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  4. Intramedullary Spinal Hemorrhage in Behcet’s Syndrome: A Case Report and Systematic Review

Intramedullary Spinal Hemorrhage in Behcet’s Syndrome: A Case Report and Systematic Review

Cureus, 2023 · DOI: 10.7759/cureus.47134 · Published: October 16, 2023

RheumatologyImmunologyNeurology

Simple Explanation

Behcet's syndrome (BS) is a systemic vasculitis affecting arterial and venous vessels of all sizes, resulting in a broad array of clinical manifestations. Neurological involvement is rare, but when it occurs, it is often accompanied by severe disability and significant morbidity and mortality. The diversity of clinical manifestations proves challenging for early diagnosis; therefore, timely management is of paramount importance to compensate for the severity of progression at the time of presentation.

Study Duration
Not specified
Participants
12 cases of spontaneous bleeding associated with Behcet’s syndrome
Evidence Level
Level 4: Case Report and Systematic Review

Key Findings

  • 1
    The case presents the first reported instance of an abrupt onset of neurological injury secondary to intramedullary spinal cord bleed in Behcet’s syndrome.
  • 2
    The systematic review demonstrates no difference in mortality for patients managed conservatively compared to those who undergo surgical treatment.
  • 3
    New-onset neurological findings in patients with Behcet’s syndrome should raise suspicion for possible spontaneous hemorrhage.

Research Summary

This is a rare case of spontaneous intramedullary spinal cord hemorrhage in a patient with BS. The existing literature documenting cases of spontaneous hemorrhages in patients with BS reflects the immense variety in the presentation of BS. Our review attempts to encapsulate and centralize the variety of presentations of Behcet's disease to promote ease of diagnosis.

Practical Implications

Clinical Awareness

Increased awareness of intramedullary spinal bleeds in Behcet's patients with new neurological symptoms can lead to earlier diagnosis.

Diagnostic Approach

Prompt investigation for spinal hemorrhage is needed when Behcet's patients present with new neurological findings.

Treatment Strategies

Timely management, either surgical or medical, should be based on the location and progression of the lesion and the patient's systemic profile.

Study Limitations

  • 1
    Small sample size in the systematic review
  • 2
    Heterogeneity of the included case reports
  • 3
    Lack of long-term follow-up data in some cases

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