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13.9 Paratrigeminal oculosympathetic (Raeder’s) syndrome


Constant, unilateral pain in the distribution of the ophthalmic division of the trigeminal nerve, sometimes extending to the maxillary division, accompanied by ipsilateral Horner’s syndrome and caused by a disorder in the middle cranial fossa or of the carotid artery.

Diagnostic criteria:
  1. Constant, unilateral headache fulfilling criterion C
  2. Ipsilateral Horner’s syndrome, with imaging evidence of underlying disease of either the middle cranial fossa or the ipsilateral carotid artery
  3. Evidence of causation demonstrated by both of the following:
      1. headache has developed in temporal relation to the onset of the underlying disorder, or led to its discovery
      2. headache has either or both of the following features:
        • a) localized to the distribution of the ophthalmic division of the trigeminal nerve, with or without spread to the maxillary division
        • b) aggravated by eye movement
  4. Not better accounted for by another ICHD-3 diagnosis.

The original description of 13.9 Paratrigeminal oculosympathetic (Raeder’s) syndrome is regarded as a classical example of clinico-anatomical methodology in the early 20th century, and was useful because the involvement of oculopupillary sympathetic fibres indicated a lesion of the middle cranial fossa. Whether the term Raeder’s syndrome should be used today is heavily debated, but painful Horner’s syndrome is still considered by some authors to be a diagnostically useful indication of a middle cranial fossa lesion or of carotid artery dissection.