Neuroradiology

Familial amyloidotic polyneuropathy

dominantly inherited amyloidosis, in which the amyloid is deposited prominently in the peripheral nerves and contains a mutant form of a serum protein, transthyretin, that usually binds and transports thyroxine and retinole (hence the name). Type I familial amyloid polyneuropathy has been reported to cause amyloid deposition along the leptomeninges of the spinal cord and brain in addition to the visceral organs and the peripheral somatic and autonomic nerves and should therefore be considered in the differential diagnosis of leptomeningeal enhancement.

FS