Neuro-Behçet's disease presenting as an isolated progressive cognitive and behavioral syndrome

Dario Saracino; Lia Allegorico; Anna Maria Barbarulo; Bianca Pollo; Giorgio Giaccone; Alessandra D'Amico; Ludovico D'Incerti; Orso Bugiani; Giuseppe Di Iorio; Simone Sampaolo; Mariarosa Anna Beatrice Melone

doi:10.1080/13554794.2018.1561898

Neuro-Behçet's disease presenting as an isolated progressive cognitive and behavioral syndrome

Neurocase. 2018 Oct-Dec;24(5-6):238-241. doi: 10.1080/13554794.2018.1561898. Epub 2018 Dec 25.

Affiliations

¹ a Second Division of Neurology , Center for Rare Neurological and Neuromuscular Diseases & Inter University Center for Research in Neurosciences, Department of Advanced Medical and Surgical Sciences, University of Campania Luigi Vanvitelli , Naples , Italy.
² b Division of Neurology V - Neuropathology , Fondazione IRCCS Istituto Neurologico Carlo Besta , Milan , Italy.
³ c Department of Advanced Biomedical Sciences , Neuroradiology, University "Federico II" , Naples , Italy.
⁴ d Neuroradiology Unit , Fondazione IRCCS Istituto Neurologico Carlo Besta , Milan , Italy.
⁵ e Sbarro Institute for Cancer Research and Molecular Medicine, Department of Biology , Center for Biotechnology, College of Science and Technology, Temple University , Philadelphia , PA , USA.

PMID: 30583716
DOI: 10.1080/13554794.2018.1561898

Abstract

Behçet's disease is a chronic inflammatory disorder manifesting as a vasculitis that affects arteries and veins of any size. Up to 44% of cases may also present with neurological symptoms, thus defining Neuro-Behçet's disease. We describe a case of Neuro-Behçet's disease characterized by progressive behavioral and cognitive deterioration prevailing over other neurological symptoms, without evident systemic involvement.

Keywords: Vasculitis; cognitive syndrome; frontal lobe syndrome; leukoencephalopathy; neuropsychology.

Publication types

Case Reports

MeSH terms

Adult
Behcet Syndrome / complications*
Behcet Syndrome / diagnosis
Behcet Syndrome / pathology
Behcet Syndrome / physiopathology
Cognition Disorders / diagnosis
Cognition Disorders / etiology*
Cognition Disorders / pathology
Cognition Disorders / physiopathology
Humans
Leukoencephalopathies / diagnosis
Leukoencephalopathies / etiology*
Leukoencephalopathies / pathology
Leukoencephalopathies / physiopathology
Magnetic Resonance Imaging
Vasculitis / diagnosis
Vasculitis / etiology*
Vasculitis / pathology
Vasculitis / physiopathology