Original article
Clinicopathologic features and outcomes of neuro-Behçet disease in Spain: A study of 20 patients

https://doi.org/10.1016/j.ejim.2010.08.003Get rights and content

Abstract

Background

To describe the clinical characteristics and evolution of a series of adult patients hospitalized for neuro-Behçet disease (NBD).

Methods

Consecutive patients admitted for NBD in a teaching hospital were retrospectively selected. Disability at discharge and during follow-up was graded with the modified Rankin Scale, and outcome classified as good or poor (grades 3–6).

Results

Twenty patients were included (M/F, 13/7). Mean age at NBD diagnosis was 36.3 years. Nineteen patients had other manifestations of Behçet disease (BD) before NBD developed, but only 7 met the complete diagnostic criteria for BD. Fever, headache, motor weakness, and cranial nerve palsy were each present in approximately 60% of patients. There was a low prevalence of behavioral changes (5%), seizures (5%), and sphincter incontinence (0%), and a relatively high prevalence of meningism (25%). Non-neurologic manifestations of BD were concurrently detected in 15 patients (75%). 80% had parenchymal involvement. Brain biopsies during 5 attacks showed perivascular lymphocytic infiltration with reactive astrocytosis, but no frank vasculitis. During a mean follow-up of 6.3 years per patient, 12 had at least one relapse. In total, there were 22 relapses; all but two were in the same location and were symptomatically similar in each patient. At the end of follow-up, 7 patients (35%) had a poor outcome, including 4 who died.

Conclusion

Recording of previous manifestations of BD and a physical examination to detect concomitant systemic manifestations of BD may help establish an early diagnosis of NBD. Relapses frequently occurred in the same location. No frank vasculitis was present in brain biopsies.

Introduction

Behçet disease (BD) is a chronic inflammatory systemic disease of unknown cause, characterized by recurrent oral and genital ulcers, and uveitis (1). Although this classic triad remains diagnostically important, it has become increasingly apparent that the inflammatory lesions can arise in almost any tissue, resulting in cutaneous, vascular, articular, gastrointestinal, renal, cardiopulmonary, or neurologic involvement [1], [2], [3], [4]. Classification criteria for this condition have been proposed by the International Study Group (ISG) for Behçet disease (5).

Neuro-Behçet disease (NBD) predominates in men and usually appears about 5 years after the onset of BD symptoms, although it may be the first manifestation or even precede other manifestations of the disease by several years [3], [6]. The frequency of NBD among BD patients varies widely from 1.3% to 59% [2], [7], [8], [9]. An autopsy series of 170 cases of BD showed pathological evidence of neurologic involvement in 20% of cases (10). Neurologic and major vessel involvement are the main causes of mortality in BD [2], [10].

Pathological studies investigating central nervous system (CNS) involvement in BD have mainly reported perivascular lymphocytic infiltration [10], [11], [12]. Because of the nonspecific pathology and lack of pathognomonic neuroimaging findings, the diagnosis of NBD requires a high index of clinical suspicion. Genetic and environmental factors are thought to modulate the expression of both BD and NBD. Hence, a familiarity with the clinical presentation of the disease in different geographic areas would be useful for diagnostic purposes (13).

The aim of this study was to describe the characteristics of NBD in a series of consecutively hospitalized adult patients with this condition in a tertiary care teaching hospital in Barcelona (Spain).

Section snippets

Materials and methods

This is a retrospective study covering a 20-year period (September 1988 to December 2008), including all adult patients (18 years and older) admitted to Hospital Universitari de Bellvitge (L'Hospitalet de Llobregat, Barcelona, Spain) with a diagnosis of NBD. Complete data from our NBD inpatient records were simultaneously evaluated by two internal medicine specialists (AR-M and AV) and two neurologists (SM-Y and AM-Y). We selected patients with a first neurologic attack who showed objective

Epidemiology

Twenty patients met the inclusion criteria for NBD (13 males and 7 females; M/F ratio, 1.8), representing 18% of patients with BD attended in our hospital over the study period. Sixteen patients were Caucasian and 4 were of African ethnic origin. On admission, BD had been established in only 7 of the 20 patients, with a mean age at diagnosis of 34 (19–54) years. The mean interval between the diagnoses of BD and NBD was 2.5 (0.2–4) years. Prior to admission for NDB symptoms, 12 of the remaining

Discussion

The percentage of BD patients in our series with neurologic involvement and the demographic characteristics of this population are similar to the data reported in earlier studies from different geographical areas [2], [10], [16]. However, the predominance of affected males, as was noted here, was not found in two recent European series [16], [17]. As in other studies from Western Europe, the percentage of NBD cases in patients without a prior diagnosis of BD was higher than the values recorded

Learning points

  • In this Spanish series of adult patients hospitalized for NBD, fever, headache, motor weakness, and cranial nerve palsy were each present in approximately 60% of patients. There was a low prevalence of behavioral changes (5%), seizures (5%), and sphincter incontinence (0%). In contrast, meningism was present in a quarter of our patients, a finding reported less frequently in other studies.

  • In our patients, all but 2 relapses occurred in the same location and patients presented symptoms similar

Acknowledgment

The authors thank Prof. Hasan Yazici, Division of Rheumatology, Cerrahpaşa Medical School, University of Istanbul, Istanbul, Turkey for his critical review of the manuscript.

References (35)

  • U. Tursen et al.

    Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet's disease

    Int J Dermatol

    (2003)
  • S. Farah et al.

    Behçet's syndrome: a report of 41 patients with emphasis on neurological manifestations

    J Neurol Neurosurg Psychiatry

    (1998)
  • M.G. Hadfield et al.

    Neuro-Behcet's disease

    Clin Neuropathol

    (1996)
  • Y. Arai et al.

    Autopsy case of neuro-Behçet's disease with multifocal neutrophilic perivascular inflammation

    Neuropathology

    (2006)
  • R. Mahr et al.

    Population-based study of Behçet's disease: differences by ethnic origin and low variation by age at immigration

    Arthritis Rheum

    (2008)
  • J.C. van Swieten et al.

    Interobserver agreement for the assessment of handicap in stroke patients

    Stroke

    (1988)
  • N. Caballol et al.

    Endovascular treatment of carotid and pulmonary aneurysms in Behçet's disease

    Neurologia

    (2005)
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