Wegener's granulomatosis presenting with bulbar palsy and bilateral jugular vein compression

staining) in the area of the left supratrochlear and supraorbital nerve and the left ramus nasociliary nerve branch. The lacrimal fluid was also VZV positive, determined by polymerase chain reaction. The ophthalmologic examination revealed conjunctivitis and episcleritis, and the sensibility status showed a sensory reduction in the area of vesicles and rush. The vesicles healed within 3 weeks with 2 weeks of acyclovir therapy. During the entire time of acute herpes zoster ophthalmicus infection to the end of healing, the patient reported no pain in the affected region. At the two following monthly visits, she had mild skin scarring in the affected areas, a mild left-sided keratitis, and a mild paralytic lid closure related to the left oculomotor nerve. One year after the onset of HZP, the same patient was diagnosed with a secondary glaucoma in the left eye, as a result of scarring of the entrance to the meshwork drains between cornea and peripheral iris. Still, no pain was recorded. Seven years later, the patient presented to our pain clinic because of constant burning pain in the left supraand infraorbital region combined with hyperpathia and crawling sensations. The slowly developing burning, partially searing pain was first recognized several weeks earlier. The examination showed multiple small scars, a mild ptosis, but no vesicles a t the left eye. The sensory status showed hyperesthesia and allodynia in the supratrochlear, supraand infraorbital region and a mild left eye ptosis. The latter was interpreted as a result of the involvement of the third cranial nerve to the former herpes zoster infection in combination with a mild intis. The skin temperature did not differ from that of the other side. No inflammatory disease was found, and cerebral CT as well as MRI angiography showed normal conditions. The dermatologic examination found neither a new VZV infection nor any connective tissue disease. The ophthalmologic examination confirmed a mild ptosis. In light of the acute herpes zoster syndrome 7 years earlier and excluding other possible alternative causes of facial pain syndromes (idiopathic trigeminal neuralgia or cluster headache), we assigned this actual pain syndrome to a late appearance of PHN. No experimental or clinical study to date can explain the latent mechanism suggested in PHN. There are many hypotheses explaining possible pathways. One of them is the central sensitization theory. Sensitized nociceptors fail to return to normal after the inflammation subsides or they are more easily sensitized than in normal conditions, so they might contribute to spontaneous pain sensation^.^ Another explanation may be the fact that afferents from surrounding undamaged nerves innervate the partially denervated territory of the herpetic dermatome and cause abnormal pain sensations.5 In our case, we do have some evidence for PHN occurring 7 years after acute HZP infection without pain. PHN may be found several years after an acute herpes zoster syndrome without appearance of acute pain. The difference from the recently described acute zoster sine herpete, as described by Gilden et al. in 1994,‘j is the chronic pain condition, which is a constant burning, but not a state of sharp, shooting pain. The pathophysiologic background of the very late appearance of PHN remains unclear. Epidemiologic studies should be done to determine to what extent PHN occurs after acute and pain-free herpes zoster. This case report may stimulate additional reports from other investigators.