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Lhermitte-Duclose Disease: Test Your IQ

Test your knowledge about this hamartomatous tumor of the cerebellar cortex.

A 39-year-old woman presented with 2-month history of progressively worsening headache and right-sided ataxia. Brain MRI showed a nonenhancing, T1 hypointense mass on the right cerebellar hemisphere, consistent with Lhermitte-Duclos disease

Which of the following statements about Lhermitte-Duclose disease is false?