Case Report

Raeder's syndrome. A case with an unusual localization

José Pimentel, Isabel Pavão Martins

Department of Neurology, Hospital de Santa Maria, Lisbon, Portugal

Cephalalgia Pimental J, Martins IP. Reader's syndrome. A case with an unusual localization. Cephalalgia 1993;13:135.Oslo. ISSN 0333-1024

We describe a patient with a Raeder's paratrigeminal neuralgia consisting of left-sided frontal and orbital pain,Homer syndrome (including anhydrosis of the forehead), and sensory loss in the territory of the first division ofthe trigeminal nerve. The involvement of the ophthalmic nerve is not consistent with the usual localization ofthis syndrome to the pericarotid sympathetic plexus. Oculosympathetic and sympathetic fibers supplying thesweat glands of the forehead join the ophthalmic nerve in the cavernous plexus localized in the cavernoussinus. Therefore, this seems to be the most likely site of the lesion when the ophthalmic nerve is involved. •Headache, Raeder's paratrigeminal neuralgia

José Pimentel, Department of Neurology, Hospital de Santa Maria, Av. Prof. Egas Moniz, 1600 Lisboa,Portugal. Received 13 November 1992, accepted 4 January 1993

Raeder's paratrigeminal neuralgia is a rare syndrome with controversial topography and pathogenesis. Based on a review ofthe published cases, Boniuk and Schleszinger (1) divided this syndrome into two sub-groups: the first included cases similarto the original description by Raeder (2) and was generally associated with parasellar pathology; the second included caseswith no recognized structural damage, the lesion being probably postulated to the pericarotid plexus. We describe a patientwith a Raeder's paratrigeminal neuralgia whose symptoms are not consistent with any of these postulated localizations.

Case report

A 39-year-old man was admitted because of a persistent left periorbital and frontal pain of acute onset associated with afeeling of numbness in the forehead, and left-sided ptosis. He had no fever, history of inflammatory disease, cranial trauma orotological symptoms. The general physical examination was normal. Neurological examination disclosed a left-sided completeHomer syndrome and a sensory loss of the left forehead and left anterior scalp. Routine biochemical and hematological tests(including erythrocyte sedimentation rate), CT scan of the sellar and parasellar region and bilateral carotid angiograms wereall normal. He received corticosteroids (prednisone 60 mg daily) for five weeks. The sensory loss subsided within a month, butthe pain and oculosympathetic paralysis persisted for three more weeks.

Discussion

In 1924, Raeder described a syndrome consisting of orbital pain, parasellar nerve involvement (III, IV and VI) and anoculosympathetic palsy (2). Since then, other cases have been described under the same name, although they did notcompletely fit the original description of the syndrome (3). In 1962, Boniuk and Schleszinger (1) reviewed the literature andproposed subdividing Raeder's syndrome into two subgroups: the first group includes cases with multiple parasellar cranialnerve palsies (including the trigeminal nerve), plus an oculosympathetic paralysis but with intact facial sweating, i.e., similar tothe original report. These cases are usually associated with a variety of pathological processes such as tumors, aneurysms,inflammation or trauma, localized to the parasellar region (2). The second group included cases with a rapid onset ofperiorbital pain, oculosympathetic paralysis, and anhydrosis (when tested), but without evidence of other cranial nerveinvolvement. These cases are not consistently associated with a known structural disease and the localization of theunderlying lesion has been attributed to a posterolateral branch of the internal carotid nerve, the so-called sympathetic plexus,hence the designation pericarotid syndrome (3). Unlike the patients characterized as group II by Boniuk and Schleszinger (1),our patient had sensory impairment of the first division of cranial nerve V. This suggests that the site of the lesion extendsbeyond the carotid sympathetic plexus (3), with possible extension into the cavernous sinus.

Corticosteroid therapy provided significant relief of our patient's symptoms, suggesting an inflammatory pathophysiology ofthe syndrome even in idiopathic cases.

Acknowledgment. -We thank Maria Gabriela Baptista for typing the manuscript.

References

1. Boniuk M, Schleszinger NS. Raeder's paratrigeminal syndrome. Am J Ophthalmol 1962;54:1074-84

2. Raeder JG. "Paratrigeminal" paralysis of oculo-pupillary sympathetic. Brain 1924;47:149-58

3. Vijayan N, Watson C. Pericarotid syndrome. headache 1978;18:244-54