AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

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 Image 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. 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 (Murane, 1996). 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

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

• History 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 (Murane, 1996).
  • 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 (Nolph, 1982).
• 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 (Nolph, 1982).
  • 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.
• • 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 (Law, 1968).
• Grimson and Thompson described 3 major groups in 1980. 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 (Vijayan, 1978). 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.
• 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 Image 2).
  • If additional parasellar cranial nerves are involved, the lesion may localize more specifically to the middle cranial fossa (Murnane, 1996).

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 (Selky, 1995).
  • With the addition of parasellar cranial involvement, a mass lesion in this area should be considered (Murnane, 1996).
  • Horner syndrome is an additional consideration.

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Brainstem syndromes
Granulomatous angiitis of the central nervous system
Carotid disease and stroke

WORKUP

Section 5 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• While most cases are benign, a thorough evaluation to exclude secondary causes may be warranted, particularly if parasellar nerve involvement is evident.
• Basic laboratory analysis to evaluate for inflammatory or infectious etiologies also may be warranted.
• A basic chemistry profile, CBC count, erythrocyte sedimentation rate, antinuclear antibody, and rheumatoid factor may be helpful in screening for inflammatory or infectious causes.

Imaging Studies

• The neurodiagnostic evaluation should include magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the brain. These studies help to exclude secondary causes such as dissection, vascular anomaly, or aneurysm.
• Cerebral arteriography may be considered if clinically indicated or if MRA findings warrant further evaluation.

TREATMENT

Section 6 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical Care

• Unless intracranial pathology exists, treatment remains predominantly symptomatic.
• Avoidance of vasodilators and alcohol is recommended due to potential for exacerbation of pain.
• When parasellar involvement is absent, steroids (either oral or intravenous) may be beneficial.
• Nolph and Dion also have suggested analgesics, ergotamines, and vitamin B therapy.

Surgical Care

Surgery is not indicated for most patients unless a secondary cause is found that justifies surgical intervention.

MEDICATION

Section 7 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

For pain management, gabapentin, pregabalin, carbamazepine, other anticonvulsants, or baclofen may be effective. Anti-inflammatory agents can also be effective, and, at times, narcotic analgesics may be necessary. The efficacy of TCAs has been demonstrated in controlled trials for idiopathic facial pain and appears to be independent of the antidepressant effect. Steroids may also be effective in some patients.

Drug Category: Anticonvulsants

Many agents in this class are effective in the treatment of chronic pain syndromes.

Drug Category: Analgesics

Pain control is essential to quality patient care. Ensure patient comfort, promote pulmonary toilet, and have sedating properties, which are beneficial for patients who have sustained trauma or injuries.

Drug Category: Corticosteroids

Have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Drug Category: Tricyclic antidepressants

A complex group of drugs that have central and peripheral anticholinergic effects, as well as sedative effects. They have central effects on pain transmission. They block the active reuptake of norepinephrine and serotonin.

Drug Category: Antispasticity agents

Effective in some patients for pain management.

FOLLOW-UP

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Prognosis

• Symptomatic resolution usually occurs within an interval of 2-3 months. If pain persists or if atypical features are noted, investigate and manage secondary causes.

MISCELLANEOUS

Section 9 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• If the diagnosis is in question, atypical features are present, or neurologic deficits are noted on the neurologic examination, a thorough evaluation for other etiologies (eg, aneurysm, mass) is warranted.

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Evident are the mild ptosis of the left upper eyelid, the slight elevation of the left lower eyelid, and the miosis of the left eye. Reprinted with permission (Copyright American Society of Contemporary Ophthalmology. Annals of Ophthalmology 1978; 10(9); 1181-1187.)
	View Full Size Image
Media type:  Photo

Media file 2:  Cervical sympathetic pathway including oculosympathetic fibers. A lesion at A would produce a complete Horner syndrome with ipsilateral loss of facial sweating; a lesion at B would produce oculosympathetic paresis, but with preserved facial sweating. Reprinted with permission (Hanley and Belfus, Inc. Academic Emergency Medicine 1996; 3(9); 864-867.)
	View Full Size Image
Media type:  Graph

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

• Appen RE, Sturm RJ. Raeder''s paratrigeminal syndrome. Ann Ophthalmol. Sep 1978;10(9):1181-7. [Medline].
• Bajwa ZH, Ho CC. Causes of Facial Pain [UpToDate Web site]. 2004.
• Boniuk M, Schlezinger NS. Raeder's paratrigeminal syndrome. Am J Ophthalmol. Dec 1962;54:1074-84. [Medline].
• Goadsby PJ. Raeder''s syndrome [corrected]: paratrigeminal paralysis of the oculopupillary sympathetic system. J Neurol Neurosurg Psychiatry. Mar 2002;72(3):297-9. [Medline].
• Grimson BS, Thompson HS. Raeder''s syndrome. A clinical review. Surv Ophthalmol. Jan-Feb 1980;24(4):199-210. [Medline].
• Law WR, Nelson ER. Internal carotid aneurysm as a cause of Raeder''s paratrigeminal syndrome. Neurology. Jan 1968;18(1 Pt 1):43-6. [Medline].
• Murnane M, Proano L. Raeder''s paratrigeminal syndrome: a case report. Acad Emerg Med. Sep 1996;3(9):864-7. [Medline].
• Nolph MB, Dion MW. Raeder''s syndrome associated with internal carotid artery dilation and sinusitis. Laryngoscope. Oct 1982;92(10 Pt 1):1144-8. [Medline].
• Raeder JG. "Paratrigeminal" paralysis of oculo-pupillary sympathetic. Brain. 1924;47:149-158.
• Selky AK, Pascuzzi R. Raeder''s paratrigeminal syndrome due to spontaneous dissection of the cervical and petrous internal carotid artery. Headache. Jul-Aug 1995;35(7):432-4. [Medline].
• Solomon S. Raeder syndrome. Arch Neurol. Apr 2001;58(4):661-2. [Medline].
• Vijayan N, Watson C. Pericarotid syndrome. Headache. Nov 1978;18(5):244-54. [Medline].

Article Last Updated: Aug 29, 2006