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How about Kuru -- transmissable spongiform encephalopathy (TSE)? This zoonotic disease is very similar to (and may be identical to) Mad Cow Disease. It's comparatively rare in humans, with most cases appearing in the New Guinea area, and its transmission being generally attributed to cannibalism.

Canavan disease is another rare brain disease.

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Q: What is the name of a rare brain disease?
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