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[Chronic Progressive Neuro-Behçet's Disease].

Shunsei Hirohata1

  • 1Department of Internal Medicine, Teikyo University School of medicine.

Brain and Nerve = Shinkei Kenkyu No Shinpo
|May 19, 2021
PubMed
Summary

Neuro-Behçet's disease (NB) has acute and chronic forms. Chronic progressive NB shows dementia and brain stem atrophy, linked to high CSF IL-6, unresponsive to standard treatments but manageable with methotrexate.

Area of Science:

  • Neurology
  • Immunology
  • Rheumatology

Background:

  • Neuro-Behçet's disease (NB) presents as acute meningoencephalitis or chronic progressive neurological decline.
  • Diagnosis requires confirmation of Behçet's disease and characteristic MRI findings.
  • Chronic progressive NB is associated with persistent elevation of cerebrospinal fluid (CSF) Interleukin-6 (IL-6) levels.

Purpose of the Study:

  • To differentiate between acute and chronic progressive NB.
  • To review current understanding of NB pathophysiology and diagnostic criteria.
  • To evaluate treatment efficacy for different NB subtypes.

Main Methods:

  • Review of clinical presentations and MRI findings in acute and chronic progressive NB.
  • Analysis of CSF IL-6 levels as a biomarker for chronic progressive NB.

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  • Assessment of treatment responses to corticosteroids, cyclophosphamide, azathioprine, methotrexate (MTX), and infliximab.

    • Main Results:

    • Acute NB presents with meningoencephalitis and focal lesions on MRI.
    • Chronic progressive NB is characterized by dementia, ataxia, and brain stem atrophy correlated with CSF IL-6.
    • Corticosteroids, cyclophosphamide, and azathioprine are ineffective for chronic progressive NB; low-dose MTX can reduce CSF IL-6, and infliximab is effective for MTX-resistant cases.

    Conclusions:

    • Accurate diagnosis of NB subtypes is crucial for appropriate management.
    • Low-dose methotrexate and infliximab represent effective therapeutic options for chronic progressive NB.
    • Established clinical guidelines aid in the routine management of NB.