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

Neuropathology. 2010 Jun;30(3):267-72. doi: 10.1111/j.1440-1789.2009.01055.x. Epub 2009 Sep 14.

Abstract

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Cranial Nerve Diseases / complications
  • Cranial Nerve Diseases / diagnosis*
  • Cranial Nerve Diseases / therapy
  • Fatal Outcome
  • Humans
  • Male
  • Spinal Cord Diseases / complications
  • Spinal Cord Diseases / diagnosis*
  • Spinal Cord Diseases / therapy
  • Thoracic Vertebrae / pathology
  • Vasculitis, Central Nervous System / complications
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / therapy