C. Paolino, R. Castaldo, A. Urbano, A. Crescenzi, F. Ianniello, S. Miano, M.P. Villa. Department of Pediatric, Sleep Disease Centre, University of Rome La Sapienza, S.Andrea Hospital, Rome, Italy Introduction: There are evidences that children with sleep disorder breathing (SDB) show neurobehavioral disorders, such as hyperactivity, daytime sleepiness, learning diﬃculties and attention deﬁcit. Some evidence demonstrates that obesity could be associated with some neurocognitive anomalies. Because obesity and SDB can coexist, we evaluated four distinct populations, including obese subjects, SDB subjects, children with SDB and obesity and control children. Methods: We included 173 children matched for age and sex (53.2% males, mean age 9.4±2.3 years, BMI 19.5±4.2kg/m2 ), 79 healthy control subjects, 39 with SDB, 23 with SDB and obesity and 32 with only obesity. All patients underwent a polysomnographic study. Neurocognitive abilities were evaluated in all subjects using the Welchsler Intelligence Scale for Children (WISC-r). Attention and hyperactivity were examined using a standardized questionnaire (SDAG from DSM-IV 1994). Results: Children with SDB and obesity had the lowest values of verbal (VIQ) (90.74±13.4), performance (PIQ) (98.74±10.1) and total IQ (TIQ) (93.91±9.5) compared with healthy children (respectively, 115.84±10.6; 118.24±11.8; 110.08±11.2), not obese SDB (96.51±13.2; 100.36±15.3; 97.87±12.9) and obese children (115.22±14.1; 99.81±11.8; 109.78±13.1) (p<0.001, ANOVA). There was no signiﬁcant correlation between the cognitive scores and severity or duration of the sleep breathing disease. Conclusion: Children with SDB and obesity seem to be more vulnerable to neurocognitive anomalies.
VIDEO-POLYSOMNOGRAPHIC ASPECTS OF OBSTRUCTIVE SLEEP APNEA (OSA) IN ISAAC’S SYNDROME
G.C. Pinnola, M.D. Silva. Associação das Pioneiras Sociais Introduction: Isaacs’ Syndrome is a rare voltage-gated potassium channelopathy characterized by muscular stiffness, even during periods of sleep. On electromyographies, there is continuous muscle ﬁber activity even after the blockade of peripheral nerve innervation by lidocaine. Obstructive sleep apnea syndrome (OSA) is characterized by repetitive episodes of partial or complete obstruction of the upper airway caused by pharyngeal muscles collapse. There are no reports in the literature (PubMed) about the relationship between Isaac’s Syndrome and OSA. Objective: To report polysomnographics aspects of OSA in a patient with Isaac’s Syndrome. Patient and Methods: A 16-year-old boy complaining of progressive rigidity, mainly in the distal limbs and muscular pain at night, frequent snoring and breathing pauses was observed. On examination, the patient presented a Herculean appearance, paraparetic gait, hypertrophied musculature, and diﬃculty opening the eyes and hands. Electromyography from the abductor digiti minimi and abductor pollicis brevis showed continuous muscle ﬁber activity even after blockade of the right brachial plexus with 2% lidocaine. Results: The polysomnographic study revealed macrostructural alterations during sleep, a moderate degree of obstructive apnea and increased and persistent muscle contraction during all sleep phases, including during REM. After nine weeks of speciﬁc treatment for Isaac’s Syndrome, a new polysomnographic study showed improved sleep macrostructure and a drop in the obstructive apnea index from a moderate to light degree, however, the muscle tonus supported increased inclusion during REM sleep. Conclusion: Our paper demonstrates improved respiration index despite persistant increase in muscle tonus in a patient with Isaac’s Syndrome and OSA undergoing speciﬁc treatment for Isaacs‘ Syndrome. These ﬁndings indicate a possible relationship between both diseases and identify OSA as a possible co-morbidity factor. Persistent muscle contraction during sleep, even during REM, suggests that this technique may be used as a diagnostic tool to distinguish Isaac’s Syndrome from REM Behavior Disorders (RBD).
MANDIBULAR HYPOPLASIA CAUSING OBSTRUCTIVE SLEEP APNEA WITH PULMONARY HYPERTENSION IN FYBRODISPLASIA OSSIFICANS PROGRESSIVA
G.C. Pinnola, D.R. Carvalho. Associação das Pioneiras Sociais
EVALUATION OF THE UPPER AIRWAY BY MULTI SLICE CT IN PATIENTS WITH OSA
U.L. Passos 1 , B.F. Marcondes 2, G. Lorenzi 2 , R.L.E. Gomes 1 , G.G. Cerri 1 , E.M.M.S. Gebrim 1 . 1 HCFMUSP; 2 INCOR Purpose: To evaluate the changes in soft tissue structures surrounding the upper airway during wakefulness and sleep in patients with obstructive sleep apnea syndrome (OSA). Methods and Materials: Patients with severe OSA (apnea hypoapnea-index, AHI >30/h) were evaluated by 16-channel, multidetector computed tomography (MDCT) and simultaneous polysomnography. The exam was ﬁrst carried out while the patient was awake and then during natural sleep without sedation. Airway and soft-tissue 2-D measurements were performed on the retropalatal (RP) and retroglossal (RG) levels; 3-D volumetric measurements were also done. Results: We studied 10 patients, 9 of whom were male (age=57.7, dp: 8.5, BMI=34.5, dp:8.1, AHI=79.5, dp:47.4). The area measured during wakefulness was reduced during sleep by 54.9% on the RP (p=.001) level, with signiﬁcant reduction in both transversal (35.9%, p=0.006) and antero-posterior (39%, p=0.001) dimensions. In contrast, there were no signiﬁcant changes on the RG level (18.5%, p:0.286) or in airway volume measurements (8.48%, p:0.262). Conclusion: These results demonstrated signiﬁcant changes in the upper airways during sleep in patients with OSA, with a greater variability in the RP area.
Introduction: Fibrodysplasia ossiﬁcans progressiva (FOP) is an autosomal dominant genetic disorder of connective tissue that causes heterotopic ossiﬁcation (HO) and developmental skeletal defects. Among the craniofacial abnormalities in FOP, mandibular hypoplasia can develop during the skeletal maturation. Cardiopulmonary complications are often described in FOP patients as a consequence of restrictive chest wall disease. Pulmonary hypertension (PH) is considered an important ﬁnding in adult life, but the exact mechanism of this complication has not been identiﬁed. Although other causes of PH have not been considered in FOP, sleep studies of affected patients have not been performed. Objective: To report a case of obstructive sleep apnea (OSA) with secondary PH in a boy with FOP and suggest a possible relationship with cardiopulmonary complications. Patients and Methods: FOP was conﬁrmed in the boy by detection of mutation in the ACRV1 gene by RFLP analysis. Snoring and pauses in breathing were observed during sleep. He also complained of nonrestorative sleep and daytime sleepiness. Cephalometry CT scan supported the micrognatism. Echocardiography revealed an elevated pulmonary artery pressure. The polysomnography (PSG) showed a severe OSA. Three months later, after treatment with BiPAP during sleep, he did not indicate sleepiness or daytime naps. Subsequent echocardiography showed a normal pulmonary artery pressure. Discussion: OSA can provoke asymptomatic degrees of PH and should be treated in childhood. In clinical practice, cardiopulmonary symptoms are not frequent in FOP. Nevertheless, the main cause of death in FOP in adult life is progressive right-sided heart failure. Our report also suggests that PH could initially progress in FOP from adolescence as a consequence of OSA. Normal values of pulmonary artery pressure after BiPAP treatment denoted a possible relationship. The cause of micrognathia is not usually discussed. Some authors suggest that micrognatia could appear even without ankylosis of the temporal madibular joint or involvement of submandibular region by