AAO: A headache with visual loss may require honing of detective skills

Reviewed by Kathleen B. Digre, MD

The occurrence of headache in conjunction with visual loss is a scenario that requires an in-depth examination including evaluation of the visual fields, pupils, and fundus to gather clues to the diagnosis. Imaging is a must for patients who describe a “thunderclap” onset of headache, according to Kathleen Digre, MD, speaking during the virtual American Academy of Ophthalmology (AA0) 2020 annual meeting.

Dr. Digre, a Distinguished Professor of Neurology and Ophthalmology at the John A. Moran Eye Center at the University of Utah in Salt Lake City, advised that physicians SNOOP to uncover Systemic symptoms, Neurologic signs/symptoms, Onset rapid in less than 1 minute, Older age (over 50 years), and Previous headache history/Progressive headache/Postural involvement (worse when upright)/Precipitated by exertion or Valsalva/Pulsatile tinnitus, visual obscuration/Pregnancy.

Pearls

Case 1
A 79-year-old man presented with transient monocular blindness and new holocranial headaches. He had a bilateral visual acuity (VA) of 20/20 and no afferent pupillary defect, but visual field evaluation showed an enlarged blind spot in the right eye.

His history was especially noteworthy for fatigue and recent weight loss, previous hypertension, and no previous headaches.

New headaches accompanied by visual loss in older patients always require further evaluation, Dr. Digre advised.

“Remember to snoop for clues in the patients’ history, including age, fatigue, weight loss, new-onset headache, although the headache type may not be helpful,” she said. Clues may be found in the VA, visual fields, pupils, and fundus. Fluorescein is a valuable tool.

She noted that amaurosis fugax is an important early visual symptom of giant cell arteritis and an ominous sign of impending blindness.

A pitfall is overlooking an underlying cause of the headaches even in the presence of good vision. In this patient, the erythrocyte sedimentation rate and the C reactive protein, respectively, were 60 and 10. The biopsy diagnosis was giant cell arteritis.

Case 2
A 58-year-old woman who reported no previous headaches presented with the complaint of new dull continuous right-sided headaches. However, she described episodes of severe headache with very sharp pain in the right frontotemporal area that lasted 10 to 15 minutes and recurred 5 times daily in addition to tearing and painful eyes and blurry vision.

The examination was essentially normal with 20/20 vision, full visual fields to confrontation, slight ptosis in the right eye, no pupillary defect but anisocoria with the smaller pupil on the right, and a normal fundus.

The combination in this case of homonymous hemianopia, Horner’s syndrome, and cluster-like headache pointed to a pituitary lesion. The diagnosis was a pituitary adenoma.

Dr. Digre commented, “The phenotype can often be a new headache and can be trigeminal autonomic cephalgia, like cluster headaches. This can be accompanied by tearing and Horner’s syndrome.” 

In cases in which the patients report unilateral continuous headaches, she advised looking for a Horner’s syndrome. Imaging, especially magnetic resonance imaging (MRI), is paramount in cases in which the headache is side-locked to one side.

Case 3
A 31-year-old pregnant woman with 2 children reported a sudden headache onset and a seizure accompanied by visual loss. Her blood pressure was 150/100; the patient was lethargic with briskly reactive pupils, and reported counting fingers vision. All extremities were able to move.

“In severe pre-eclampsia and eclampsia, the sites of the lesions that can affect vision include serous retinal artery detachment, hypertensive retinopathy, vasospasm, retinal artery occlusion, choroidal infarction, and anterior ischemic optic neuropathy. But don’t forget that it can also cause cerebral blindness,” Dr. Digre said.

This patient was diagnosed with Posterior reversible encephalopathy syndrome, which is characterized by sudden-onset headaches, visual loss, confusion, and seizure.

This syndrome, which often is associated with pregnancy, can cause blindness. It can be overlooked during evaluation. One of the keys to the diagnosis was that the patient’s pupillary constriction was brisk and intact. 

Another closely linked condition that requires consideration is reversible cerebrovascular constriction syndrome. This is characterized by thunderclap-onset repeated headaches, which patients can describe as the worst headache.

The segmental vasoconstriction can lead to stroke. Diagnosis of both of these conditions requires MR imaging and referral to a neurologist.

“Always ‘snoop’ if a patient presents with visual loss and headache, Dr. Digre concluded. “Call into play examination skills and tools in the evaluation of the visual fields, pupils, and fundus.

Patients with a side-locked headaches or those with a thunderclap onset should always undergo MRI unless the physician suspects an acute bleed.”

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Kathleen Digre, MD
E: Kathleen.digre@hsc.utah.edu​
Dr. Digre has no financial interest in this subject matter.