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Central and peripheral respiratory electrophysiological studies in myotonic dystrophy.

Acute and chronic respiratory failure is a common and potentially life-threatening feature in patients with myotonic dystrophy (MD). The causes may be varied, and can involve both the central and peripheral nervous system. To evaluate the incidence of respiratory muscle involvement and the function of the central motor inspiratory pathway to phrenic motor neurons we performed magnetic stimulation of the cortex and cervical spinal cord, phrenic nerve conduction studies and needle EMG of diaphragm and intercostal muscles in 25 patients with MD. The results were compared with those from 35 healthy subjects. In addition, pulmonary function tests, blood gas analyses and static mouth pressures were evaluated. Abnormalities in response to magnetic stimulation, including a reduced compound muscle action potential (CMAP) from the diaphragm and increased excitability threshold, indicated impaired central inspiratory drive in 20% of cases. Phrenic nerve conduction showed a reduced diaphragmatic CMAP amplitude in 20%, and a delayed negative peak onset latency in 4% of cases. Abnormalities in diaphragm and intercostal muscle needle EMG were found in 76% of cases, these were mainly myotonic discharges (68%) and a decrease in the number of active motor units (36%). Patients with abnormal respiratory electrophysiological parameters had a significantly lower functional vital capacity (FVC; P = 0.005). The duration of the disease correlated negatively with diaphragmatic CMAP amplitude to phrenic nerve, but not magnetic, stimulation. Our results demonstrate that the involvement of the central inspiratory pathway is common in MD patients. Central and peripheral electrophysiological studies of the diaphragm should be considered in the diagnosis and management of patients with MD and dyspnoea.

Adult↗

Clinical characteristics of acute dysphagia in pediatric patients following traumatic brain injury.

PRIMARY OBJECTIVE: To document the clinical characteristics of acute dysphagia in a group of pediatric patients after traumatic brain injury (TBI). RESEARCH DESIGN: Prospective group study. METHODS: Fourteen subjects (7 males, 7 females), aged 4 years 1 month to 15 years, with moderate or severe TBI (Glasgow Coma Scale [GCS] < 12). Subjects were assessed via clinical bedside examination documenting cognitive status, oromotor function, feeding function, dietary recommendations, and an indication of overall feeding severity. RESULTS: A pattern of impaired cognition, altered behavior related to feeding, severe tonal and postural deficits, oromotor, respiratory, and laryngeal impairments, and oral sensitivity issues was revealed. CONCLUSIONS: Swallowing impairment was affected by multilevel deficits, which both individually and in combination had a negative impact on swallowing competence and safety. In light of deficits identified, which could not be observed on videofluoroscopic investigation alone, this study highlighted the importance of the clinical bedside examination in assessing dysphagia in pediatric patients post-TBI for identifying targets for intervention.

Acute Disease↗

The upper airway and sleep apnoea in the Prader-Willi syndrome.

Obesity, short stature, hypotonia and excessive daytime sleepiness are characteristic features of the Prader-Willi syndrome. Excessive daytime sleepiness has been attributed to obstructive sleep apnoea (OSA). To investigate the role of anatomical factors in OSA in the Prader-Willi syndrome, clinical and ENT assessment, radiology of the upper airway and polysomnography including sleep oximetry were done in 14 subjects. Excessive daytime sleepiness was present in eight of 14 subjects as determined by a mean sleep latency to non-rapid eye movement stage I-II of < 5 min and/or self-rating sleepiness score > 9 (Epworth Sleepiness scale). Seven subjects were snorers or mouth breathers and dental abnormalities were present in 11. Sleep apnoea, as determined by a combined apnoea-hypopnoea index of more than 10 respiratory events per hour was present in 12 of 14 subjects. On clinical assessment, the nasopharynx, oropharynx and hypopharynx were small in one subject. No subject had redundant pharyngeal mucosa or an enlarged tongue. However, radiological studies performed in the awake supine posture showed a slight reduction in the cross-sectional area in nine subjects at the oropharyngeal level and in four subjects at the nasopharyngeal level as compared with normal control subjects. Sleep apnoea and minor radiological evidence of narrowing of the upper airway are common in the Prader-Willi syndrome, although clinical otolaryngological examination is often unremarkable. Excessive daytime sleepiness occurs in approximately 50% of all patients with Prader-Willi syndrome. Although obstructive sleep apnoea is one important factor related to sleepiness, an additional central disturbance of sleep mechanisms is present.

Adult↗

Endometrial disease after treatment with oestrogens and progestogens in the climacteric.

A prospective study of 745 women receiving different regimens of hormone treatment for the climacteric for a total of 21 736 months was performed. There was a lower incidence of endometrial hyperplasia in biopsy specimens in the women receiving cyclical low-dose oestrogen by mouth than in those receiving cyclical high-dose oestrogen by mouth. The incidence of abnormalities in the women receiving sequential oestrogen and progestogen was lower than in either of these two groups. Among the women receiving subcutaneous oestrogen implants the incidence was higher still, but over half of the abnormal specimens were from women who had not taken their progestogen. The incidence of hyperplasia fell with longer courses of progestogen, and no hyperplasia was found in patients taking progestogen for over 10 days each month. The incidence of adenomatous and atypical hyperplasia is significantly reduced by a progestogen when taken for 10 or more days monthly. The absence of vaginal bleeding or of a regular bleeding response does not guarantee histologically normal endometrium in patients taking oestrogens without progestogen.

Climacteric↗

Tardive dyskinesia associated with fluoxetine.

Three cases in which patients who were taking fluoxetine for relief of depression showed patterns of abnormal movements suggestive of tardive dyskinesia are presented. In the first case, abnormal facial movements began four weeks after fluoxetine was added to doxepin and lithium and remitted after fluoxetine was discontinued. In the second case, abnormal movements of the mouth and hands were noticed four years after the patient started taking fluoxetine and continued to be present a year after withdrawal of the medication. In the third case, orofacial dyskinesia that had remitted after withdrawal of sertraline and paroxetine and reappeared with fluoxetine was still present eight months after fluoxetine was withdrawn.

Adult↗

[Thalidomide. Clinical trials in cancer].

The breakthrough discovery of the anti-angiogenic effects of thalidomide gave impetus to a series of clinical trials in patients with solid tumors and hematologic malignancies. Thalidomide has been shown to block the activity of angiogenic substances like bFGF, VEGF and interleukin 6. This drug also down-regulates TNF alpha. Thalidomide has shown clinical antitumor activity in single-agent, phase II clinical trials in AIDS-related Kaposi sarcoma, glioma, multiple myeloma refractory to chemotherapy, and hormone-refractory prostate cancer. In contrast, thalidomide was inactive in breast, lung and kidney cancer. The dose-limiting toxicity of thalidomide is sedation. Other adverse effects include skin rash, constipation, dry mouth and liver function abnormalities, along with peripheral neuropathy and the drug's well-known teratogenic potential. The advantages of thalidomide include the convenience of the oral route of administration, the drug's toxicity profile--substantially milder than that of chemotherapy--and its low cost. The potential role of thalidomide in the treatment of human neoplasia will be confirmed by means of randomized clinical trials.

Angiogenesis Inhibitors↗

[Problems posed by difficult intubations].

The difficulties of tracheal intubation are mainly encountered in facial surgery and E.N.T. surgery, but they may nevertheless, occur in any form of surgery. The indications for nasal intubation, without visible glottis, the problems raised and the means of solving them, whilst respecting safety precautions, are briefly recalled.

Cicatrix↗

[Diagnosis of pediatric obstructive sleep apnea hypopnea syndrome].

OBJECTIVE: To discuss the clinical feature and diagnosis of pediatric obstructive sleep apnea hypopnea syndrome (OSAHS) and analyze characteristics of polysomnography. METHODS: Cephalometric analysis, fibrous endoscopic examination and sleep breathing studies were performed in 49 cases with suspected complaints and signs. RESULTS: Most of them had marked history about snore, sleep apnea and signs of upper airway obstruction caused by enlargement of tonsil and/or adenoid. Polysomnography (PSG) was given to 41 cases who was proved 1 case with primary snore, 5 cases with upper airway resistance syndrome, 35 cases with OSAHS. Another 8 cases with OSAHS were diagnosed by Autoset I studies. CONCLUSION: Many features of pediatric OSAHS are different from adult OSAHS. The common symptom of pediatric OSAHS include snore, restless sleep, struggling to breathe, abnormal paradoxical chest/abdomen motion, mouth breathing, failure-to-thrive. Obesity and excessive daytime sleepiness are little present. Severe cases may be associated with cor pulmonale. PSG play an important role in exact diagnosis of pediatric sleep related breathing disorder. The results demonstrate that pediatric OSAHS are characterized by partial upper airway obstruction, more or less apnea and associated with staged desaturation. They have little sleep structure disorder and difficulty breathing are not associated with arousal. Apnea hypopnea index (AHI), lowest oxygen saturation (LSaO2) and desaturation index below 90% (SIT90%) are very important factors to measure about serious degree of pediatric OSAHS.

Child↗

[Clinical analysis of the results in 146 patients with benign tumors or malformations of the oral cavity treated by microwave].

To evaluate the clinical effect of microwave on commonly seen benign tumors or malformations of the oral cavity, 146 patients were randomly selected. There were 78 patients with mucous cyst, 22 patients with papilloma, 24 patients with hemangioma or malformation, 6 patients with lymphatic malformation and 16 patients with epulis. All the patients were treated by ECO-100 microwave. It is concluded that 126 patients were cured (86.3%), 20 patients had good response (13.7%) after treatment.

Hemangioma↗

[Animal models of tardive dyskinesia].

It is possible to induce in monkeys abnormal movements of the mouth and tongue resembling strikingly tardive dyskinesia. Such movements can be induced by chronic treatment with neuroleptics or by lesions placed in the habenular interpeduncular tract or nucleus parafascicularis thalami. Dopaminergic D1 and D2 receptors are involved in the modulation of such movements.

Animals↗