To report the clinical course, treatment strategies, and responses of two cases with probable Neuro-Behçet syndrome (NBS).
Approach:
Case Description: Two cases of probable NBS were described, detailing their clinical presentations, diagnostic challenges, and treatment responses.
Key Findings:
Both cases presented with longitudinally extensive transverse myelitis and brainstem lesions.
Recurrent relapses and disease progression were resistant to multiple immunotherapies.
Autologous hematopoietic stem cell transplantation (aHSCT) did not confer clinical benefit in either case.
Interpretation:
NBS should be considered in patients with myelitis and brainstem involvement who have limited treatment response. The cases highlight diagnostic challenges and the heterogeneity of disease presentation.
Limitations:
The diagnostic criteria for NBS are less well defined compared to Behçet syndrome.
Limited evidence exists guiding the treatment of NBS, particularly regarding aHSCT.
Conclusion:
Further studies are required to optimize therapeutic strategies for NBS.
by Charlotte Schubert, Lea I. Walter, Marina Herwerth, Imke Metz, Jakob Nilsson, Ilijas Jelcic, Patrick Roth, Nicolaus Kröger, Ina Kötter, Christoph Heesen, Vivien Häußler, Veronika Kana
