The effects of everolimus on tuberous sclerosis-associated lesions can be dramatic but may be impermanent.

BACKGROUND: Tuberous sclerosis complex (TSC) predisposes to the development of benign lesions within multiple organ systems, including the brain, kidneys, heart, lungs, and skin. Disease mortality is due to space-occupying subependymal giant cell astrocytomas and hemorrhage-prone renal angiomyolipomas. The recent use of mTORC1 inhibitors, such as everolimus, has allowed for direct targeting of TSC-associated mass lesions without apparent effect on surrounding tissues. Because of the mechanism of these drugs, there is reason to believe that these effects are not durable and that there may be need for continued long-term maintenance therapy. CASE-DIAGNOSIS/TREATMENT: We present a case of TSC-associated mass lesions that were ill-suited for definitive surgical therapy. The patient was started on everolimus, however due to a complex social situation treatment was discontinued and ultimately resumed many months later. Radiologic studies acquired before and after each period of therapeutic onset/cessation reveal the dramatic but impermanent effects of mTORC1 inhibition. CONCLUSIONS: While everolimus provides a non-invasive way to treat TSC-associated lesions, patients may require lifelong therapy. When termination of therapy is considered, the patient should be made aware of the expectation of potentially dramatic increases in lesion size. If consideration is to be given to definitive surgical therapy, it should be pursued while the patient is still on the medication, or at least soon after treatment is halted.

Vowel category dependence of the relationship between palate height, tongue height, and oral area.

This article evaluates intertalker variance of oral area, logarithm of the oral area, tongue height, and formant frequencies as a function of vowel category. The data consist of coronal magnetic resonance imaging (MRI) sequences and acoustic recordings of 5 talkers, each producing 11 different vowels. Tongue height (left, right, and midsagittal), palate height, and oral area were measured in 3 coronal sections anterior to the oropharyngeal bend and were subjected to multivariate analysis of variance, variance ratio analysis, and regression analysis. The primary finding of this article is that oral area (between palate and tongue) showed less intertalker variance during production of vowels with an oral place of articulation (palatal and velar vowels) than during production of vowels with a uvular or pharyngeal place of articulation. Although oral area variance is place dependent, percentage variance (log area variance) is not place dependent. Midsagittal tongue height in the molar region was positively correlated with palate height during production of palatal vowels, but not during production of nonpalatal vowels. Taken together, these results suggest that small oral areas are characterized by relatively talker-independent vowel targets and that meeting these talker-independent targets is important enough that each talker adjusts his or her own tongue height to compensate for talker-dependent differences in constriction anatomy. Computer simulation results are presented to demonstrate that these results may be explained by an acoustic control strategy: When talkers with very different anatomical characteristics try to match talker-independent formant targets, the resulting area variances are minimized near the primary vocal tract constriction.

Fulminant clostridium difficile colitis: comparing computed tomography with histopathology: are they concordant?

A Total abdominal colectomy (TAC) is recommended for fulminant Clostridium difficile colitis (FCDC) because intraoperative assessment of diseased segments is inaccurate. To determine whether computerized tomography (CT) provides an accurate assessment of disease, we examined the concordance between CT and histopathologic colitis distribution in patients undergoing TAC for FCDC. The ileocolon was divided into seven distinct segments. Of 20 patients meeting criteria, the median interval between preoperative CT and TAC was 1.5 days (range, 0 to 23 days), and mortality was 65 per cent. The CT distribution of colitis was pancolitis in 12 patients and segmental in eight. Nine of the 12 patients with CT pancolitis had histologic pancolitis (75% concordance). Four of the eight patients with CT-diagnosed segmental disease had histologic segmental disease (50% concordance). For patients with FCDC, the distribution of colitis on CT agrees with the histopathologic extent of disease in the majority of patients. However, discordance between CT and histologic extent of disease was present in 25 to 50 per cent of patients. Therefore, the recommendation for TAC rather than segmental resection for FCDC remains justified.