Cerebellum-related characteristics of Scn8a-mutant mice.

Among ten sodium channel alpha-subunit genes mapped in human and mouse genomes, the SCN8A gene is primarily expressed in neurons and glia. Mice with two types of Scn8a null mutations--Scn8a ( med ) and Scn8a ( medTg )--live for only 21-24 days, but those with incomplete mutations-Scn8a ( medJ ) and Scn8a ( medJo )--and those with knockout of Scn8a only in cerebellar Purkinje cells live to adult age. We review here previous work on cerebellum and related regions of Scn8a mutant mice and include some newer immunohistochemical and microchemical results. The resurgent sodium current that underlies the repeated firing of Purkinje cells is reduced in Scn8a mutant and knockout mice. Purkinje cells of mutant mice have greatly reduced spontaneous activity, as do the analogous cartwheel cells of the dorsal cochlear nucleus. Up-regulation of GABA(A) receptors in regions to which Purkinje cells project may partially compensate for their decreased activity in the mutant mice. The somata of cerebellar Purkinje cells of Scn8a ( medJ ) and Scn8a ( medJo ) mice, as revealed by PEP-19 immunoreaction, are slightly smaller than normal, and their axons, especially in Scn8a ( medJo ) mice, sometimes show enlargements similar to those in other types of mutant mice. Density of GABA-like immunoreactivity is decreased in Purkinje somata and regions of termination in deep cerebellar and vestibular nuclei of Scn8a ( medJ ) mice, but measured GABA concentration is not significantly reduced in microdissected samples of these regions. The concentrations of taurine and glutamine are significantly increased in cerebellar-related regions of Scn8a ( medJ ) mice, possibly suggesting up-regulation of glial amino acid metabolism.

Ultrasound-guided needle localization of recurrent paratracheal thyroid cancer.

OBJECTIVES: Ultrasound-guided needle localization techniques have been used to direct the resection of targeted axillary lymph nodes in the management of breast cancer. To date, there has been only one other description of this technique as a localization method to direct cancer resection in the neck. We offer further support for the broader application of this technique by reporting its use in the successful identification and resection of recurrent papillary thyroid cancer after a paratracheal node dissection failed to localize the cancer. METHODS: We report a case and discuss the relevant literature regarding ultrasound-guided localization and resection of recurrent well-differentiated thyroid cancer. RESULTS: We were able to achieve successful identification and resection of recurrent papillary thyroid cancer using this technique. CONCLUSIONS: This technique may be useful in the treatment of selected cases of recurrent thyroid cancer to increase the efficacy and safety of surgical resection.

Physician specialty is associated with differences in the evaluation and management of acute bacterial rhinosinusitis.

BACKGROUND: Acute bacterial rhinosinusitis (ABRS) is a common illness that is routinely managed by physicians from several different specialties. However, the actual diagnostic and treatment preferences of physicians from these different specialties are not known. OBJECTIVE: To determine whether the radiographic evaluation and management of community-acquired ABRS differs according to medical specialty. DESIGN, SETTING, AND PARTICIPANTS: Randomized survey of 450 board-certified physicians in the United States from family medicine, general internal medicine, and otolaryngology. MAIN OUTCOME MEASURES: Responding physicians' use of diagnostic radiography as well as choice and duration of antimicrobial and adjunctive treatments of ABRS. RESULTS: Otolaryngologists were more likely to use supportive diagnostic radiography (P =.04). They were also more likely to treat patients with adjunctive therapy, such as topical decongestants (P =.01), guaifenesin (P =.01), and saline nasal irrigation (P =.01), in addition to antibiotics. Otolaryngologists prescribed more medications to treat patients with ABRS than primary care physicians (P =.01). There were no significant differences in diagnosis and management by family physicians and general internists. CONCLUSIONS: Otolaryngologists use more health care resources to diagnose and treat ABRS than primary care physicians despite an absence of evidence that such tests and treatments lead to better outcomes. Otolaryngologists typically treat a patient population with a higher prevalence of ABRS and frequently see referred patients with recurrent acute sinusitis and chronic rhinosinusitis, which may explain their tendency to treat patients more aggressively. Nevertheless, these survey results illustrate a lack of consensus within the medical community regarding the evaluation and management of community-acquired ABRS, suggesting that widely accepted evidence-based practice guidelines need to be developed.

Facial nerve sacrifice and radiotherapy in parotid adenoid cystic carcinoma.

OBJECTIVES: To evaluate the impact of facial nerve sacrifice and postoperative radiotherapy on the outcome of adenoid cystic carcinoma of the parotid gland. STUDY DESIGN: Inception cohort. METHODS: Retrospective review of Department of Pathology, SNOMED database, and Tumor Registry including health-related quality of life data for the subset enrolled in the longitudinal Outcomes Assessment Project. Fisher exact, chi, and Wilcoxon tests were used to determine significant differences. RESULTS: Fifty-two cases (follow-up mean: 9.1 years, range: 0.5-40.8 years) demonstrated local control rates of 84.6% (5 years), 76.9% (10 years), and 50% (20 years). Compared with facial nerve preservation, facial nerve sacrifice had better control at 5 years (100 vs. 78.9% P = .259) while having detrimental effects on eating, speech, and esthetics. Local control at 5 years was significantly better (P = .048) with postoperative radiotherapy (100%) than without (84.6%). Overall survival was 79.4% (5 years), 50% (10 years), and 32.3% (20 years). At 10 years, there was a trend toward improved survival with facial nerve sacrifice (58.8 vs. 46.8%, P = .569) and postoperative radiotherapy (62.4 vs. 39.3%, P = .409). Eleven patients with lung metastases survived an average of 67.8 months after metastases were identified. Only 4 of 46 patients N0 patients (8.3%) subsequently developed lymph node metastases. CONCLUSION: Selective facial nerve sacrifice was associated with trends toward improved local control and survival but worse quality of life. Patients managed with postoperative radiotherapy had better local control rates than those without. N0 patients rarely developed metastases to regional lymph nodes.

Report of an obstructive goiter and its surgical treatment during delivery.

We report a case of a morbidly obese young woman in her third trimester of pregnancy presenting with a history of goiter and respiratory disease. The recent history of this patient was significant for worsening respiratory symptoms over a period of 2 weeks, and, on presentation at 36 weeks gestation, she was stridorous, dyspneic at rest, and had a hoarse voice. Evaluation revealed a morbidly obese individual with a large goiter. She was biochemically euthyroid. Fiberoptic laryngoscopy revealed a left true vocal cord paresis, and ultrasound evaluation was significant for diffuse multinodular enlargement, with each lobe measuring greater than 10 cm and the isthmus measuring 5. Pulmonary function testing revealed a significant degree of upper airway obstruction without significant lower airway disease. Given the patient's clinical signs and symptoms, her tenuous airway, poor candidacy for urgent tracheotomy, and her proximity to delivery, it was agreed that the patient should undergo elective cesarean section and at its completion undergo subtotal thyroidectomy for the obstructive goiter.