This page was last updated on: September 17, 2007

Central nervous system vasculitis: The first case of CNS vasculitis was reported in 1988 by Stewart et al and was confirmed microscopically on autopsy as resulting from diffuse small-artery and medium-artery vasculitis of the brain.

• Since the neurologic sequelae of RPC are diverse, patients may present with seizures, limb weakness, paresthesias or gait disturbances, or other cerebellar symptoms.
  

• Neurologic symptoms may present before other more frequent manifestations of RPC.
  

• Marie et al reported a case of diplopia as the first presenting manifestation of RPC, which led to the finding that the patient had a right sixth cranial nerve palsy because of CNS vasculitis. McAdam et al reported a case of left seventh cranial nerve palsy in RPC. Cranial nerve damage is common in RPC-associated CNS vasculitis and most often affects the second cranial nerve, followed less commonly by the sixth, seventh, and eighth cranial nerves.
  

• Aseptic meningitis has been reported infrequently in RPC.
  

• Clinical neurologic assessment is an important aspect of the physical examination of patients with RPC.
  

The neurologic sequelae of Polychondritis are quite diverse, patients  may present with seizure, limb weakness, paresthesia or gait disturbance, or other cerebellar symptoms. Neurologic symptoms may manifest before other more frequent manifestations of RPC. Marie and coworkers reported a case of diplopia as the first presenting manifestation of RPC, which led to the finding that the patient had a right sixth nerve palsy due to CNS vasculitis. McAdam and co-workers reported a case of left seventh nerve palsy in Polychondritis. Cranial nerve damage is common in Relapsing Polychondritis associated CNS vasculitis and most often affects the second cranial nerve; the sixth, seventh, and eighth nerves are affected less commonly. Aseptic meningitis has been reported infrequently in patients with Polychondritis.

Clinical neurological assessment is an important aspect of the physical examination of patients with RPC.

Central Nervous System Vasculitis