Emergencies in Neuro-Ophthalmology: A Case Based Approach

REFERENCES

Biousse V, Touboul PJ, D’Anglejan-Chatillon J, et al. (1998) Ophthalmologic manifestations of internal carotid artery dissection. Am J Ophthalmol 126:565–577.

Purvin V, Kawasaki A. (2005) Neuro-ophthalmic emergencies for the neurologist.

Neurologist 11:195–233.

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15

Jaw Pain and Headache in an

Elderly Woman

CASE NO. 15

A 70-year-old woman presents with a three-week history of right sided headache. She described the pain as dull, present throughout the day with no relieving factors. She also admitted to feeling unusually tired and had had an unplanned 10 lb weight loss over the past one month. Over the previous few days, she described pain over the right jaw after chewing her food. She denied any visual symptoms. At a party the previous day, several friends commented on a prominent “bump” which had appeared on the right side of her head (Fig. 15.1). Her husband reported that she was reluctant to comb her hair due to the pain.

Her past medical history was significant for hypertension and osteoarthritis. Regular medication included metoprolol and ibuprofen. There was no family history of headaches. She was a non smoker and did not consume alcohol.

On examination, her visual acuity was 20/25 OD and 20/30 OS. There was no relative afferent pupillary defect. Fundus examination showed possible mild disc pallor OU and the Goldmann perimetry showed mild constriction. An OCT showed normal retinal nerve fiber layer OU. The remainder of the eye exam was normal.

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Emergencies in Neuro-Ophthalmology: A Case Based Approach

Fig. 15.1. Prominent vessel on the right side of the head.

Dr. Lee. Elderly patients who present with an acute onset of any of the following symptoms or signs should be considered as having giant cell arteritis until proven otherwise:

•new onset headache

•jaw pain or fatigue with chewing

•neck or ear pain

•acute loss of vision

•transient visual loss

•bulging, tender temporal artery

•optic disc edema

•transient diplopia

•unexplained ophthalmoplegia.

Pain when combing the hair or tenderness along the temporal artery is highly suggestive of symptoms of temporal arteritis. A relatively normal eye exam should not dissuade the clinician from consideration of the diagnosis. The most common error for the ophthalmologist would be finding a normal eye exam and then either ignoring or misdiagnosing the headache and other constitutional symptoms. Patients with temporal arteritis may have a prior history of active

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Jaw Pain and Headache in an Elderly Woman

symptoms of polymyalgia rheumatica (proximal muscle pain in the hip and shoulder girdle with morning stiffness). In patients suspected of having temporal arteritis, I typically start empiric steroids and order both a serum erythrocyte sedimentation rate (ESR) and a C-reactive protein (CRP). Other acute phase reactants (e.g. elevated platelet count) could be considered but I favor the ESR and CRP as screening tests, followed by a unilateral temporal artery biopsy. If the clinical suspicion is high for the diagnosis, I would proceed to a contralateral temporal artery biopsy if the initial biopsy was negative. I tend to treat with prednisone (1.0 to 1.5 mg per kg per day) during the evaluation phase. Although the evidence is somewhat controversial, I do consider intravenous steroids for patients who are monocular, have bilateral simultaneous or rapidly sequential visual symptoms or signs, have severe visual loss (especially if less than a few days of onset), or who have transient visual loss. There is no prospective, randomized, head to head data comparing intravenous to oral steroids, however. Most of my patients with biopsy proven temporal arteritis require therapy for months to years. I write (or call) the primary care doctor to provide them with information about the diagnosis, warn them about the impending steroid related side effects (especially in frail older patients), encourage them not to taper the steroids, and ask them to follow the patient and provide treatment for osteoporosis prophylaxis (e.g. vitamin D, calcium, DEXA bone density).

Dr. Brazis. I agree with Dr. Lee’s comments. In our Clinic, we perform a temporal artery biopsy unilaterally and interpret this immediately via frozen section. If the unilateral biopsy is negative, we go on to perform a biopsy on the contralateral side. If the surgeon does not have the “luxury” of frozen section interpretation, I would suggest bilateral biopsies in most patients as the yield of doing the second side is perhaps 2 to 3%, and one never wants to miss this diagnosis because of the potentially catastrophic visual loss that may result if the diagnosis is missed and treatment is not continued. We also suggest intravenous corticosteroids in the scenarios described by Dr Lee. We place all patients on aspirin, if there are no contraindications, as this agent may decrease subsequent ischemic risk; the

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