Publications by authors named "A Demas"

Introduction: VEXAS (Vacuoles, E1 Enzyme, X-linked, autoinflammatory, Somatic) syndrome is a recently described severe adult-onset autoinflammatory disorder mediated by X-linked gene UBA1 somatic mutations, responsible of recurrent fever, skin involvement, chondritis, macrocytic anemia and inflammatory syndrome. Neurological manifestations are rarely described, and predominantly involve peripheral nervous system (PNS) impairment.

Results: We report the first central nervous system (CNS) vasculitis in VEXAS syndrome, characterized by headache, cognitive dysfunction and focal signs (cerebellar ataxia).

View Article and Find Full Text PDF

Background: Melioidosis is an endemic disease in South-East Asia and Northern Australia caused by a Gram-negative bacillus, . Manifestations are wide and neurological involvement have rarely been described.

Methods: In this paper, we report a patient returning from Asia with an unusual infection including CNS involvement consistent with a melioidosis.

View Article and Find Full Text PDF