Twin Disorders

What can two little girls teach us about Alzheimer's disease?

By Alison McCook

hen you meet identical four-year old twins Addi and Cassi Hempel, you might notice something about the way they walk. They used to run around like other toddlers, but now they are more wobbly, more uncertain, and walk with their legs somewhat wide apart, as if aboard a boat. They can sway in any direction, losing their balance. They fall more often than they should.

They will notice you, and smile. They don't say words but they talk, a rhythmic, nonsensical babble from which a crystal-clear sound occasionally escapes: "ice cream," "paddycake," "four." Their heads have a slight bobble, and they sometimes can't angle their eyes downward, so they fall again.

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1 Neurons affected by both diseases experience dramatic accumulations of autophagic vacuoles and lysosomes, and cells affected by both disorders have enlarged...

1. J.V. Reddy et al., "Clues to neuro-degeneration in Niemann-Pick Type C disease from global gene expression profiling," PLoS ONE 1(1):e19, 2006.
2. S.D. Woodlhouse et al., "Iminosugars in combination with interferon and ribavirin permanently eradicate noncytopathic bovine viral diarrhea virus from persistently infected cells," Lancet Neurol, 6:765-72, 2007.
3. N. Aynun et al., "Curcumin structure-function, bioavailability, and efficacy in models of neuroinflammation and Alzheimer's disease,"J Pharmacol Exp Ther, 326:196-208, 2008.

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