Transthyretin amyloidosis can present as an infiltration of the leptomeninges by amyloidosis (see Blevins et al). The clinical syndrome includes dementia, seizures, stroke-like episodes, subarachnoid hemorrhage, ataxia, myelopathy, deafness, radiculopathy, and ocular amyloidosis, usually affecting the vitreous. The leptomeninges enhance with gadolinium on MRI; the CSF has elevated protein but is otherwise nondescript. There is no proven therapy, but liver transplantation could theoretically be effective.
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