Primary angiitis of the CNS (PACNS) with predominant cranial neuropathy and spinal cord involvement.

We describe a 70-year old man with a history of repeated epidural injections for chronic low back pain, presenting with headache, cranial nerve palsies and progressive myelopathy. Meningeal enhancement was initially seen in the posterior epidural space of the T(10)-T(12) spine on MRI. Extensive laboratory investigation showed normal or negative results except for persistent pleocytosis, elevated protein and absence of demonstrable microorganisms on CSF studies. Despite conventional and empirical treatments, the patient developed progressive neurological deterioration leading to death. Autopsy showed Primary angiitis of the CNS (PACNS) with predominant cranial neuropathy, spinal cord involvement and extensive myelomalacia.