Structural signature of sporadic Creutzfeldt-Jakob disease.

BACKGROUND AND PURPOSE: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rapidly progressive neurodegenerative disease caused by an abnormal isoform of the human prion protein. Structural magnetic resonance imaging in patients with pathologically confirmed sCJD was compared with cognitively normal individuals to identify a cortical thickness signature of sCJD. METHODS: This retrospective cross-sectional study compared patients with autopsy-confirmed sCJD with dementia (n = 11) with age- and sex-matched cognitively normal individuals (n = 22). We identified regions of interest (ROIs) in which cortical thickness was most affected by sCJD. Within patients with sCJD, the relationship between ROI cortical thickness and clinical measures (disease duration, cerebrospinal fluid tau and diffusion-weighted imaging abnormalities) was evaluated. RESULTS: Compared with cognitively normal individuals, patients with sCJD had significantly reduced cortical thickness in multiple ROIs, including the fusiform gyrus, precentral gyrus, precuneus and superior temporal gyrus bilaterally; the caudal middle frontal gyrus, superior frontal gyrus, postcentral gyrus, inferior temporal gyrus and transverse temporal gyrus in the left hemisphere; and the superior parietal lobule in the right hemisphere. Only one patient with sCJD had co-pathology consistent with Alzheimer's disease. Reduced cortical thickness did not correlate with disease duration, presence of diffusion restriction or elevated cerebrospinal fluid tau. CONCLUSION: Cortical signature changes in sCJD may reflect brain changes not captured by standard clinical measures. This information may be used with clinical measures to inform the progression of sCJD and patterns of prion protein spread throughout the brain. These results may have implications for prediction of symptomatic progression and plausibly for development of therapeutic strategies.

Strategies for surviving a shakeout.

Shakeouts are a fact of life in almost every industry-witness the shrinking number of players in areas as diverse as banking, software, and hospital supply distribution. The key to survival is sensing your industry's shakeout before the competition does. And the first hurdle for managers to overcome is the belief that it can't happen to them. It can and it probably will. George Day describes two shakeout syndromes that affect different types of industries. A boom-and-bust shakeout afflicts hot emerging markets or highly cyclical businesses. A glut of competitors enter the market during boom times, but many of them fail when growth slows or a dominant design emerges. A seismic-shift shakeout strikes mature industries that have enjoyed years of protected prosperity as a result of, for example, local regulations or import barriers. But deregulation, globalization, or technological change can pull the rug out from under them. Day outlines how companies can detect the early warning signs of a shakeout. He explains how adaptive survivors, such as Dell Computer, successfully adjust their businesses in the midst of a bust, and how aggressive amalgamators, such as Arrow Electronics, cut costs and acquire smaller rivals in order to remain standing after a seismic shift. But the fact remains that most companies will get squeezed out during a consolidation. Although it is enormously difficult for executives to come to terms with the grim news, the sooner they do so, the better. And, as Day points out, all is not necessarily lost: with the right timing, also-rans can make a profitable exit from an industry.