title: Brain Microbleeds 12 Years after Orthotopic Liver Transplantation in Val30Met Amyloidosis.
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.
authors: IRCCS Istituto delle Scienze Neurologiche, UOC Neurologia, Ospedale Bellaria, Bologna, Italy.
authors: Fabrizio
authors: F
authors: Salvi
authors: IRCCS Istituto delle Scienze Neurologiche, UOC Neurologia, Ospedale Bellaria, Bologna, Italy.
authors: Francesca
authors: F
authors: Pastorelli
authors: IRCCS Istituto delle Scienze Neurologiche, UOC Neurologia, Ospedale Bellaria, Bologna, Italy.
authors: Rosaria
authors: R
authors: Plasmati
authors: Department of Internal Medicine, University of Bologna and S.Orsola-Malpighi Hospital, Bologna, Italy.
authors: Cristina
authors: C
authors: Morelli
authors: Cardiovascular Department, University of Bologna and S.Orsola-Malpighi Hospital, Bologna, Italy.
authors: Claudio
authors: C
authors: Rapezzi
authors: "Mario Serio" Department of Experimental and Clinical Biomedical Sciences, University of Florence, Florence, Italy.
authors: Andrea
authors: A
authors: Bianchi
authors: "Mario Serio" Department of Experimental and Clinical Biomedical Sciences, University of Florence, Florence, Italy. Electronic address: m.mascalchi@dfc.unifi.it.
authors: Mario
authors: M
authors: Mascalchi
mesh_terms: D028227:Amyloid Neuropathies, Familial / Q000473:pathology* / Q000601:surgery; D001921:Brain / Q000473:pathology*; D005260:Female; D006801:Humans; D020300:Intracranial Hemorrhages / Q000473:pathology*; D016031:Liver Transplantation; D008279:Magnetic Resonance Imaging; D008875:Middle Aged
keywords: Cerebrovascular amyloidosis; TTR-amyloidosis; magnetic resonance imaging; microbleeds