Two cases of probable Neuro-Behçet syndrome treated with autologous HSCT - Report - MDSpire

Two cases of probable Neuro-Behçet syndrome treated with autologous HSCT

  • 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

  • July 13, 2026

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Management of Two Cases with Likely Neuro-Behçet Syndrome Using Autologous Hematopoietic Stem Cell Transplantation

Background

Neuro-Behçet syndrome is a rare neurological manifestation of Behçet syndrome, often leading to significant disability due to brainstem and spinal cord lesions. The diagnosis and treatment of NBS can be complicated by atypical presentations and limited therapeutic options.

Data Highlights

No numerical data or trial data is provided in the article.

Key Findings

  • Both cases presented with longitudinally extensive transverse myelitis and brainstem lesions.
  • Patients experienced recurrent relapses and disease progression despite multiple immunotherapies.
  • Autologous hematopoietic stem cell transplantation did not confer clinical benefit in either case.
  • Diagnostic challenges were noted due to atypical presentations and limitations of current diagnostic criteria.
  • Both patients had a history of treatment with anti-TNF-α therapy prior to the onset of NBS symptoms.

Clinical Implications

Clinicians should be aware of the potential ineffectiveness of aHSCT as a rescue therapy in NBS.

Conclusion

The cases illustrate the diagnostic and therapeutic challenges associated with Neuro-Behçet syndrome, particularly the limited efficacy of aHSCT.

Related Resources & Content

  1. Author(s)/Org, Source, Year -- Title
  2. Bone Marrow Transplantation — The 47th Annual Conference of the European Society for Blood and Marrow Transplantation: Recognition of Van Bekkum Award Recipients
  3. Bone Marrow Transplantation — The 46th Annual Conference of the European Society for Blood and Marrow Transplantation: Recognition of Van Bekkum Award Recipients
  4. Bone Marrow Transplantation — The 51st Annual Conference of the European Society for Blood and Marrow Transplantation: Recognition of Van Bekkum Award Recipients
  5. Autologous haematopoietic stem cell transplantation for rheumatic diseases: best practice recommendations from the EBMT Practice Harmonization and Guidelines Committee | Bone Marrow Transplantation
  6. Bone Marrow Transplantation — The 49th Annual Conference of the European Society for Blood and Marrow Transplantation: Recognition of Van Bekkum Award Recipients
  7. https://academic.oup.com/bjd/article/194/1/e1/8268116
  8. Autologous haematopoietic stem cell transplantation for rheumatic diseases: best practice recommendations from the EBMT Practice Harmonization and Guidelines Committee | Bone Marrow Transplantation
  9. Autologous haematopoietic stem cell transplantation in non-MS neuroimmunological diseases - ScienceDirect

Original Source(s)

Two cases of probable Neuro-Behçet syndrome treated with autologous HSCT

  • 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

  • July 13, 2026

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