Neurological Involvement in a Portuguese Cohort of IgG4-Related Disease

Authors

  • João Moura *Shared first co-authorship. Neurology Department. Centro Hospitalar Universitário de Santo António. Porto. https://orcid.org/0000-0002-4212-906X
  • Maria João Malaquias *Shared first co-authorship. Neurology Department. Centro Hospitalar Universitário de Santo António. Porto.
  • Firmina Jorge Neurology Department. Centro Hospitalar Universitário de Santo António. Porto.
  • Eduarda Pinto Neurorradiology Department. Centro Hospitalar Universitário de Santo António. Porto.
  • Ana Sardoeira Neurology Department. Centro Hospitalar Universitário de Santo António. Porto.
  • Inês Laranjinha Neurology Department. Centro Hospitalar Universitário de Santo António. Porto.
  • Vanessa Oliveira Neurology Department. Centro Hospitalar Universitário de Santo António. Porto.
  • Ana Paula Sousa Neurophysiology Department. Centro Hospitalar Universitário de Santo António. Porto.
  • Joana Damásio Neurology Department. Centro Hospitalar Universitário de Santo António. Porto; Center for Predictive and Preventive Genetics (CGPP). Institute for Molecular and Cell Biology (IBMC). Instituto de Investigação e Inovação em Saúde (i3S). Universidade do Porto. Porto.
  • Luís Maia Neurology Department. Centro Hospitalar Universitário de Santo António. Porto.
  • Nuno Vila-Chã Neurology Department. Centro Hospitalar Universitário de Santo António. Porto.
  • Raquel Samões Neurology Department. Centro Hospitalar Universitário de Santo António. Porto.
  • Ricardo Taipa Portuguese Brain Bank. Centro Hospitalar Universitário de Santo António. Porto; Unit for Multidisciplinary Research in Biomedicine. Instituto de Ciências Biomédicas Abel Salazar. Universidade do Porto. Porto.
  • Ana Martins da Silva Neurology Department. Centro Hospitalar Universitário de Santo António. Porto; Unit for Multidisciplinary Research in Biomedicine. Instituto de Ciências Biomédicas Abel Salazar. Universidade do Porto. Porto.
  • Ernestina Santos Neurology Department. Centro Hospitalar Universitário de Santo António. Porto; Unit for Multidisciplinary Research in Biomedicine. Instituto de Ciências Biomédicas Abel Salazar. Universidade do Porto. Porto.

DOI:

https://doi.org/10.20344/amp.20767

Keywords:

Immunoglobulin G, Immunoglobulin G4-Related Disease/diagnosis, Nervous System Diseases

Abstract

Introduction: Neurological involvement in immunoglobulin G4-related disease (IgG4-RD) is increasingly recognized. Its diagnosis can be challenging due to clinical mimics and difficulty in obtaining nervous system biopsies. The aim of this study was to describe a cohort of neurological IgG4-RD patients.
Methods: Patients were recruited from a neuroimmunology tertiary center. Clinical, laboratory, neuroimaging and histological data were reviewed.
Results: Fifteen patients (60% women), with a median age of 53 years (48.5 – 65.0) were included: 13 (86.7%) classified as possible IgG4-RD, one (6.7%) as probable and one (6.7%) as definitive. The most common neurological phenotypes were meningoencephalitis (26.7%), orbital pseudotumor (13.3%), cranial neuropathies (13.3%), peripheral neuropathy (13.3%), and longitudinally extensive transverse myelitis (LTEM) (13.3%). Median serum IgG4 concentration was 191.5 (145.0 – 212.0) mg/dL. Seven in 14 patients had CSF pleocytosis (50.0%) and oligoclonal bands restricted to the intrathecal compartment, while most cases presented elevated CSF proteins (64.3%). Magnetic resonance imaging abnormalities included white matter lesions in four (26.7%), hypertrophic pachymeningitis in two (13.3%), and LETM in two (13.3%). Two patients had biopsy-proven IgG4-RD in extra-neurological sites.
Conclusion: This study highlights the phenotypical variability of the neurological IgG4-RD. Biopsy inaccessibility reinforces the importance of new criteria for the diagnosis of this subset of patients.

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Published

2024-04-26

How to Cite

1.
Moura J, Malaquias MJ, Jorge F, Pinto E, Sardoeira A, Laranjinha I, Oliveira V, Sousa AP, Damásio J, Maia L, Vila-Chã N, Samões R, Taipa R, Martins da Silva A, Santos E. Neurological Involvement in a Portuguese Cohort of IgG4-Related Disease . Acta Med Port [Internet]. 2024 Apr. 26 [cited 2024 May 6];. Available from: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/20767

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