18

Acute Painful Ophthalmoplegia

CASE NO. 18

A 37-year-old African American female presented with an acute onset of painful ophthalmoplegia OS. Her past medical, family, surgical, and social history were unremarkable. She was taking no medications. She did not smoke or consume alcohol. She had no other neurologic signs or symptoms.

Exam

The patient’s visual acuity was 20/20 OU. The pupils were 4 mm OU and were reactive to light bilaterally. There was no relative afferent pupillary defect. The motility exam showed a primary position exotropia of 40 prism diopters that increased on gaze to the left to >55 prism diopters. There was limitation of adduction, elevation, and depression OD and there was a mild abduction deficit OD as well (Fig. 18.1). Her pain was limited to the V1 distribution on the face OD and she had mildly decreased cutaneous pinprick sensation in the V1 distribution on the right. The fourth nerve function seemed intact with intorsion in downgaze seen OD.

Dr. Brazis. In a patient with ophthalmoplegia and ophthalmic branch trigeminal (V1) distribution pain or numbness, a cavernous sinus lesion should be considered. The mild abduction deficit is also suggestive of a partial sixth nerve dysfunction. Combined ocular motor paresis and sympathetic denervation (not seen in this patient) are virtually pathognomonic of a cavernous sinus lesion. Compressive

163

Emergencies in Neuro-Ophthalmology: A Case Based Approach

Fig. 18.1. Motility photographs show the limitation of elevation, depression and adduction OD. There was a minimal abduction deficit OD as well with a small esotropia in right gaze of 5 prism diopters. Note that the primary position deviation is not as large as one might expect from a medial rectus palsy alone, suggesting that the mild abduction deficit OD is an associated sixth nerve palsy.

cavernous sinus lesions may also spare the pupil because they often preferentially involve only the superior division of the oculomotor nerve, which carries no pupillomotor fibers, or the superior aspect of the nerve anterior to the point where the pupillomotor fibers descend in their course near the inferior oblique muscle. The pupillary “sparing” with anterior cavernous sinus lesions may be more apparent than real, resulting from simultaneous injury of nerve fibers to both the pupillary sphincter and dilator, resulting in a midposition, fixed pupil. The sympathetic fibers to the eye join the abducens nerve for a short distance within the cavernous sinus, and thus a unilateral abducens nerve lesion associated with an ipsilateral Horner syndrome is of localizing value.

Pituitary adenomas, nasopharyngeal carcinomas, craniopharyngiomas, and metastases most commonly may cause a cavernous

164

Acute Painful Ophthalmoplegia

sinus syndrome. Sphenoid sinus carcinoma often causes a sphenocavernous syndrome, but may also present with an isolated sixth nerve palsy. Nasopharyngeal carcinoma may compress the sixth nerve as many of these tumors arise from the fossa of Rosenmuller immediately beneath the foramen lacerum. Extension of the tumor through the foramen lacerum may cause a trigeminal sensory loss (e.g. affecting a V2 distribution) and a sixth nerve palsy. Thus, the combination of facial pain or V2 sensory loss with a sixth nerve palsy is a common presentation of nasopharyngeal carcinoma. Serous otitis media is a frequent accompaniment due to blockage of the Eustacian tube.

Carotid-cavernous sinus dural arteriovenous fistulae may present with unilateral or bilateral cavernous sinus syndromes. Tolosa–Hunt syndrome, which is caused by inflammatory processes of various etiologies involving the cavernous sinus, presents with ocular motor weakness and retroorbital pain. Facial sensation and visual acuity may be diminished. Tolosa–Hunt syndrome or painful ophthalmoplegia is a diagnosis of exclusion. Sudden onset of headache and dysfunction of multiple ocular motor nerves on either one or both sides, with or without retroorbital pain or visual impairment, suggests the possibility of pituitary apoplexy. In all patients with a cavernous sinus syndrome, infectious etiologies, especially due to Aspergillus or Mucormycosis, are of concern, especially if the patient is immunocompromised.

The patient described needs an MRI with specific attention to the cavernous sinus and superior orbital fissure regions.

Dr. Lee. I completely agree with Dr. Brazis that the combination of an ispilateral third, fifth (V1), and in this case a possible sixth nerve palsy suggests a cavernous sinus localization. The Tolosa–Hunt syndrome is a painful ophthalmoplegia syndrome that is typically steroid responsive and is as Dr. Brazis said, a “diagnosis of exclusion.” I generally order an MRI with contrast and I direct specific attention to the ipsilateral cavernous sinus. The International Headache Society (IHS) redefined the prior diagnostic criteria for the

165

Emergencies in Neuro-Ophthalmology: A Case Based Approach

entity known as the “Tolosa–Hunt syndrome” (THS). The diagnostic criteria require that granuloma be demonstrated by MRI or biopsy. La Mantia et al. reviewed the literature on THS from 1988 to 2002 and found 124 THS cases. Forty-four cases (35%) had inflammation on MRI or biopsy, evidence of granuloma, and 41/124 (33%) had normal neuroimaging. Interestingly, 39 (31%) had a specific cause and so the THS was “secondary.” The authors concluded that the clinical presentations for THS are common to several conditions and “their application alone does not guarantee a correct diagnosis.”

Most of the cases of THS referred to me end up being secondary to an underlying and potentially treatable etiology and I would caution the readers to use the diagnosis with caution.

Course. This patient underwent a contrast MRI (Fig. 18.2) which showed enhancement in the right cavernous sinus. An extensive

Fig. 18.2. Coronal MRI with contrast shows posterior cavernous sinus enhancement (arrow).

166

Acute Painful Ophthalmoplegia

evaluation for alternative etiologies was performed and was negative, and a presumptive diagnosis of the Tolosa–Hunt syndrome was made. Intravenous steroid therapy alleviated the patient’s pain. In patients with a negative radiographic and laboratory evaluation, complete resolution of clinical signs and symptoms and radiographic findings, and no recurrence of findings after steroid treatment, the diagnosis of Tolosa–Hunt syndrome can be presumed without necessarily pursuing a biopsy.

167