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Neurologic manifestations of Behçet disease: rheumatology experience

Neurologische Manifestationen des M. Behçet: rheumatologische Erfahrungen

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Abstract

Objective

Neurologic involvement in Behçet disease (BD) is a rare manifestation. Herein, we aimed to evaluate the clinical features and treatment choices of neuro-Behçet (NB) patients.

Methods

There were records of 800 BD patients between 1998 and 2021. Fifty-five of the BD patients had NB and the files of these patients were retrospectively evaluated. Patients were grouped into three subgroups: 22 (40%) had non-parenchymal, 25 (45%) had parenchymal, and 8 (15%) had both parenchymal and non-parenchymal (mixed) involvement.

Results

Of the 55 patients, 32 were male. Twenty-six of the NB patients were diagnosed with BD simultaneously. The most common complaint was headache (n = 24, 44%). The most affected site was periventricular white matter (n = 21, 38%). All patients had received corticosteroids. Azathioprine (AZA; n = 39, 71%) was the most common immunosuppressive agent after corticosteroids, followed by cyclophosphamide (n = 16, 29%).

Conclusion

Neurologic involvement is a rare complication of BD but is related to increased mortality and morbidity. Neurologic manifestations may be the initial symptom of BD, thus leading to diagnosis. Both neurology and rheumatology specialists should be aware of this rare condition.

Zusammenfassung

Ziel

Eine neurologische Beteiligung im Rahmen eines M. Behçet stellt eine seltene Manifestation dar. In der vorliegenden Arbeit war es das Ziel, die klinischen Merkmale und die zur Wahl stehenden Therapien bei Patienten mit Neuro-Behçet (NB) zu erörtern.

Methoden

Zwischen 1998 und 2021wurden 800 Fälle mit M. Behçet dokumentiert. Von den Patienten mit M. Behçet wiesen 55 einen NB auf, und die Krankenakten dieser Patienten wurden retrospektiv untersucht. Dabei wurden die Patienten in 3 Subgruppen unterteilt: Bei 22 (40%) bestand eine nichtparenchymale, bei 25 (45%) eine parenchymale und bei 8 (15%) sowohl eine parenchymale als auch eine nichtparenchymale (gemischte) Beteiligung.

Ergebnisse

Von den 55 Patienten waren 32 männlich. Bei 26 der NB-Patienten wurde gleichzeitig die Diagnose M. Behçet gestellt. Das häufigste Symptom waren Kopfschmerzen (n = 24; 44%). Die meistbetroffene Lokalisation stellte die periventrikuläre weiße Substanz dar (n = 21; 38%). Alle Patienten hatten Kortikosteroide erhalten. Azathioprin (AZA; n = 39; 71%) war das am häufigsten verabreichte immunsupprimierende Präparat nach den Kortikosteroiden, dann folgte Cyclophosphamid (n = 16; 29%).

Schlussfolgerung

Eine neurologische Beteiligung ist eine seltene Komplikation des M. Behçet, allerdings geht sie mit erhöhter Mortalität und Morbidität einher. Sowohl Neurologen als auch Rheumatologen sollten sich dieser seltenen Symptomkonstellation bewusst sein.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Osmangazi University, Eskişehir, Turkey

    Mustafa Dinler, Nazife Şule Yaşar Bilge MD, Reşit Yıldırım & Timuçin Kaşifoğlu

  2. Department of Internal Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey

    Ayşe Erçin Arslan

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  1. Mustafa Dinler
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  2. Nazife Şule Yaşar Bilge MD
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Corresponding author

Correspondence to Nazife Şule Yaşar Bilge MD.

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Conflict of interest

M. Dinler, N.Ş. Yaşar Bilge, A.E. Arslan, R. Yıldırım, and T. Kaşifoğlu declare that they have no competing interests.

For this article no studies with human participants or animals were performed by any of the authors. All studies mentioned were in accordance with the ethical standards indicated in each case. The study was approved by the Local Ethics Committee on 26/04/2022 with decision number 23.

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Ulf Müller-Ladner, Bad Nauheim

Uwe Lange, Bad Nauheim

Mustafa Dinler and Nazife Şule Yaşar Bilge share first authorship.

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Dinler, M., Yaşar Bilge, N.Ş., Arslan, A.E. et al. Neurologic manifestations of Behçet disease: rheumatology experience. Z Rheumatol 83 (Suppl 1), 200–205 (2024). https://doi.org/10.1007/s00393-023-01436-0

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