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Wolfram syndrome

Wolfram syndrome, also called DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and Deafness), is a rare genetic disorder, causing diabetes mellitus, optic atrophy, and deafness. It was first diagnosed in 1938 by a physician named Wolfram in four siblings. The disease affects both the brain (especially the brain stem) and the central nervous system. It is thought to be caused by both a malfunction of the mitochondria and of myelination, the latter in effect similar to multiple sclerosis. There is no known treatment. more...

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Life expectancy of people suffering from this syndrome is about 30 years.

See also : List of rare diseases

Read more at Wikipedia.org


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You Can't Think About What You Don't Know
From eWEEK, 7/22/02 by Peter Coffee

It would be, at best, a gloomy satisfaction to be the person who wrote the report—released on September 11, 1998, if you're looking for painful ironies—that predicted a "catastrophic" intelligence failure if American agencies' funding priorities weren't substantially revised.

Last week's report from the House Subcommittee on Terrorism and Homeland Security found the resulting systemic problems, such as widespread shortage of foreign-language skills, to be much greater threats to our security than bogeymen such as off-the-shelf encryption.

I was struck by an associated story on National Public Radio, which noted that headquarters counter-terrorism staff had been expanded since that time—but that this larger analysis group had less raw material to analyze, thanks to a concurrent reduction in field intelligence collection assets during that same period. More analysis of less actual data? This may be the defining syndrome of the era of cheap computation, as people are tempted to do more of what costs less with every step down the Moore's-Law curve—while cutting back on the old-fashioned efforts that seem ever more "inefficient."

It's nice to see an exception to this trend in the latest release of Mathematica, version 4.2, from Wolfram Research, in Champaign, Ill. A tool like Mathematica could encourage the introspective, "I'm sure I can figure this out myself" approach that is embodied in company founder Stephen Wolfram's controversial book, "A New Kind of Science," now topping every true geek's summer reading list.

But even while Mathematica's continuing computational refinements enable ever-deeper exploration of an isolated idea, the XML facilities in the new release—combined with the XML APIs now being exposed by many Web sites—also make it a tool for going out into the world and collecting new raw facts. For example, during a conversation last week, Wolfram's Director of R&D, Roger Germundsson, showed me that he could write an "AmazonSearch" function in Mathematica that would drop a formatted table of authors and titles meeting certain criteria into the middle of a Mathematica "notebook" document, querying the XML-based interface that Amazon makes available for use by its retail partners.

It's also important that Mathematica 4.2 emphasizes enhanced tools for publishing its results, as well as for collecting initial data. For a counterexample, look at the way that the House subcommittee formatted the online report that I hyperlinked above: It's a Word file, but all it contains is images of printed pages. Terrific: You need Word to open it, but it can't be indexed or searched or readily excerpted. How many ways can we do this wrong in a single, simple document?

Applications need continuing access to new data if they're going to do anything more than confirm our initial hypotheses. Analysis needs to be shared, in ways that both expose our assumptions and make our results available as catalysts for others' thinking. And enterprise applications, such as those for relationship management, need to avoid making the mistake of thinking that more analysis can ever make up for having less, or lower quality, information from the real world.

Tell me how you keep your applications well informed.

Copyright © 2002 Ziff Davis Media Inc. All Rights Reserved. Originally appearing in eWEEK.

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