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Raeder Paratrigeminal Syndrome

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.
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.
A rare cause includes carotid dissection and should be considered early in the differential diagnosis of Raeder syndrome.

Subtypes (Grimson and Thompson)

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, 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

An additional variant

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

 

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