Central nervous system involvement as the presenting manifestation of autoimmune rheumatic diseases: An observational study using the American College of Rheumatology nomenclature for neuropsychiatric lupus

Vasileios Mastorodemos, Marilena Mamoulaki, Herakles Kritikos, Andreas Plaitakis, Dimitrios T. Boumpas

Research output: Contribution to journalArticlepeer-review

9 Scopus citations

Abstract

Objective: We sought to describe CNS involvement as initial presentation of autoimmune rheumatic diseases using a standardized nomenclature. Patients and methods: A 6-year observational study (1999-2005) was conducted in the University Hospital of Heraklion Crete, a regional referral secondary/tertiary care academic center. Patients presenting with new neurological symptoms of acute/sub-acute onset underwent clinical and laboratory screening for systemic autoimmune disorders. The diagnosis of an autoimmune rheumatic disorder was based upon the American College of Rheumatology (ACR) classification criteria, whereas for primary antiphospholipid syndrome (PAPS) we used the Sapporo preliminary criteria. In order to describe the neurological syndromes we used the ACR nomenclature for neuropsychiatric lupus. Results: During this period fourteen patients (ten females and four males) were recorded. Eight patients had systemic lupus erythematosus (SLE), four had primary APS and the remaining two had systemic vasculitis. Four out of the eight SLE patients had secondary APS. Two of them presented with movement disorder (chorea). The other two and all four patients with primary APS presented with cerebrovascular disease (CVD). These six patients comprised the 5.7% of young adults under < 45 years old with cerebrovascular accident admitted over the 6-year period. Conclusions: SLE and APS either primary or secondary to SLE were the most common underlying systemic autoimmune rheumatic diseases, in patients presenting with a neurological event of acute onset. Young adults (< 45 years old) with CVD should undertake screening for SLE/APS.

Original languageEnglish
Pages (from-to)629-635
Number of pages7
JournalClinical and Experimental Rheumatology
Volume24
Issue number6
StatePublished - Nov 2006
Externally publishedYes

Keywords

  • Antiphospholipid syndrome
  • Cerebrovascular disease
  • Initial
  • Neuropsychiatric disease

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