Excerpt from Raeder Paratrigeminal Syndrome

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Background

First described by Raeder in 1918, Raeder paratrigeminal syndrome (ie, paratrigeminal neuralgia) is characterized by severe unilateral facial pain and headache in the distribution of the ophthalmic division of the trigeminal nerve in combination with ipsilateral oculosympathetic palsy or Horner syndrome (see Media file 1). The first patient described by Raeder had an incomplete Horner syndrome with preserved sweating on the side of the lesion.

Pathophysiology

The pathophysiologic site of the painful oculosympathetic palsy involves the location where oculosympathetic fibers exit the internal carotid artery to join the ophthalmic division of the trigeminal nerve. Various combinations of cranial deficiencies (nerves II-VI) also may be involved.

Raeder originally described absent facial anhidrosis in the paratrigeminal syndrome, although the literature suggests no definite consensus concerning the facial sweating pattern.

According to Goadsby, paratrigeminal oculosympathetic syndrome may be a more accurate name than Raeder paratrigeminal syndrome, because an analysis of the anatomy of the oculosympathetic innervation might place the lesion best in the middle cranial fossa, medial to the trigeminal ganglion.¹ Therefore, careful imaging of this area is highly recommended.

Frequency

United States

Raeder paratrigeminal neuralgia is a rare syndrome. The exact incidence of Raeder syndrome is unknown.² It is less common than Horner syndrome.

Mortality/Morbidity

Raeder syndrome has been associated with several conditions, including head trauma, hypertension, vasculitis, migraine headaches, parasellar mass lesions, and internal carotid artery dissection or aneurysm. Therefore, the morbidity and mortality depend on the underlying etiology, and the diagnosis of the condition warrants a full evaluation to identify an underlying cause.

Race

No racial predilection is reported.

Sex

Males seem to be affected almost exclusively.

Age

Case reports vary as to the age of onset or diagnosis. The original case reports from Raeder involved patients aged 18-65 years. However, onset appears most prevalent in middle or old age.

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