Central nervous system involvement in common variable immunodeficiency: A case of acute unilateral optic neuritis in a 26 -year-old Italian Patient

Elena Abati, Irene Faravelli, Francesca Magri, Alessandra Govoni, Daniele Velardo, Delia Gagliardi, Eleonora Mauri, Roberta Brusa, Nereo Bresolin, Giovanna Fabio, Giacomo Pietro Comi, Maria Carrabba, Stefania Corti

Research output: Contribution to journalArticle

Abstract

Common Variable Immunodeficiency (CVID) is a group of heterogeneous primary immunodeficiencies sharing defective B lymphocytes maturation and dysregulated immune response and resulting in impaired immunoglobulin production. Clinical picture encompasses increased susceptibility to infections, hematologic malignancies, inflammatory, and autoimmune diseases. Neurological manifestations are uncommon and optic neuritis has been previously reported only in one case with bilateral involvement. We hereby report a case of a 26-year-old man affected by CVID undergoing regular immunoglobulin supplementation, who presented with acute unilateral demyelinating optic neuritis and lymphocytic pleocytosis in the cerebrospinal fluid. A variety of infectious, inflammatory, and neoplastic conditions were excluded and a diagnosis of clinically isolated optic neuritis was made. The patient was treated with a short course of intravenous steroids with complete recovery. Overall, this case expands our current knowledge about clinical spectrum of complications in CVID and highlights the need for further research about this complex disease.

Original languageEnglish
Article number1031
JournalFrontiers in Neurology
Volume9
Issue numberNOV
DOIs
Publication statusPublished - Nov 30 2018

Keywords

  • Autoimmunity
  • Clinically isolated syndrome
  • Common variable immunodeficiency
  • Optic neuritis
  • Optic neuropathy
  • Primary immunodeficiencies

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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