Little Rock Eye Clinic

Neuro-Opthalmology Newsletter- July 2010
Neuro-Ophthalmology Newsletter Andrew Lawton, M.D. July 2010 - Volume VII, Number 7 Sometimes you have to be careful what you publish. Rougier (J French Ophthalmol 2010; 33:424-429) indicate in his review article that the proper diagnosis of optic disc edema and papilledema requires the use of direct examination of the optic disc, fluorescein angiography, and OCT. The author clearly comes from the environment of socialized medicine since the added expense and risk of FA and OCT in the US system would be severely frowned upon. The author also stated that all patients with papilledema receive and MRI and MRV. One might make a better argument for the addition of the MRV but, I can tell you from experience, ordering an MRV on every patient with papilledema would result in a hit on utilization review. The danger here would be heightened if the article had been printed a widely disseminated journal in the US. A precedent for attorneys to pull out in a malpractice case is unlikely to be set by a journal few people here read, but a similar article in the AJO or Archives would institute a legal field day. There is a certain degree of responsibility in publication and, without adequate support by evidence-based studies, an article like this is a time bomb. Interferon alpha has been shown to produce retinal problems, but a new article opens up a whole new area of concern. Bert, et al (J Neuro-Ophthalmol 2010; 20:117-122) presented a case report of a patient under treatment with peginterferon-alpha 2 for hepatitis C who simultaneously developed bilateral anterior ischemic optic neuropathy and a peripheral neuropathy. Following discontinuation of the medication, his peripheral neuropathy and the vision in one eye improved while the vision in the opposite eye worsened. The authors identified 23 more patients who suffered AION after institution of interferon-alpha therapy. The process occurred one to 40 weeks after institution of therapy. Of 21 eyes with follow up, eight eyes had improvement of vision, one worsened, and the remainder remained unchanged after cessation of treatment. The authors concluded that patients should be assessed for the know risk factors for AION and the risk-benefit ratio of interferon-alpha determined for the individual patient before starting treatment. Tinley, et al (Strabismus 2010; 18:41-47), looked at treatment options for strabismus in chronic progressive external ophthalmoplegia. A total of 28 patients were reviewed who received treatment for diplopia or cosmetic issues. Potential therapeutic modalities included surgery, prisms, and botulinum injections. The majority of patients had large exotropias often complicated by vertical deviations. The most successful treatment for the exotropias was a maximal bilateral lateral rectus recession with medial rectus resections on adjustable sutures. Unless the lateral rectus recessions were maximal, patients ended up undercorrected. The big problem was the progressive nature of CPEO since patients with initially good results redeveloped exotropia over time. The authors also found that botulinum injections could be of significant benefits in patients post strabismus surgery with recurrent exotropia. . Neuro-Ophthalmology Newsletter Volume VII, Number 7 - July 2010 Case Study Patient VII7 is a 35-year-old woman with a chief complaint of the sudden onset of a cold, clammy sensation and a strange smell. She started vomiting. She found herself falling to her right and unable to get up without assistance. She noted vertical, binocular double vision. Her vision was jumping and seemed to move in a circular direction. She noted vertigo. If she moves to quickly, the environment rotates and the image jerking worsens. Her right eye looks smaller than her left and the right pupil is smaller than the left. A medical history was significant for prior good health. A review of systems revealed no additional contributory factors in the dermatological, neurological, respiratory, cardiovascular, genitourinary, alimentary, and hematologic systems. The ocular history was significant for the problems listed above. The family history indicated cataracts, cancer, and diabetes. The social history showed bi tobacco or alcohol consumption. Current medications included Meclizine, Scopolamine, Aspirin, Plavix, and vitamins. Allergies were reported to no medications. At examination, visual acuity at distance without correction was 20/30 (+) in the right eye and 20/40 in the left eye. The right pupil measured 5 mm in the dark and 3 mm in the light. The left pupil measured 7 mm in the dark and 3 mm in the light. Her right pupil dilated more slowly than her left. No relative afferent pupillary defect was noted. Both eyes moved fully in all directions. She had a clockwise torsion /downbeating jerk nystagmus. She had an 18 diopter left hypertropia in neutral position that disappeared in the supine position. An external examination revealed a normal appearance of the eyelids, orbits, lacrimal glands, lacrimal drainage system and regional lymph nodes. Confrontation visual fields showed no defects. A slit lamp examination demonstrated a normal appearance of the eyelids. The conjunctiva was quiet in both eyes. The corneal epithelium, endothelium, tear film, and stroma had a normal appearance. The anterior chambers were deep and quiet. The irises appeared normal. Both lenses demonstrated clear anterior and posterior capsules, nuclei, and stroma. Intraocular pressures by applanation were 18 mm Hg on the right and 21 mm Hg on the left at 10:25 AM. Following dilation, examination of the posterior segments revealed pink, flat optic discs with cup-to-disc ratios of 0.2 on the right and 0.2 on the left. The nerve fiber layers, maculae, retinal blood vessels, vitreous, and peripheral retinas were intact in both eyes. The patient was oriented to person, place, affect, and date. Discussion of Case VII6 from June 2010 Newsletter Patient VII7 underwent a series of tests prompted by the combination of bilateral optic neuropathy and pigmentary changes in his retinas. He turned out to have tertiary syphilis. After appropriate antibiotic therapy, the vision in his right eye improved to 20/25 (-3) and in his left eye to 20/70. Tertiary syphilis is not identified as often as a cause of optic neuropathy or uveitis as it used to be. Any patient with these conditions and without a clear etiology, however, should have blood testing for luetic serology. An FTA-abs or MHA-TP should be performed rather than just a VDRL since patients with tertiary disease may be so far out that their VDRL becomes negative. In a patient such as this with positive FTA-abs and optic neuropathy, a lumbar puncture is indicated to confirm the presence of positive serology in the cerebrospinal fluid. If the VDRL is positive, it can be used to assess treatment success since the test will decrease if the disease is adequately treated. The standard therapeutic regimen for patients with positive CSF serology and signs of central nervous system involvement is aqueous crystalline penicillin G 2–4 million U intravenously every 4 hours for 10 to 14 days, followed by benzathine penicillin 2.4 million U intramuscularly weekly for 3 weeks (1.2 million U in each buttock). If the CSF is negative and the patient has purely ocular involvement such as uveitis, some authors recommend benzathine penicillin 2.4 million U intramuscularly weekly for 3 weeks with other authors recommending the full intravenous/intramuscular treatment plan if the retina or optic nerve is involved with normal CNS serology. If the patient is allergic to penicillin, consultation with an infectious disease specialist is strongly recommended to assure complete eradication of the disease. Keep in mind that patients with one sexually transmitted disease may have others as well. Testing for HIV infection is prudent. Chlamydia is another consideration

Neuro-Opthalmology Newsletter- June 2010
Neuro-Ophthalmology Newsletter Andrew Lawton, M.D. June 2010 - Volume VII, Number 6 Botulinum toxin injections to the extraocular muscles are used at times particularly in cases of cranial nerve palsies. Santos, et al (J Pediatr Ophthalmol Strabismus 2010; epublished April 2010), measured the strength of isometric contraction of medial and lateral rectus muscles in esotropic and exotropic individuals one month after Botulinum injection. Volunteers without strabismus were also injected and used as controls. The authors determined that the injections altered the strength ratio between agonist and antagonist muscles resulting in a shift of the point of equilibrium nasally in esotropic patients and temporally in exotropic patients. The change resulted in patients having a move of the deviated eye towards primary position. Both groups of patients achieved about a 10 diopter alteration in their deviations. The authors concluded that, with the results above, Botulinum injections would be best suited to patients with smaller deviations. Botulinum injections can be beneficial in patients with both benign essential blepharospasm and hemifacial spasm. Cannon, et al (Clin Experiment Ophthalmol 2010; epublished April 2010), prospectively studied patients over the course of six months to see if there was a difference in response to treatment between the two conditions. The authors had patients fill out a questionnaire to assess results. The authors tracked patient demographics, cumulative dosing of Botulinum, duration of Botulinum effects, and overall patient satisfaction. A total of 30 patients had blepharospasm and 34 hemifacial spasm. The authors found that patients with hemifacial spasm required a lower mean dose of Botulinum than with blepharospasm. The hemifacial spasm patients also reported a longer duration of action of the medication less frequent treatments required. 90% of patients in both groups were satisfied with the results of their injections. Three blepharospasm patients required surgical treatment. The authors concluded that the management of blepharospasm is more complex than that of hemifacial spasm. Corticosteroid therapy for orbital Graves’ disease has its supporters and detractors Of course, physicians have more than one delivery technique to get the drug where they want it. Alkawas, et al (Clin Experiment Ophthalmol 2010, epublished May 2010), wanted to compare the effectiveness of orbital corticosteroid injections versus oral corticosteroid therapy. 29 patients were enrolled in the study with 15 patients randomized to oral Prednisolone and 14 patients to a peribulbar Triamcinolone injection. 12 patients in each group remained at the end of the study. Both groups had improvement in ocular symptoms and in orbital inflammatory markers. Most patients had improvement of eye movements and proptosis. At six months, no patients los visual acuity. Patients on the oral regimen tended to gain weight, have an elevation of blood sugar and blood pressure, and develop gastritis. These complications were not apparent in the peribulbar injection cohort. None of the patients who received Triamcinolone injections reported any local side effects. The authors concluded that both modalities were equally effective in treating Graves’ orbital disease. The found, however, a much lower incidence of adverse reactions with peribulbar Triamcinolone injections and recommended the injections over oral medication. Neuro-Ophthalmology Newsletter Volume VII, Number 6 - June 2010 Case Study Patient VII6 is a 58-year-old man with a chief complaint of suddenly losing vision in both eyes in mid-January 2008. He associated the visual change with shortness of breath. He had no pain or headaches. His vision went dark in both eyes and then started to recover after one to two hours. The vision in his left eye is now significantly worse than in his left eye. A medical history was significant for heart valve surgery in January 2008 and a gunshot wound in 1971. A review of systems revealed no additional contributory factors in the dermatological, neurological, respiratory, cardiovascular, genitourinary, alimentary, and hematologic systems. The ocular history was significant for the problems listed above. The family history indicated cataracts. The social history showed smoking a pack of cigarettes per day and no alcohol. Current medications included Coumadin, Darvocet, and Baclofen. Allergies were reported to no medications. At examination, visual acuity at distance without correction was 20/400 in the right eye and counting fingers only in the left eye. The right pupil measured 5 mm in the dark and 3 mm in the light. The left pupil measured 5 mm in the dark and 3 mm in the light. Both pupils constricted relatively sluggishly to a light in either eye. A 2+ left relative afferent pupillary defect was noted. Both eyes moved fully in all directions. Pursuit and saccades were intact. He showed a 4 diopter exophoria. An external examination revealed a normal appearance of the eyelids, orbits, lacrimal glands, lacrimal drainage system and regional lymph nodes. Humphrey 24-2 threshold visual fields showed diffuse central depression in both eyes; the tests were unreliable. A slit lamp examination demonstrated a normal appearance of the eyelids. The conjunctiva was quiet in both eyes. The corneal epithelium, endothelium, tear film, and stroma had a normal appearance. The anterior chambers were deep and quiet. The irises appeared normal. Both lenses demonstrated clear anterior and posterior capsules, nuclei, and stroma. Intraocular pressures by applanation were 14 mm Hg on the right and 15 mm Hg on the left at 10 AM. Following dilation with Tropicamide 1%, examination of the posterior segments revealed relatively pink, optic discs with cup-to-disc ratios of 0.2 on the right and 0.2 on the left. The left optic disc had mild edema. His nerve fiber layers looked good. Both retinas showed areas of pigmentary darkening in a "salt-and-pepper" pattern. The patient was oriented to person, place, affect, and date. Discussion of Case VII5 from May 2010 Newsletter Patient VII6 presented with essentially absent extraocular motility of both eyes in all directions of sudden onset. He also had some ptosis. A presentation like this represents a potentially life-threatening medical emergency and has to be taken very seriously. Once one goes through the differential diagnosis, the reason for urgency becomes apparent. Let’s look at congenital motility problems first. Patients with bilateral Duane’s syndrome could look sort of like this but do better vertically. Mobius syndrome as well produces motility problems but not in all directions. Besides, our patient was healthy before and had a sudden onset of symptoms. Now we can move on to infectious causes. One potential etiology would be botulism. In this condition, paresis shows up in the facial and extraocular muscles first in this condition. If the patient also has difficulty swallowing and chewing, things are starting to get ominous. A second, post-viral process that could appear like this would be Guillain-Barre syndrome. Usually this starts distally and proceeds proximally. In the C. Miller Fisher variant, however, the cranial nerves are affected first. These patients are at imminent risk because the paralysis may spread to involve the phrenic nerves resulting in respiratory arrest. Autoimmune processes are in the mix also. The primary concern in this patient would be a fairly fulminant myasthenia gravis. A patient with onset this rapid may be at risk for severe respiratory symptoms or loss of the ability to swallow. Without the complete history, I certainly would have included chronic progressive external ophthalmoplegia in the differential. This condition, however, would never have such an abrupt onset. The same goes for myotonic dystrophy. My immediate concerns centered on botulism and Guillain-Barre syndrome. He showed no sign of fatiguing or of recovery with rest. With such dangerous conditions a possibility, I sent him off to the ER for an urgent evaluation. .

Neuro-Opthalmology Newsletter- July 2010

Neuro-Opthalmology Newsletter- June 2010