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[Avellis syndrome in systemic rheumatoid vasculitis]
K Kashihara1, H Ishizu, T Shomori
1Department of Neurology, Okayama University.
Rinsho Shinkeigaku = Clinical Neurology
|October 1, 1995
Summary
This case report details a patient with rheumatoid vasculitis presenting with Avellis syndrome, characterized by hoarseness and dysphagia. It highlights that vagus nerve mononeuritis, not just brainstem infarction, can cause this rare neurological condition.
Area of Science:
- Neurology
- Rheumatology
- Pathology
Background:
- Rheumatoid vasculitis is a rare systemic inflammatory condition affecting blood vessels.
- Avellis syndrome, typically associated with lateral medullary infarction, presents as unilateral vocal cord and soft palate paralysis, leading to aphonia and dysphagia.
Observation:
- A 74-year-old man developed sudden hoarseness and dysphagia, preceded by arthralgia and pleuritis.
- Clinical examination revealed right vocal cord paralysis and soft palate dysfunction.
- Laboratory tests showed elevated inflammatory markers, rheumatoid factor, C1q-binding immune complexes, and positive p-ANCA, along with hemolytic anemia and hypoproteinemia.
Findings:
- Nerve biopsy and autopsy revealed segmental demyelination in the sural and vagus nerves, consistent with mononeuritis.
- The patient's symptoms were attributed to systemic rheumatoid vasculitis affecting the vagus nerve.
Implications:
- This case demonstrates that Avellis syndrome can be caused by mononeuritis of the vagus nerve secondary to rheumatoid vasculitis.
- It expands the differential diagnosis for Avellis syndrome beyond brainstem lesions.
- Highlights the importance of considering systemic vasculitis in the etiology of cranial nerve palsies presenting with aphonia and dysphagia.