eMedicine Specialties > Neurology > Headache and Pain

Raeder Paratrigeminal Syndrome

Author: Steven H Schechter, MD, Clinical Assistant Professor, Department of Neurology, Wayne State University School of Medicine; Consulting Staff, Division of Neurology, Assistant Medical Director of Stroke Unit, William Beaumont Hospital
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Updated: Sep 11, 2008

Introduction

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.

Clinical

History

• History for Raeder paratrigeminal syndrome suggests trigeminal nerve involvement with pain, sensory or motor deficits, and/or ipsilateral oculosympathetic paresis.
  • It resembles Horner syndrome and manifests as oculosympathetic paresis with ptosis and miosis.
  • Unlike Horner syndrome, facial sweating and ipsilateral trigeminal sensory irritation are preserved, leading to production of facial pain.²
  • Other parasellar cranial nerves also may be involved. An enophthalmos also may be apparent, but iris pigmentation, which is common in acquired Horner syndrome, is rare in Raeder syndrome.³
• The pain associated with Raeder syndrome is deep and boring and is localized in or around the eye. Intermittent, lancinating pain also may occur.
  • Typically self-limited, the pain usually remits in 2-3 months.³
  • The pain occasionally follows a recurrent pattern. It can be associated with conjunctival tearing, erythema, enophthalmos, and decreased intraocular pressure. The pain is less well defined than the pain of trigeminal neuralgia.
• In 1962, Boniuk and Schlezinger described 2 subtypes.⁴
  • Group I included patients with neuralgia, oculosympathetic paralysis, and parasellar nerve involvement. The responsible lesions were believed to be localized to the middle cranial fossa, requiring an extensive workup (eg, local or metastatic tumors).
  • In group II, the condition occurred without parasellar involvement but was associated with oculosympathetic paralysis and neuralgia. These cases were felt to be more benign than those of group I.
  • Some causative conditions in group II included migraine, cluster headache, hypertension, trauma, inflammatory disease, sinusitis, syphilitic osteitis, herpes zoster, otitis, and pneumonitis.
  • Subcranial aneurysms also have been described as a cause in either subtype.⁵
• Grimson and Thompson described 3 major groups in 1980.⁶ While those in group I were felt to require an extensive evaluation, those in the 2 latter groups were felt to have a more benign prognosis. However, obtain a thorough evaluation as indicated, regardless of the subtype.
  • Group I included patients with multiple parasellar cranial involvement or involvement of any or all divisions of the trigeminal nerve.
  • Group II included those with cluster headache with an isolated oculosympathetic paresis.
  • Group III included those with pain atypical for cluster headache with involvement of the ophthalmic division of the trigeminal nerve.
• In 1978, 6 patients were described and 31 similar patients reviewed whose symptoms were consistent with a pericarotid syndrome.⁷ These patients' conditions were characterized by oculosympathetic paralysis, ipsilateral head pain, and anhidrosis with otherwise intact facial sweating.
  • In these cases, the site of the lesion of the oculosympathetic fibers was believed to be pericarotid rather than paratrigeminal in distribution.
  • According to these authors, the syndrome thus was renamed in the absence of intracranial disease. These patients constitute a unique clinical group with findings similar to Raeder syndrome that can be localized to an area in and around the internal carotid artery and affected by diverse pathologic processes.

Physical

• In the paratrigeminal syndrome described by Raeder, unilateral oculosympathetic paresis and evidence of trigeminal nerve involvement are the 2 hallmark features on clinical examination.⁸
• However, sweating is preserved, which is distinct from Horner syndrome, since some third-order sympathetic fibers are spared.
  • These particular fibers travel with the external carotid artery and its branches and are involved in the production of facial sweating.
  • The clinical localization is therefore above the carotid bifurcation (see Media file 2).
  • If additional parasellar cranial nerves are involved, the lesion may localize more specifically to the middle cranial fossa.²

Causes

Raeder felt the syndrome was due to a limited space-occupying lesion in the paratrigeminal area of the middle cranial fossa.

• Other diagnostic considerations
  • The differential diagnosis is broad and may include cerebral vascular dissection, middle cranial fossa tumors, carotid body tumor, fibromuscular dysplasia, migraine and cluster headache, syphilis, sinusitis, and osteitis.⁹
  • With the addition of parasellar cranial involvement, a mass lesion in this area should be considered.²
  • Horner syndrome is an additional consideration.

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