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Raeder Paratrigeminal Syndrome

Author: Steven H Schechter, MD; Chief Editor: Robert A Egan, MD more...

Updated: Feb 1, 2012

Background
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 Etiology and Pathophysiology and Presentation.)

The first patient described by Raeder, in 1918, had an incomplete Horner syndrome with preserved sweating on the
side of the lesion. Raeder originally described absent facial anhidrosis in the paratrigeminal syndrome, although the
literature suggests no definite consensus concerning the facial sweating pattern.

Subtypes (Boniuk and Schlezinger)

In 1962, Boniuk and Schlezinger described 2 subtypes of Raeder paratrigeminal syndrome.[1] 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 patients, 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.[2] A rare cause includes carotid
dissection and should be considered early in the differential diagnosis of Raeder syndrome.[3]

Subtypes (Grimson and Thompson)

Grimson and Thompson described 3 major groups in 1980.[4] 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, the 3 of which are characterized as follows (see Etiology and
Pathophysiology and Presentation):

Group I - Includes patients with multiple parasellar cranial involvement or involvement of any or all divisions of
the trigeminal nerve
Group II - Includes patients with cluster headache with an isolated oculosympathetic paresis
Group III - Includes patients with pain atypical for cluster headache, with involvement of the ophthalmic division
of the trigeminal nerve

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An additional variant

In 1978, 6 patients were described and 31 similar patients reviewed whose symptoms were consistent with a
pericarotid syndrome.[5] 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.

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.

Epidemiology

Raeder paratrigeminal neuralgia is a rare syndrome. The exact incidence of this disorder in the United States is
unknown.[6] It is less common than Horner syndrome.

Raeder paratrigeminal syndrome seems to occur almost exclusively in males. 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.

Etiology and Pathophysiology

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

Benign forms of Raeder syndrome without paratrigeminal cranial nerve involvement have also been reported.[7] One
such case later evolved into an indomethacin-responsive hemicranial headache that fulfilled the diagnostic criteria for
hemicrania continua.[8]

The pathophysiologic site of the painful oculosympathetic palsy involves the location at which 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.

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 may place the
lesion best in the middle cranial fossa, medial to the trigeminal ganglion.[9]

Raeder syndrome localizes to lesions of the middle cranial fossa involving oculopupillary sympathetic fibers originating
from the internal carotid artery plexus and traveling with the trigeminal and oculomotor nerves.[10] Therefore, careful
imaging of this area is highly recommended.

Sweating is preserved in Raeder paratrigeminal syndrome, in contrast to Horner syndrome, since some third-order
sympathetic fibers are spared (see the image below). 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 distal to the
carotid bifurcation. If additional parasellar cranial nerves are involved, the lesion may localize more specifically to the
middle cranial fossa.[6]

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.)

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Associated morbidities

Raeder syndrome has been associated with several conditions, including the following:

Head trauma
Hypertension[11]
Vasculitis
Migraine headaches
Parasellar mass lesions
Internal carotid artery dissection or aneurysm

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

Morbidity and mortality

As previously mentioned, 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.

Contributor Information and Disclosures

Author
Steven H Schechter, MD Clinical Assistant Professor, Department of Neurology, Wayne State University School
of Medicine; Consulting Staff, Division of Neurology, William Beaumont Hospital

Steven H Schechter, MD, is a member of the following medical societies: American Academy of Neurology

Disclosure: Pfizer Honoraria Speaking and teaching; Novartis Honoraria Speaking and teaching; Forest Honoraria
Speaking and teaching; Biogen Idec Honoraria Speaking and teaching; Bayer Honoraria Speaking and teaching;
Accera Honoraria Speaking and teaching; Teva Speaking and teaching; Serono Honoraria Speaking and teaching

Chief Editor
Robert A Egan, MD Director of Neuro-Ophthalmology, St Helena Hospital

Robert A Egan, MD is a member of the following medical societies: American Academy of Neurology, American
Heart Association, North American Neuro-Ophthalmology Society, and Oregon Medical Association

Disclosure: Nothing to disclose.

Additional Contributors
Jorge E Mendizabal, MD Consulting Staff, Corpus Christi Neurology

Jorge E Mendizabal, MD is a member of the following medical societies: American Academy of Neurology,
American Headache Society, National Stroke Association, and Stroke Council of the American Heart Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College
of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

References
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1962;54:1074-84. [Medline].

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Jan 1968;18(1 Pt 1):43-6. [Medline].

3. Epifanov, Y, Back, T. Oculosympathetic Paratrigeminal Paralysis With Isolated V@ Involvement in Carotid

Artery Dissection. Arch Neurol. march/2007;64:448-449.

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12. 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].

13. Raeder JG. "Paratrigeminal" paralysis of oculo-pupillary sympathetic. Brain. 1924;47:149-158.

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15. Murphy, MM, Szabados, EM, Mitty, JA. Lyme disease Associated with Postganglionic Horner Syndrome and
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