1/23. Evaluation of sleep apnea syndrome with low-field magnetic resonance fluoroscopy.The aim of this study was to assess the upper airway status of sleep apnea syndrome (SAS) patients with low-field MR fluoroscopy. Twenty patients with clinically diagnosed SAS underwent upper airway monitoring using MR fluoroscopy for 5 min while awake and for 30 min while asleep. A 0.064-T permanent-magnet MR imaging system was used for the study. No patients required any sedative drugs because of the very small gradient noise, except in one case. No occlusion was observed while patients were awake. Nine patients showed repeated occlusion at retropalatal (Rp) pharynx, whereas 11 demonstrated both simple Rp occlusion and combined retropalato-retroglossal (Rp Rg) occlusions (complex occlusion). The mean frequency of occlusion in complex cases was significantly higher than that in simple Rp cases (p < 0.05). Low-field MR fluoroscopy was useful in determining the occlusion level while asleep in patients with SAS because of its quiet gantry and long-term monitoring capability. The MR fluoroscopy technique should prove to be a valuable clinical tool for the diagnosis and for determining the appropriate therapy in patients with SAS.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/23. Congenital central hypoventilation syndrome associated with Hirschsprung's disease: mutation analysis of the RET and endothelin-signaling pathways.Three cases of congenital central hypoventilation syndrome (CCHS) associated with Hirschsprung's disease (HSCR) were examined with respect to their genomic dna on the coding region of the receptor tyrosine kinase (RET) and the endothelin-B receptor (EDNRB). No causative mutations for the disease were detected, but one polymorphism was observed in exon 11 of the RET proto-oncogene. In cases with CCHS, HSCR occurs with a high incidence, and this disease complex has been described as neurocristopathy due to aberrations in neural crest cell proliferation, differentiation or migration during the early fetal period. Both the RET and EDNRB may play important roles in the modulation of neurocristopathies; however, further systemic studies in a large population of patients and control subjects are necessary for elucidating the pathogenesis of this disorder.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
3/23. narcolepsy in the older adult: epidemiology, diagnosis and management.narcolepsy is a disorder of impaired expression of wakefulness and rapid-eye-movement (REM) sleep. This manifests as excessive daytime sleepiness and expression of individual physiological correlates of REM sleep that include cataplexy and sleep paralysis (REM sleep atonia intruding into wakefulness), impaired maintenance of REM sleep atonia (e.g. REM sleep behaviour disorder [RBD]), and dream imagery intruding into wakefulness (e.g. hypnagogic and hypnopompic hallucinations). Excessive sleepiness typically begins in the second or third decade followed by expression of auxiliary symptoms. Only cataplexy exhibits a high specificity for diagnosis of narcolepsy. While the natural history is poorly defined, narcolepsy appears to be lifelong but not progressive. Mild disease severity, misdiagnoses or long delays in cataplexy expression often cause long intervals between symptom onset, presentation and diagnosis. Only 15-30% of narcoleptic individuals are ever diagnosed or treated, and nearly half first present for diagnosis after the age of 40 years. attention to periodic leg movements (PLM), sleep apnoea and RBD is particularly important in the management of the older narcoleptic patient, in whom these conditions are more likely to occur. diagnosis requires nocturnal polysomnography (NPSG) followed by multiple sleep latency testing (MSLT). The NPSG of a narcoleptic patient may be totally normal, or demonstrate the patient has a short nocturnal REM sleep latency, exhibits unexplained arousals or PLM. The MSLT diagnostic criteria for narcolepsy include short sleep latencies (<8 minutes) and at least two naps with sleep-onset REM sleep. Treatment includes counselling as to the chronic nature of narcolepsy, the potential for developing further symptoms reflective of REM sleep dyscontrol, and the hazards associated with driving and operating machinery. Elderly narcoleptic patients, despite age-related decrements in sleep quality, are generally less sleepy and less likely to evidence REM sleep dyscontrol. Nonpharmacological management also includes maintenance of a strict wake-sleep schedule, good sleep hygiene, the benefits of afternoon naps and a programme of regular exercise. Thereafter, treatment is highly individualised, depending on the severity of daytime sleepiness, cataplexy and sleep disruption. Wake-promoting agents include the traditional psychostimulants. More recently, treatment with the 'activating' antidepressants and the novel wake-promoting agent modafinil has been advocated. cataplexy is especially responsive to antidepressants which enhance synaptic levels of noradrenaline (norepinephrine) and/or serotonin. Obstructive sleep apnoea and PLMs are more common in narcolepsy and should be suspected when previously well controlled older narcolepsy patients exhibit a worsening of symptoms. The discovery that narcolepsy/cataplexy results from the absence of neuroexcitatory properties of the hypothalamic hypocretin-peptidergic system will significantly advance understanding and treatment of the symptom complex in the future.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
4/23. Leak of the bypassed stomach after laparoscopic gastric bypass, presenting as abdominal wall cellulitis.The development of surgical staplers devised for laparoscopy has enabled advancements in complex laparoscopic procedures, such as gastric bypass. This procedure, considered by many as the gold standard for bariatric surgery, is now frequently performed laparoscopically, with the advantages inherent in the minimally invasive approach. Technical failure of surgical staplers is, however, a well known complication of these devices in digestive surgery. We report the case of a leak of the bypassed stomach into the abdominal wall through a trocar site following laparoscopic gastric bypass. The mechanisms responsible for this life-threatening complication and the options to avoid it are discussed.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
5/23. Sleep-disordered breathing in mobius syndrome.mobius syndrome is a complex neurologic disorder characterized by congenital bilateral facial paralysis associated with lateral gaze paralysis. The syndrome has variable manifestations and several variants, some with somatic stigmata. In 1990, mobius syndrome is conceptualized as a spectrum of clinical caudal brain-stem signs. Some deficits are manifested by laryngeal paralysis and aspiration. Sleep-disordered breathing syndromes have not been previously reported in association with mobius syndrome. We report two children with Mobius syndrome and sleep-disordered breathing. Based on known pathologic findings and clinical manifestations, we believe that sleep-disordered breathing may be a common complication of mobius syndrome and should be sought, since potential outcomes of such complications include serious morbidity.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
6/23. Videoradiography at submental electrical stimulation during apnea in obstructive sleep apnea syndrome. A case report.Percutaneous submental electrical stimulation during sleep may be a new therapeutic method for patients with obstructive sleep apnea syndrome (OSAS). Electrical stimulation to the submental region during obstructive apnea is reported to break the apnea without arousal and to diminish apneic index, time spent in apnea, and oxygen desaturation. The mode of breaking the apnea by electrical stimulation has not yet been shown. However, genioglossus is supposed to be the muscle responsible for breaking the apnea by forward movement of the tongue. To visualize the effect of submental electrical stimulation, one patient with severe OSAS has been examined with videoradiography. Submental electrical stimulation evoked an immediate complex muscle activity in the tongue, palate, and hyoid bone. This was followed by a forward movement of the tongue which consistently broke obstructive apnea without apparent arousal. time spent in apnea was diminished but intervals between apnea were not affected.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
7/23. Lipomas of the mesencephalic tectum and rostral pons associated with sleep apnea syndrome.Two patients with mixed sleep apnea and autopsy-documented lipomas of the mesencephalic tectum and rostral pons are presented. Microscopically, the locus ceruleus was unilaterally invaded by a tumor in one case and may have been compressed in the other. adipocytes and fibrous tissue were present adjacent to pial surfaces and around small blood vessels within the parenchyma. There was prominent astrogliosis in the adjacent neuropil. Although respiratory control is a complex, multifocal phenomena, these findings raise the possibility that the locus ceruleus or adjacent brain stem regions may be affected in some instances of sleep apnea.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
8/23. Complex partial status epilepticus: report of one case.Complex partial status epilepticus (CPSE) has rarely been reported in children. We experienced a 5-year-old girl having had an abrupt onset of complex partial seizure with a fluctuating state of consciousness and aphasia. Electroencephalogram revealed repetitive epileptiform discharges originating from bilateral temporal and parieto-occipital regions over the background of diffuse continuous slow activity. Computerized tomography of the brain showed mild atrophy without focal lesions. All the other studies including bacteriology, virology, toxicology and metabolic screening were unremarkable. Intravenous administration of diazepam was ineffective for recovery of consciousness level and cease of seizure activity. A lasting control of the status was not achieved until intravenous phenytoin and oral carbamazepine were added for one more weeks. Paroxysmal attacks of periodic apnea with subsequent hyperpneic movements occurred the fourth month after the onset of illness. She regained language on rehabilitation program. Unfortunately, the patient had recurrence four months later despite medication. Finally, she died of aspiration pneumonia and status epilepticus eleven months after the onset of the disease. Compared with the other previously documented cases of prolonged complex partial status epilepticus, this case is notable for its unusual, complicated and severe symptomatology and long duration (three more months) with poor prognosis.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
9/23. Familial 'sleep apnea plus' syndrome: report of a family.We describe a familial disorder consisting of sleep apnea, anosmia, colorblindness, partial complex seizures, and cognitive dysfunction. The phenotypic expression of the syndrome suggests an autosomal dominant inheritance with incomplete penetrance.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
10/23. Silence as resistance to medical intervention.A group of 47 individuals initially silent about their physical symptoms and/or dysfunction were studied after they finally had come to medical attention. They were then interviewed, using the associative anamnesis technique, to evaluate the psychologic aspects of their silence. It was found to be a key element in the defensive complex directed against intense neurotic feelings activated by the patient's current physical condition. The tendency toward silence appeared to have been reinforced by emotionally traumatic experiences involving physical illness or injuries in early life. Also found associated with silence were such different factors as a series of ostrich-like denying rationalizations, a fanatic commitment to cultist beliefs, a conviction that emotional conflict is the primary cause of organic illness, and motivations linked to retention of power in reality situations. Clinical examples drawn from these categories are given. Means of increasing the awareness that such silence exists and methods of dealing with it are discussed.- - - - - - - - - - ranking = 0.5keywords = complex (Clic here for more details about this article) |
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