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Isaacs syndrome associated with polyarteritis nodosa.
Lola Aubry1, Thierry Badoux1, Thomas Reygaerts1
1Division of Internal medicine, Department of Medicine and Specialties, Fribourg Hospital and University of Fribourg, Fribourg, Switzerland.
BMJ Case Reports
|June 23, 2026
Summary
Isaacs syndrome, a rare nerve disorder, was found co-occurring with polyarteritis nodosa in a young woman. Treatment with cyclophosphamide and corticosteroids led to clinical remission.
Area of Science:
- Neurology
- Rheumatology
- Vascular Medicine
Background:
- Isaacs syndrome (IS) is a rare peripheral nerve hyperexcitability disorder.
- IS is often associated with an underlying systemic disease.
- Polyarteritis nodosa is a systemic vasculitis affecting medium-sized arteries.
Purpose of the Study:
- To report a previously undescribed co-occurrence of Isaacs syndrome and systemic polyarteritis nodosa.
- To describe the clinical presentation and diagnostic findings in this unique case.
- To highlight the successful management of this combined condition.
Main Methods:
- A comprehensive clinical case review was performed.
- Diagnostic workup included detailed neurological examination, laboratory tests, and MRI.
- Treatment involved immunosuppressive therapy.
Main Results:
- A young woman presented with symptoms suggestive of both IS and systemic vasculitis, including back pain, weight loss, fasciculations, and cutaneous lesions.
- MRI revealed mesenteric vasculitis, confirming the polyarteritis nodosa diagnosis.
- The patient experienced significant clinical improvement after treatment.
Conclusions:
- This case represents a novel association between Isaacs syndrome and systemic polyarteritis nodosa.
- Early diagnosis and aggressive immunosuppressive treatment, including cyclophosphamide and corticosteroids, can lead to remission.
- Further research is warranted to understand the potential link between these conditions.
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