Faculty Bibliography

Cerebrovascular disease is a major cause of death in patients with end-stage renal disease, particularly in those with diabetes mellitus. Cardiac disease frequently presents itself atypically in diabetic patients. This awareness has led to earlier detection and treatment of cardiac disease in diabetic patients. Whether cerebrovascular disease may also present itself in a highly atypical fashion in the diabetic patient with end-stage renal disease has never been addressed. We report the case of a diabetic hemodialysis patient who had an extremely unusual manifestation of stroke. A 67-year-old diabetic hemodialysis patient had marked distress secondary to the sudden onset of a foreign body sensation in the oropharynx. Results of a laryngoscopy were negative; x-rays of the neck and computerized tomography of the head and neck showed no abnormalities. Neurologic evaluation revealed an inability to detect oropharyngeal stimuli and an absent gag reflex but no other deficits. Magnetic resonance imaging of the brain revealed an infarction in the left corona radiata that extended to the thalamocapsular region and external capsule, and a lacunar infarct in the right pons. Cerebrovascular disease in the diabetic patient with end-stage renal disease may present itself atypically, and we suggest that cerebrovascular disease in these patients merits the same level of suspicion as cardiac disease

Hereditary nonchromaffin paragangliomas (PGL; glomus tumors; MIM 168000) are mostly benign, slow-growing tumors of the head and neck region, inherited from carrier fathers in an autosomal dominant fashion subject to genomic imprinting. Genetic linkage analysis in two large, unrelated Dutch families assigned PGL loci to two regions of chromosome 11, at 11q23 (PGL1) and 11q13.1 (PGL2). We ascertained a total of 11 North American PGL families and confirmed maternal imprinting (inactivation). In three of six families, linkage analysis provided evidence of linkage to the PGL1 locus at 11q23. Recombinants narrowed the critical region to an approximately 4.5-Mb interval flanked by markers D11S1647 and D11S622. Partial allelic loss of strictly maternal origin was detected in 5 of 19 tumors. The greatest degree of imbalance was detected at 11q23, distal to D11S1327 and proximal to CD3D. Age at onset of symptoms was significantly different between fathers and children (Wilcoxon rank-sum test, P < .002). Affected children had an earlier age at onset of symptoms in 39 of 57 father-child pairs (chi2 = 7.74, P < .006). However, a more conservative comparison of the number of pairs in which a child had > or = 5 years earlier age at onset (n = 33) vis-a-vis that of complementary pairs (n = 24) revealed no significant difference (chi2 = 1.42, P > .2). Whether these data represent genetic anticipation or ascertainment bias can be addressed only by analysis of a larger number of father-child pairs

Traditionally surgeons have avoided performing rhinoplasty in conjunction with sinus surgery for rhinosinusitis. With advances in rhinoplasty and endoscopic sinonasal surgery and its added benefits of precision, minimal trauma, and hemorrhage, the combined procedure is now feasible. The indications, management, and results are discussed in 40 patients who underwent the combined procedure. Patients were divided into three categories based on the location and severity of the sinus disease: mild, moderate, or severe. The majority of patients had mild or moderate sinus disease. The most common presenting symptoms were nasal obstruction and postnasal drip. All patients had a history of sinusitis recalcitrant to conservative medical management. Results demonstrate the combined treatment modality to be safe and effective in patients with mild to moderate sinus disease and in selected patients with severe sinus disease

BACKGROUND: This study defines the clinical settings in which extended radical neck dissection (ERND) was performed and determines its impact on control of disease in the neck and on survival. METHODS: We reviewed the records of 106 patients undergoing ERND between 1984 and 1993. Most (76) had metastatic squamous cell carcinoma (SCC) that had extended to extranodal structures in the upper neck. RESULTS: Five-year disease-free survival was 39%, and disease was controlled in the neck in 72 patients (68%) with a median follow-up of 5.5 years. A trend toward better survival was seen in patients with SCC (47% at 5 years), compared with those with other histologies (24% at 5 years; P <0.12). Patients with levels I, II, or III involved had better survival (46% at 5 years) than those with level IV, V, or multiple levels involved (14% at 5 years; P <0.0088). Finally, when prior radiation therapy precluded additional irradiation of the neck, survival was only 22% at 5 years, compared with 47% for those who received postoperative radiation (P <0.017). CONCLUSIONS: Although advanced neck disease invading adjacent structures remains an ominous sign, neck control and 5-year survival were achieved in nearly one half of these patients when multimodality therapy was possible

The pathobiology of salivary neoplasms can best be studied in a model system that reflects the native state of the tumor. The present study describes the use of a three-dimensional collagen gel (organoid) system in which pleomorphic adenomas of the parotid gland were propagated in vitro. Five pleomorphic adenoma cultures were established as organoid gels and compared with touch-preparations or cryopreserved specimens of native tumor. The organoid cultures demonstrated normal DNA content, the expression of myoepithelial cell proteins, and the production of sulfated acid mucins; these cellular and secretory features mimicked those found in the archival specimens. Further, organoid cultures of pleomorphic adenoma could be initiated after monolayer culture, demonstrating that culture on a plastic support does not alter the nature of the cells. Development of an in vitro culture system that maintains the native state of pleomorphic adenoma is an important tool for studying the pathobiology of these tumors

The gamma band response (GBR) is a predominantly exogenous, sinusoidal evoked response that occurs usually between 20 and 130 msec following stimulus onset. This response is believed to represent the synchronization of neuronal assemblies that serve auditory feature integration. The objective of the present investigation was to describe the characteristics of the scalp-recorded auditory evoked gamma band potential (aeGBP) recorded from normal young adult subjects. Results showed the aeGBP to consist of up to four oscillations and were best recorded at the frontal-central midline. The aeGBP was present 80 percent to 100 percent of the time, appeared as onset responses, and was recorded at the shortest latency over the contralateral anterior temporal scalp