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Peculiar snoring in patients with multiple system atrophy: its sound source, acoustic characteristics, and diagnostic significance.

It is known that abductor paralysis (AP) of the vocal folds sometimes occurs in patients with multiple system atrophy (MSA), and some of them have sleep apnea and loud snoring during sleep. However, the site of obstruction and the sound source of the snoring are still unknown. We performed fiberscopic examinations under diazepam sedation in 8 MSA patients with AP and analyzed the snoring sound. We found that the peculiar snoring occurred with inspiratory vibration of the vocal folds, and there was no obstruction in this portion. Acoustic analysis showed that the fundamental frequency of vocal fold snoring was 260 to 330 Hz, which is different from that of ordinary soft palate snoring. Recognition of vocal fold snoring is important in the early diagnosis of MSA and sleep-related breathing disorders.

Adult↗

[A case of acute spinal epidural hematoma in a patient with antiplatelet therapy].

The authors report a case of acute spinal epidural hematoma occurring in a patient receiving antiplatelet drugs. A 76-year-old man with a history of cerebral infarction had been taking antiplatelet agents for one year. He suddenly developed severe back pain which woke him from sleep, and numbness of his lower extremities was then noted. He was hospitalized 15 hours later. Neurological examination revealed flaccid paralysis of both lower extremities with negative Babinski's reflex, and sensory disturbance below the level of L1. The bleeding time and prothrombin time were prolonged. Computed tomographic (CT) scan revealed a biconvex, relatively hyperdense mass in the posterior spinal canal at the level of T12. Metrizamide myelography disclosed an incomplete blockage caused by an epidural mass at the level of T11. Post-myelographic CT scan demonstrated a sharply demarcated extradural filling defect at the level of T11. Seventeen hours after the onset of symptoms, an emergency laminectomy was performed extending from T12 to L3, and the epidural clot was totally evacuated. Histological examination of the capsule of the hematoma revealed no vascular anomalies. The patient made a good postoperative recovery. To the authors' knowledge, this is the first reported case of spontaneous intraspinal hemorrhage in a patient taking antiplatelet drugs. Acute onset of persistent pain anywhere along the spinal axis and the development of spinal neurological deficits in a patient on antiplatelet therapy should raise the suspicion of a spinal epidural hematoma. It should be stressed that prompt neuroradiological diagnosis and rapid surgical decompression are essential to allow good recovery. The present case illustrates that neurological emergencies can occur in patients receiving antiplatelet therapy.

Acute Disease↗

[Facial paraspasm].

Thirty-two patients with facial paraspasm were subjected to comprehensive examination with the use of psychometric and electropolygraphic methods, with the brain awake and asleep. On the basis of the results obtained the author considers clinical differential diagnostic criteria, as well a possible neurodynamic substrate of the above syndrome. Many levels of the nervous system, particularly nonspecific formations of pontomesencephalic portions of the brain stem closely linked with subcortical and rhinencephalic structures, are involved in ensuring this substrate.

Adult↗

[Sleep disorders in multiple system atrophy].

Sleep disorders are so common in multiple system atrophy that they should be considered an integral part of the disease. Sleep fragmentation occurs in more than half of the patients, with sleep onset and sleep maintenance insomnia. Periodic leg movements of sleep are also common, although they are generally asymptomatic. REM sleep behavior disorder, a parasomnia in which the patient presents vigorous movements associated with intense dreaming dreaming during REM sleep, is detected polysomnographically in almost all the patients with variable severity. Nocturnal stridor is produced by obstruction on the glottis level. The cause of stridor is unknown but two alternative explanations have been proposed: paralysis of the muscles opening the vocal chords and excessive contraction of the muscles that close them. A combination of both, however, is the most likely explanation. Nocturnal stridor is associated with decreased survival and its treatment is based on continuous positive air pressure (CPAP) when it occurs only during sleep or tracheostomy when it worsens and becomes also diurnal.

Humans↗

Andersen syndrome: an association of periodic paralysis, cardiac arrhythmia and dysmorphic abnormalities.

Andersen syndrome (AS) is a rare disease characterized by the presence of periodic paralysis (PP), cardiac arrhythmia and dysmorphic abnormalities. We report herein the first Brazilian patient presenting AS who also had obesity, obstructive sleep apnea (OSA) and daytime sleepiness. Clinical and genetic evaluation of six family members demonstrated that four had dysmorphic abnormalities but none had PP or cardiac arrhythmia. Sequencing of KCNJ2 revealed the R218W mutation in the index patient and her 6-year-old daughter, who presented dysmorphic abnormalities (micrognathia, clinodactyly of fourth and fifth fingers, short stature) and OSA. Three relatives had clinodactyly as the only manifestation but the R218W mutation was absent, suggesting that this characteristic may be influenced by another gene. OSA accompanied by dysmorphic features may be related to AS.

Adult↗

[Acute sciatic neuropathy--"post-Saturday palsy"].

This is a case report of 25-year old, unemployed male, admitted to hospital due to acute onset of the left foot drop, subsequent walking difficulty and numbness of the left calf and foot. Symptoms began after prolonged sleep with previous heroin abuse by sniffing. During neurological examination, mild weakness of knee flexors, moderate weakness of plantar flexors and paralysis of foot dorsiflexors, together with hypesthesia of the left calf, foot and fingers, predominantly in the innervation area of common peroneal nerve on the same side, were observed. The electrophysiologic examination revealed predominant involvement of peroneal division within the sciatic nerve, together with recorded conduction block indicating the compression as possible mechanism of nerve injury. The patient was administered corticosteroid therapy during two months, what resulted in almost complete recovery. The peculiarity of this case report is in the presence of the sciatic nerve "Saturday night palsy" with possible effect of former heroin abuse.

Acute Disease↗

Neuropsychological findings in hyperthyroid patients.

AIM: to evaluate attention, concentration, memory changes and executive function inpatients with hyperthyroidism compared with normal thyroid function patients. METHODS: a cross sectional study was conducted in 21 patients (14F, 7M) with symptoms of sweating, sleep disturbance, fine tremor, headache, palpitation, nervousness, forgetfulness and diarrhea. Neurological examination found that 2 patients had proximal paresis and periodic paralysis. Their thyroid functions (FT4 and TSHS) were done, 13 patients were hyperthyroid, 2 patients were subclinical hyperthyroid and 6 patients were euthyroid. Neuropsychological tests such as: Mini Mental State Examination (MMSE), Trail Making Test (TMT) A and B, Continuous Performance Test (CPT), Vigilance Complex Task (VCT), Digit Forward and Backward Repetition (DFR, DBR) were performed in all of those patients. The mean age of sample was 47,3 +/- 8,45 years old and all of them had minimal 6 years education background, no history of cerebro vascular disease, brain injury or tumour or infection, epilepsy, cerebral degeneration, and taking neuroleptics or antidepressants. RESULTS: using the Kendall correlation coefficients showed that FT4 had negative correlation with VCT, CPT, DFR, DBR and positive correlation with TMT A (time) and TMT B (time and error). Study T-test showed that high level of thyroid function had significant difference in TMT A (time), TMT B (time), DBR, DFR, VCT, CPT. Fischer exact gave statistically significant in VCT and fine tremor in hyperthyroid patients (RR VCT 5.60, 95% CI 0.93-33.68, RR fine tremor 0.50, 95% CI 0.28-0.88). CONCLUSION: hyperthyroid patients had significant decrease on attention, concentration, verbal memory and executive function (working memory) compared with euthyroid patients.

Adult↗

Amyotrophic lateral sclerosis presenting with sleep hypopnea syndrome.

Described is a 67-year-old man whose initial symptoms evoked an obesity-hypoventilation syndrome. Polysomnography showed hypopneas associated with O2 desaturation episodes, and no apnea; maximal changes were noted during REM sleep. A few months later, in spite of marked weight loss, acute alveolar hypoventilation occurred and necessitated mechanical ventilatory support. Tracheostomy was performed. The patient appeared to be dependent on nocturnal ventilatory assistance. Diaphragmatic paralysis was noted in addition to clinical and electrodiagnostic evidence of amyotrophic lateral sclerosis. While the patient was not ventilated, a nocturnal recording of SaO2 again revealed desaturation episodes partly corrected by O2 2 L/min administered through the tracheostomy tube. With volume-controlled ventilation, desaturations completely disappeared, although no oxygen enrichment of the air was provided. We speculate that sleep disorders with hypopneas and O2 desaturation episodes were the initial symptoms of amyotrophic lateral sclerosis. This leads us to suggest that nonspecific respiratory muscle fatigue frequently seen in COPD might be included in the hypothetic causes of nocturnal hypoxemia.

Aged↗

Sudden infant death syndrome. The role of putrefactive toxins in respiratory paralysis and cerebral coma.

The Sudden Infant Death Syndrome (SIDS) is responsible for the death of 10,000 infants annually in the U.S.A. The cause of these deaths is not known; all SIDS infants studied died during sleep in a silent fashion and there are no specific lesions observable post mortem. It may be of significance, however, that nearly half of SIDS infants had a respiratory tract infection in the last two weeks of life while forty percent had bloody froth over their mouths when found, presumably pulmonary oedema fluid. It is hypothesis that improper feeding or diarrhoea may likewise be involved in SIDS and that enterotoxins from putrefactive products are the precipitating event in this syndrome.

Bacterial Toxins↗

[A case of Guillain-Barrè syndrome with severe sciatica preceding motor paralysis in the lower extremities].

A 62-year-old man was referred to our clinic for a three-day history of intractable sciatic pain. The pain was so severe that he could not sleep for days. Initially there was no neuronal sign except for the pain, nor any abnormality in lumbar CT and MRI. Cerebrospinal fluid sampled on the third hospital day revealed elevated protein (192 mg.dl-1) with minimal cells. On the 4th hospital day motor weakness of lower extremities appeared and he developed complete flaccid paraplegia in a week without loss of sensation. He was diagnosed as Guillain-Barrè syndrome. For the relief of this pain, intermittent injection of 4 ml 0.25% bupivacaine from lumbar epidural catheter was started on the admission day. By using patient controlled analgesia system, we could treat his repetitive demand timely and well control the pain. The leg weakness started to recover slowly from the 13th hospital day and at the same time the pain started to diminish rapidly in a few days. From the 20th hospital day he started to have rehabilitation in walking and recovered completely in two months without complication. We should remember that sudden onset of severe pain is one of early symptoms of Guillain-Barrè syndrome.

Analgesia, Epidural↗

Upper airway obstruction in infectious mononucleosis.

Life-threatening upper respiratory obstruction is an unusual complication of infectious mononucleosis. Although the majority of fatalities result from progressive bulbar paralysis or the Guillain-Barré syndrome, airway impairment primarily occurs as a result of pharyngeal lymphoid hyperplasia and associated faucial arch edema. Recent experience in a young child with infectious mononucleosis who exhibited progressive hypersomnolence, sleep apnea, and stridor during sleep is presented. In addition, a retrospective analysis of 72 cases of respiratory complications of infectious mononucleosis provides guidelines for specific airway management. Mild upper respiratory obstruction with persistent fever, severe odynophagia, and malaise is treated with parenteral corticosteroids. Immediate tonsillectomy using a halothane and oxygen induction technique is recommended for severe airway occlusion. Tracheotomy is currently reserved for those patients with progressive alveolar hypoventilation, hypercarbia, atelectasis, and bulbar paralysis. In general, tonsillectomy is well-tolerated, eliminating airway obstruction, improving swallowing function, and rapidly resolving pharyngeal discomfort.

Adrenal Cortex Hormones↗

Permanent tracheostomy.

A technique designed to create a permanent, wide open, and stable tracheal stoma is based on two corresponding U-shaped flaps: one from the anterior tracheal wall, the other from the skin in the suprasternal notch. The method described in this article has proved to be efficient, practical, well tolerated, and readily reversible. Its applications when indicated may reduce the side effects and complications associated with tracheostomy. Our clinical experience with this technique covers eight patients who tolerated the procedure well. The follow-up period exceeds 2 years.

Adult↗

Vocal cord paralysis in the Shy-Drager syndrome.

Eight out of 12 unselected patients with Shy-Drager syndrome were found to have severe bilateral paresis of vocal cord abduction by fibre-optic laryngoscopy. This commonly presented as increased snoring followed by episodes of inspiratory and expiratory stridor and sometimes by sleep apnoea. Respiratory failure eventually developed in four cases and was reversed by tracheostomy. In another patient tracheostomy relieved severe attacks of sleep apnoea. This complication was not necessarily associated with advanced disease, and it should be considered in all patients with Shy-Drager syndrome as appropriate treatment can lead to a useful extension of life.

Aged↗

Vocal cord paralysis as a presenting sign in the Shy-Drager syndrome.

A 70-year-old patient with bilateral vocal cord paralysis, accompanied by inspiratory stridor and increased snoring, is presented. Respiratory dysfunction, deteriorating over 18 months, necessitated permanent tracheostomy. Only two and a half years after the onset of symptoms the patient developed extrapyramidal signs combined with severe autonomic failure, suggesting a diagnosis of Shy-Drager syndrome. Vocal cord palsy preceding any other neurological or autonomic manifestations of that syndrome has been infrequently described. This diagnosis should be considered in cases of vocal cord palsy of undetermined etiology, especially when associated with increased snoring or episodes of sleep apnea.

Aged↗