Ocular symptoms common with neurological conditions in women

Women are more likely to experience neurological problems, such as migraines and multiple sclerosis, with ophthalmic complications.

When women enter an ophthalmologist’s office complaining of headaches or sudden visual problems, the physician must be aware that these symptoms can be indicators of neurological conditions that target women. Ophthalmologists are often the first physicians to see patients who are experiencing migraines or the onset of multiple sclerosis, experts say.

“Most neurological conditions that have ophthalmic manifestations are symptomatic with symptoms such as pain, diplopia, redness and visual loss,” Deborah I. Friedman, MD, told Ocular Surgery News in an e-mail interview. “Most often, the patient will have a complaint that will guide the history and exam.”

“It’s important to do a good exam,” Jacqueline S. Winterkorn, MD, PhD, said. “If the doctor is not comfortable handling neurologic visual fields and looking for an afferent pupillary defect, he should refer the patient to a neuro-ophthalmologist,” she said.

Drs. Friedman and Winterkorn, along with Joseph F. Rizzo III, MD, spoke with OSN about neurological disorders with ocular complications that are commonly seen in women.

Multiple sclerosis

One neurological disorder that shows a definite predominance of women is multiple sclerosis, which often initially presents with visual symptoms.

Patients tend to present first to an ophthalmologist because they notice their loss of vision from optic neuritis or double vision due to inflammation, Dr. Rizzo said.

Dr. Friedman said the initial signs of multiple sclerosis include monocular vision loss associated with pain on eye movement, diplopia, oscillopsia, nystagmus, dorsal midbrain syndrome and homonymous hemianopia. A small percentage of patients present with pars planitis, perivenous sheathing or uveitis, she added.

Jacqueline S. Winterkorn

An exam, she said, may show acuity loss, relative afferent pupillary defect or visual field defect, and the patient could have optic disc edema.

Dr. Friedman said the neurologist’s treatment may include intravenous corticosteroids, immunomodulating agents, and symptomatic treatment of spasticity, depression and bladder dysfunction.

“Nowadays, anyone with optic neuritis deserves an MRI, both with and without gadolinium, and if they have signs of multiple sclerosis on their scan, even one lesion, then they are referred to a neurologist to start Avonex,” Dr. Winterkorn said.

On the ophthalmic side, “We tend to treat these patients with intravenous Solu-Medrol (methylprednisolone sodium succinate, Pfizer) but that doesn’t make them get better. It just makes them get better a little faster,” she said.

“We stay away from oral prednisone because the Optic Neuritis Treatment Trial showed that patients are more likely to have another attack of optic neuritis if you treat them with prednisone.”

Migraines

Although migraines can occur at any point, after puberty, women are more likely to suffer from the vision-affecting headaches. The peak prevalence in women and men is between ages 30 and 50.

“It becomes much more predominantly a women’s problem, such that twice as many women have migraines as men,” Dr. Winterkorn said. “Attacks would start in the 20s and continue on until 50.”

The physicians interviewed for this article said many patients with migraines present with visual phenomena or aura, which can range from zigzag lines or aura to fragmented vision or complete visual loss. These types of symptoms are associated with “classic migraines,” Dr. Rizzo said.

“They experience visual images as a part of migraine event, so patients often go to ophthalmologists to understand the nature of the problem,” he said. “The role of an ophthalmologist is to recognize that it’s a migrainous event and refer the patient to a neurologist.”

Dr. Winterkorn said she instructs residents to ask patients about the location and consistency of the visual loss and pain.

“You have to investigate any headache that is always on the same side. If it doesn’t follow the rules, if the aura takes longer than the typical time or it’s always on the same side or there are visual field defects in between attacks, then you have to go aggressively and look for some other reason for the headache,” Dr. Winterkorn said.

“You can’t do an MRI on every patient who has a headache, so you have to have certain rules about when to refer a patient to a neuro-ophthalmologists and when to send a patient home.”

Dr. Rizzo agreed, saying, “There are occasional instances in which visual phenomena will be misinterpreted to be relating to migraine but, in fact, could be related to some other more significant neurological problem.”

If the patient experiences persistent visual loss without a headache or if the visual phenomena last longer than the typical hour, Dr. Winterkorn said neuroimaging should be pursued.

Dr. Friedman discussed ophthalmoplegic migraine, which can be accompanied by diplopia and ptosis.

“Ophthalmoplegic migraine consists of a headache followed by an oculomotor nerve palsy that may last weeks after the pain resolves,” she said. “It is most likely inflammatory, rather than migrainous.”

Dr. Friedman said although there is no specific treatment for aura or visual phenomena, the migrainous headaches may be treated by prophylactic or symptomatic therapy. Transient monocular visual loss is often successfully prevented with verapamil.

Thyroid eye disease

Thyroid eye disease, also known as Graves’ orbitopathy or Graves’ disease, affects women in their 50s and 60s and may be associated with thyroid abnormalities. It is often heralded by dry eye symptoms.

Deborah I. Friedman

“One can develop the orbitopathy of Graves’ disease in situations where there’s a hyperactive thyroid gland, a hypoactive thyroid gland or a normally functioning thyroid gland, so the two conditions are related but not directly,” Dr. Rizzo said.

Dr. Friedman said patients usually present with eye irritation and other signs and symptoms of dry eyes, including conjunctival injection, diplopia and periocular pain.

An exam, Dr. Friedman said, might show corneal exposure, conjunctival injection, injection over the extraocular muscle insertions, unilateral or bilateral proptosis, restriction of ocular motility, eyelid retraction, lid lag, lid erythema or edema.

“In thyroid eye disease, the antibodies that are made to the thyroid gland attack the eye muscles, and the eye muscles get infiltrated with lymphocytes. They are inflamed and they don’t contract as well and, as a result, you get double vision,” Dr. Winterkorn said.

“Patients with orbitopathy of Graves’ disease most typically experience discomfort because of problems on the ocular surface, so they have low-level chronic discomfort of the eye,” Dr. Rizzo said. He added that the eyes could become protuberant as well, causing cosmetic problems.

All three neuro-ophthalmologists agree that in the worst cases, inflammation could be so great that the muscles swell and press on the optic nerve, causing visual loss.

Treatment comes in the form of lubricants and patching to avoid diplopia, along with radiation therapy and orbital decompression surgery, Dr. Friedman said.

She said corticosteroids are sometimes used, although there is conflicting data regarding their efficacy. Although signs of optic neuropathy are uncommon, all patients in the active phase of the disease should have routine perimetry every few months.

“If there is no known thyroid disease, patients should be evaluated for it,” Dr. Friedman said, although, “treating the thyroid condition usually has no significant effect on the ophthalmic manifestations.”

She said the disorder generally runs 18 months and then stabilizes, but it can leave the patient with dry eyes, permanent disfigurement from lid retraction, lid erythema and proptosis, diplopia and, rarely, visual loss from optic neuropathy.

Giant cell arteritis

Giant cell, or temporal, arteritis presents in women 60 or older with symptoms that may lead the patient directly to the ophthalmologist.

“Some patients have early visual symptoms, others will seek care with an internist, dentist, psychiatrist or neurologist,” Dr. Friedman said. “The symptoms may be vague, and a high index of suspicion is needed.”

She said visual symptoms include amaurosis fugax, diplopia and sudden visual loss from ischemic optic neuropathy. Other symptoms include headaches, jaw claudication, scalp tenderness or necrosis, fever, weight loss, myalgias and arthralgias, malaise and night sweats, she said.

“Typical presentation would be a 75-year-old woman who starts having worsening of her arthritis: aches and pains every place,” Dr. Winterkorn said. “When she chews, her jaw gets tired. She can’t put her shirt on over her head because her scalp is so sensitive. She can’t comb her hair because her scalp hurts so much when she touches it. She has terrible headaches and then starts to have obscurations of her vision.”

Unfortunately, Dr. Winterkorn said, if the condition is not treated immediately with steroids, the patient can lose vision quickly.

“If it’s not recognized and treated immediately it can turn a healthy 75-year-old woman who plays tennis and goes to the theater into a blind person who needs to be taken care of,” Dr. Winterkorn said. “I’ve seen them lose vision in one eye and 5 minutes later lose vision in the other eye.”

Dr. Friedman said the ophthalmologist should start corticosteroids immediately and then check the sedimentation rate, C-reactive protein, complete blood cell count with platelet count and arrange for a temporal artery biopsy to be performed within a week.

“If you have a patient complaining of aches and pains, think temporal arteritis. It’s sufficiently common, especially in 80-year-olds. It gets more and more common as you get older,” Dr. Winterkorn said.

“You have to fish out the history by asking, ‘Does your jaw get tired when you chew? Do you have scalp sensitivity? Do you have headache?’ And if there’s any inkling that they have temporal arteritis, they should be given prednisone immediately and ask questions later because they can lose vision from anterior ischemic optic neuropathy at any time.”

Idiopathic intracranial hypertension

Idiopathic intracranial hypertension is primarily a disorder that affects overweight women of childbearing age, although there are atypical cases.

Dr. Friedman said the initial symptoms include headache, short but frequent transient obscurations of vision unilaterally or bilaterally, pulsatile tinnitus, diplopia and photophobia. Other neurologic symptoms include neck and back pain, radicular pain, ataxia and facial palsy.

An exam may show visual acuity loss, visual field defect, such as an enlarged blind spot, generalized constriction, inferonasal loss or central scotoma, and relative afferent pupillary defect if there is an asymmetrical optic neuropathy, Dr. Friedman said. She added that papilledema is the hallmark of this disorder, although it may be subtle or asymmetrical.

“It produces headache and swelling of the optic nerves because of the pressure inside of the skull,” Dr. Rizzo said. “The swelling of the optic nerves, when it persists for months, can cause a slow and permanent visual loss.”

Neuroimaging and lumbar puncture should be done immediately, Dr. Friedman said, and further treatment may include diuretics, headache treatments, or surgical procedures such as optic nerve sheath fenestration or shunt.

Dr. Rizzo said even after referring a patient to a neurologist, the ophthalmologist must continue observation.

“The patient needs to have a regular examination of the visual field and regular examination of the optic nerve because complications, progression of visual loss could occur and the ophthalmologist is well-suited to detect that and then to communicate the results of the exam to the neurologist, which may result in a change in medicine or perhaps even recommendation of surgery if visual loss is progressing,” he said.

Dr. Friedman warned that ophthalmologists must also be aware that cerebral venous sinus thrombosis can have an identical presentation to idiopathic intracranial hypertension.

“Consider that in a women taking oral contraceptives, smokers and women in the peripartum period,” she said. “There may be an underlying coagulation disorder.”

For more information:

• Deborah I. Friedman, MD, can be reached at Strong Health, 601 Elmwood Ave., Rochester, NY 14642; 585-273-3937; fax: 585-276-0292; e-mail: deborah_friedman@urmc.rochester.edu.
• Jacqueline S. Winterkorn, MD, PhD can be reached at Weill Cornell, Neuro-ophthalmology, Department of Neurology and Neuroscience, 520 E. 70th St., STARR-607, New York, NY 10021; 212-746-3072; fax: 860-355-5692; e-mail: jmswinte@aol.com.
• Joseph F. Rizzo III, MD, can be reached at Massachusettes Eye & Ear Infirmary, 243 Charles St., Boston, MA 02114; 617-573-3412; fax: 617-573-3851; e-mail: jrizzo@meei.harvard.edu.

• Katrina Altersitz is an OSN Staff Writer who covers all aspects of ophthalmology.