# Anti-Neutrophil Cytoplasmic Antibody-Associated Central and Peripheral Nervous System Vasculitis

## TL;DR

This paper reports a rare case of AAV affecting both the central and peripheral nervous systems in a 59-year-old man from Oman, who showed improvement after treatment.

## Contribution

The novelty lies in the rare presentation of AAV with both central and peripheral nervous system involvement in a single patient.

## Key findings

- The patient exhibited mesenrhombencephalitis and sensorimotor axonal neuropathy with ANCA positivity.
- Treatment with intravenous steroids and rituximab led to partial neurological recovery.
- Nerve biopsy confirmed vasculitic neuropathy, supporting the AAV diagnosis.

## Abstract

The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) usually manifests as systemic vasculitis. AAV with central nervous system involvement in the form of mesenrhombencephalitis and peripheral neuropathy is extremely rare. We report a 59-year-old male patient who presented to a tertiary care hospital in Muscat, Oman, in 2024. He had a subacute presentation of altered sensorium, multiple cranial nerve palsy and ataxia with 3 months history of bilateral foot drop. Brain imaging was suggestive of mesenrhombencephalitis with subarachnoid and intraparenchymal haemorrhage. Nerve conduction studies revealed sensorimotor axonal neuropathy. Vasculitis profile showed perinuclear-ANCA positivity and the nerve biopsy was suggestive of vasculitic neuropathy. He was treated with intravenous steroids and maintained on rituximab. His neurological deficits improved with residual bilateral foot drop.