# A Case Report of Neuro-Behçet Syndrome: Frequent Neurological Manifestations Concurrent With Life-Threatening Illnesses

**Authors:** Omar Ballut, Mayas M Almahi, Banan S Alghamdi, Najla K Alzahrani, Maali A Alghamdi

PMC · DOI: 10.7759/cureus.54664 · Cureus · 2024-02-21

## TL;DR

This case report describes a 31-year-old man with neuro-Behçet syndrome and multiple severe health issues, showing how neurological symptoms were managed alongside unrelated life-threatening conditions.

## Contribution

The novelty lies in presenting a rare case of chronic progressive neuro-Behçet syndrome co-occurring with severe unrelated illnesses and detailing its management.

## Key findings

- The patient's neurological symptoms were managed with steroids, azathioprine, and colchicine.
- The patient developed aphasia and quadriplegia, reflecting the poor prognosis typical of parenchymal neuro-Behçet syndrome.
- Concurrent illnesses like infective endocarditis and septic shock were successfully treated with surgery and antibiotics.

## Abstract

​​​​Behçet disease (BD) is a recurrent, multisystemic autoimmune vasculitis that affects both small and large vessels. A combination of neurological signs and symptoms in BD is called neuro-Behçet syndrome (NBS). We present the case of a 31-year-old male diagnosed with chronic progressive NBS who presented with multiple relapsing episodes concurrent with infective endocarditis due to intravenous drug abuse, drug-induced hepatitis, acute kidney injury, and septic shock that is not related to BD. Neurological relapsing episodes were treated with steroids azathioprine and colchicine. At the same time, concurrent illnesses were managed appropriately. Infective endocarditis needed valve replacement surgery, and sepsis was treated with selected antibiotics. Fortunately, the patient's brain images and laboratory investigation improved accordingly. On average, patients with parenchymal neuro-Behçet syndrome (P-NBS) have a poor prognosis; within 10 years of diagnosis, 50% of those patients are severely disabled as our patient who became aphasic and quadriplegic.

## Linked entities

- **Chemicals:** azathioprine (PubChem CID 2265), colchicine (PubChem CID 2833)
- **Diseases:** Behçet disease (MONDO:0007191), infective endocarditis (MONDO:0000565), drug-induced hepatitis (MONDO:0002184), acute kidney injury (MONDO:0002492), aphasia (MONDO:0000598), quadriplegia (MONDO:0001590)

## Full-text entities

- **Diseases:** autoimmune vasculitis (MESH:D014657), septic shock (MESH:D012772), Behcet disease (MESH:D001528), intravenous drug abuse (MESH:D015819), acute kidney injury (MESH:D058186), Infective endocarditis (MESH:D004696), drug-induced hepatitis (MESH:D056486), quadriplegic (MESH:D011782), sepsis (MESH:D018805)
- **Chemicals:** colchicine (MESH:D003078), azathioprine (MESH:D001379), steroids (MESH:D013256)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC10961927/full.md

## References

14 references — full list in the complete paper: https://tomesphere.com/paper/PMC10961927/full.md

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Source: https://tomesphere.com/paper/PMC10961927