Brain microbleeds 12 Years after orthotopic liver transplantation in Val30Met amyloidosis

Fabrizio Salvi, Francesca Pastorelli, Rosaria Plasmati, Cristina Morelli, Claudio Rapezzi, Andrea Bianchi, Mario Mascalchi

Research output: Contribution to journalArticlepeer-review

Abstract

Unexplained focal neurologic episodes (FNEs) can occur in patients with transthyretin-related familial amyloidotic polyneuropathy (TTR-FAP) after orthotopic liver transplantation (OLT). A patient with Val30Met FAP underwent OLT at age 34 years. Twelve years after transplantation, she presented with recurrent FNEs lasting from 10 minutes to 8 hours each, with nonuniform deficitary clinical features and variably associated with headache. Magnetic resonance imaging showed multiple brain microbleeds and diffuse contrast enhancement of the craniospinal leptomeninges consistent with amyloid deposits. Our observation suggests that microbleeds associated with meningovascular amyloidosis can underlie FNEs in TTR-FAP. Moreover, it confirms that OLT does not halt progression of leptomeningeal and vascular amyloid deposition due to TTR production in the choroid plexuses. Such a progression might compromise the good long-term prognosis of patients with TTR-FAP due to increased risk of intracranial hemorrhages. Pharmacologic therapies targeting brain TTR production may modify this scenario.

Original languageEnglish
Pages (from-to)e149-e150
JournalJournal of Stroke and Cerebrovascular Diseases
Volume24
Issue number6
DOIs
Publication statusPublished - Jun 1 2015

Keywords

  • Cerebrovascular amyloidosis
  • magnetic resonance imaging
  • microbleeds
  • TTR-amyloidosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Surgery
  • Rehabilitation
  • Cardiology and Cardiovascular Medicine

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