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.

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.

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