Diagnosing neuro-Behçet’s disease

Cătălina Elena Bistriceanu,Dan Iulian Cuciureanu,Florentina Anca Danciu

doi:10.37897/rjn.2021.3.22

Abstract

Behçet’s disease is a rare systemic vasculitis characterized by uveitis, recurrent oral and genital ulcers, firstly described by the Turkish dermatologist Hulusi Behçet. The etiology is unknown, although autoimmune mechanisms are described. There is no specific test for the diagnosis of Behçet’s disease. The International Criteria for Behçet’s Disease (ICBD) proposed a new set of diagnostic criteria including oral and genital aphthosis, skin, ocular and vascular manifestations, CNS involvement and positive pathergy test. The neurologic involvement could be classified in parenchymal neuro-Behçet’s and non-parenchymal neuro-Behçet’s disease. We report a case of a woman with a very impressive personal history. Over time, she had many specific neurological complications compatible with neuro-Behçet’s disease (NBD). The other general symptoms also suggested Behçet’s disease, according to ICBD.

Highlights

Behçet’s disease is a rare systemic vasculitis characterized by uveitis and recurrent oral and genital ulcers
Large vessels are affected by a vasculitis of the vasa vasorum
The vascular injuries are superimposed on the hypercoagulability which is characteristic of Behçet’s disease and that may be due, in part, to activated endothelial cells and activated platelets [4]

Summary

INTRODUCTION

Behçet’s disease is a rare systemic vasculitis characterized by uveitis and recurrent oral and genital ulcers. It was first described in 1937 by Hulusi Behçet from Istanbul who reported two patients with this triple-symptom complex [1]. In 2010, neurologic signs and pathergy test were included given its high specificity [10] These criteria for Behçet’s disease are written in the table below (table 1). In the case of parenchymal neuro-Behçet’s, Pallis and Fudge (1956) and Wadia and Williams (1957) described the clinical syndromes and classified them into three types: first, brainstem disturbance associated with systemic symptoms such as fever, arthralgia and skin eruption. Headache with meningism was commonly cited, and CSF pleocytosis was observed; second, meningomyelitis, including a meningitis with varying neurological signs; third, a confusional syndrome, in which meningoencephalitis without focal neurological signs arose, which was in some instances chronic and progressive [14]

Cranial neuropathies

CASE PRESENTATION

Findings

CONCLUSIONS