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Joan Santamaría

Publications and source records attributed to Joan Santamaría.

5 recordsLinked to original sources

Rapid eye movement sleep behavior disorder and potassium channel antibody-associated limbic encephalitis.

Of 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. Our findings suggest that RBD is frequent in the setting of voltage-gated potassium channel antibody-associated limbic encephalitis and can be related to autoimmune-mediated mechanisms. In addition, these observations suggest that impairment of the limbic system may play a role in the pathogenesis of RBD.

Aged↗

Rapid-eye-movement sleep behaviour disorder as an early marker for a neurodegenerative disorder: a descriptive study.

BACKGROUND: 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. We sought to determine the frequency and nature of neurological disorders developing in patients diagnosed with idiopathic RBD at our sleep centre. METHODS: We retrospectively assessed 44 consecutive patients (39 men and five women with a mean age of 74 years), with at least 2 years of clinical follow-up after a diagnosis of idiopathic RBD, through a detailed clinical history, complete neurological examination, rating scales of parkinsonism, and neuropsychological tests. FINDINGS: 20 (45%) patients developed a neurological disorder after a mean of 11.5 years from the reported onset of RBD and a mean follow-up of 5.1 years from the diagnosis of idiopathic RBD at our sleep centre. Emerging disorders were Parkinson's disease in nine patients, dementia with Lewy bodies in six, multiple system atrophy with predominant cerebellar syndrome in one, and mild cognitive impairment in four in whom visuospatial dysfunction was prominent. Patients with longer clinical follow-up developed a neurological disease (OR 1.512, 95% CI 1.105-2.069; p=0.010). INTERPRETATION: Our study indicates that in people presenting to sleep centres, RBD often antedates the development of a neurodegenerative disorder. Close follow-up of patients with idiopathic RBD could enable early detection of neurodegenerative disease. This finding may be of great interest when early effective treatment strategies and neuroprotective drugs become available.

Aged↗

Severe obstructive sleep apnea/hypopnea mimicking REM sleep behavior disorder.

OBJECTIVE: 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. PARTICIPANTS: Sixteen patients that were identified during routine first evaluation visits. Patients' PSG measures were compared with those of 20 healthy controls and 16 subjects with idiopathic RBD of similar age and sex distribution and apnea/hypopnea index lower than 10. INTERVENTIONS: NA. RESULTS: Sixteen subjects were identified presenting with dream-enacting behaviors and unpleasant dreams suggesting the diagnosis of RBD, in addition to snoring and excessive daytime sleepiness. VPSG excluded RBD showing REM sleep with atonia and without increased phasic EMG activity, and was diagnostic of severe OSAH with a mean apnea-hypopnea index of 67.5 +/- 18.7 (range, 41-105) demonstrating that the reported abnormal sleep behaviors occurred only during apnea-induced arousals. Continuous positive airway pressure therapy eliminated the abnormal behaviors, unpleasant dreams, snoring and daytime hypersomnolence. CONCLUSIONS: Our study shows that severe OSAH may mimick the symptoms of RBD and that VPSG is mandatory to establish the diagnosis of RBD, and identify or exclude other causes of dream-enacting behaviors.

Adult↗

[Narcolepsy].

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Humans↗

Usefulness of uvulopalatopharyngoplasty with genioglossus and hyoid advancement in the treatment of obstructive sleep apnea.

OBJECTIVE: To evaluate the usefulness of uvulopalatopharyngoplasty plus mandibular osteotomy with genioglossus and hyoid advancement in the treatment of obstructive sleep apnea syndrome (OSAS). DESIGN: Prospective study of 20 consecutive patients with OSAS. SETTING: University medical center. PATIENTS AND INTERVENTIONS: Twenty OSAS patients with multilevel upper airway obstruction who refused continuous positive airway pressure treatment. All patients were evaluated before and 6 months after surgery by clinical history, the Epworth Sleepiness Scale, physical examination, fiberoptic nasopharyngoscopy combined with the Müller maneuver, cephalometric analysis, nocturnal polysomnography, and a second-night polysomnography with upper airway pressure recording during sleep. Surgery procedures were uvulopalatopharyngoplasty plus mandibular osteotomy with genioglossus and hyoid advancement. Surgical successful outcome was defined as an apnea-hypopnea index (AHI) lower than 20 plus subjective resolution of daytime symptoms. MAIN OUTCOME MEASURE: Surgical success rate. RESULTS: Mean +/- SD AHI decreased from 60.5 +/- 16.5 to 44.6 +/- 27(P =.007), and CT90 (percentage of time with oxyhemoglobin saturation below 90%) decreased from 39.5% +/- 26% to 25.1% +/- 26.4% (P =.002). The overall surgical success rate was 35% but increased to 57% in patients with moderate OSAS (AHI, 41-60) and to 100% in mild OSAS (AHI, 21-40). In the group of severe OSAS, the success rate was 9%. Predictors of surgical outcome success were the AHI, CT90, stages 2 and 3-4 sleep percentages, and the cephalometric ANB angle (angle formed from the deepest point on the maxillary outer contour to the nasion to the deepest point on the outer mandibular contour). CONCLUSION: Patients with mild and moderate OSAS and multilevel obstruction in the upper airway may benefit from uvulopalatopharyngoplasty plus genioglossus and hyoid advancement.

Adult↗