13.3.2.1 Painful nervus intermedius neuropathy attributed to herpes zoster

Previously used term:

13.3.2.1 Painful nervus intermedius neuropathy attributed to herpes zoster associated with facial paresis is known as Ramsay Hunt syndrome.

Description:

Unilateral continuous or near-continuous pain, with or without brief paroxysms superimposed, in the distribution of nervus intermedius and felt deeply in the auditory canal, caused by nervus intermedius herpes zoster infection and commonly associated with facial paresis and other symptoms and/or clinical signs of the infection or its aftermath.

Diagnostic criteria:
  1. Unilateral continuous or near-continuous pain1 in the distribution of nervus intermedius2 and fulfilling criterion C
  2. One or more of the following:
    1. herpetic eruption has occurred in the distribution of nervus intermedius3
    2. Varicella zoster virus (VZV) has been detected in the CSF by polymerase chain reaction (PCR)
    3. direct immunofluorescence assay for VZV antigen or PCR assay for VZV DNA is positive in cells obtained from the base of lesions
  3. Pain developed in temporal relation to the herpes zoster4
  4. Not better accounted for by another ICHD-3 diagnosis5.
Notes:
  1. Brief paroxysms may be superimposed, but are not the predominant pain type.
  2. In the auditory canal, auricle and/or region of the mastoid process.
  3. Due to viral spread, other cranial nerves may become affected.
  4. Pain can precede the herpetic eruption.
  5. The diagnosis is confirmed clinically in the acute stages by detection of vesicles on the tympanic membrane, auditory canal, auricle and/or skin overlying the mastoid process. They may also be seen in the anterior third of the tongue, which the virus has reached via chorda tympani, or on the hard palate, supplied by a vestigial remnant branch of the facial nerve.
Comments:

Other cranial nerves (VIII, IX, X, XI) may also be affected, leading to tinnitus, hearing loss, vertigo, nausea, hoarseness and dysphagia.

While little is known of the natural course of 13.3.2.1 Painful nervus intermedius neuropathy attributed to herpes zoster, the pain may continue for more than 3 months; it should then be classified as 13.3.2.2 Post-herpetic neuralgia of nervus intermedius.