Neuro-Ophthalmology Newsletter November 2012

The use of injected filler material for facial cosmetic purposes is increasing in frequency. The use of such fillers, however, is not without risk for serious permanent injury. Park, et al (Am J Ophthalmol 2012; 154:653-672), did a retrospective review of 12 patients who suffered retinal artery occlusions following injections. Seven patients suffered ophthalmic artery occlusion, two central retinal artery occlusion, and three branch retinal artery occlusion. Seven patients received autologous fat, four hyaluronic acid, and one collagen. Seven received injections in the glabellar region, four in the nasolabial fold, and one in both. Patients receiving autologous fat had the poorest visual outcomes. All patients who suffered ophthalmic artery occlusions had ocular pain and no recovery of vision. Two patients with ophthalmic artery occlusion and two with central retinal artery occlusion also suffered strokes. One patient with ophthalmic artery occlusion developed phthisis. The authors emphasized the importance of keeping this risk in mind when performing these procedures. They also warned physicians to perform careful ocular exams and consider MRI of the brain in patients who report eye pain following injection.


Infliximab is known to cause an anterior form of optic neuritis in a small number of patients. The etiology of this complication has not been clearly established but recent research may indicate a culprit. Sokumbi, et al (Mayo Clin Proc 2012; 87:739-745), performed a retrospective review of patients treated with anti-TNF-alpha agents for inflammatory disease who developed systemic vasculitis. Eight patients were identified with a mean age of 48.5 years. 75% were women with a mixture of rheumatoid arthritis, Crohn’s disease, and ulcerative colitis. Five receive Infliximab, two Etanercept, and one Adalimumab. The mean duration from initial treatment to onset of vasculitis was 34.5 months. Five patients had predominantly skin involvement with palpable purpura. Four patients had peripheral nervous system damage and one renal damage. Seven patients improved with discontinuation of the anti-TNF-alpha agent and adjuvant Prednisone therapy. No patients were re-challenged and none had a recurrence of vasculitis. The results of this study would circumstantially suggest vasculitis as the cause of anterior optic nerve damage in patients on anti-TNF-alpha medications.


Methanol optic neuropathy is much less common than it used to be but will still appear on occasion. Pakravan and Sanjari (J Neuroophthalmol 2012; epublished July 2012) reported two patients with methanol optic nerve toxicity treated with 10,000 IU of intravenous erythropoietin twice daily for three days, 500 mg methylprednisolone intravenously twice a day for five days followed by a Prednisone tail for two weeks at 1 mg/kg per day, and intense vitamin therapy for one month. Both patients studied presented initially with no light perception vision in both eyes and mild optic disc swelling in both eyes. In the first patient, visual acuity returned to 20/20 in both eyes within three days. In the second patient, final visual acuity was counting fingers at six feet in the right eye and 20/30 in the left achieved at three weeks. The authors concluded that erythropoietin therapy should be considered in a protocol for treating methanol-induced toxic optic neuropathy.



Neuro-Ophthalmology Newsletter

Volume IX, Number 11 – November 2012

Case Study


Patient IX11 is a 45-year-old man who woke up one week prior to exam with oblique, binocular diplopia. Six days ago he developed tingling in his fingers. The next day his arms felt heavy. An LP at that point was normal except for an elevated protein. Four days ago his balance was poor and he noted tingling in his toes. He later developed heaviness in his legs. He reported that he was avidly into physical fitness and that he was clearly developing weakness in his arms and legs. He had taken Levofloxacin recently for an infection but had been off for a while. He had no trouble swallowing.


At exam, his visual acuity was 20/20 in his right eye and 20/25 in his left eye. He had no relative afferent pupillary defect. Confrontation testing was full in both eyes. He had an 8 diopter esotropia in neutral position that increased to 12 diopters in right gaze and decreased to trace in left gaze. He had a 4 diopter left hypertropia in neutral position that increased to 6 diopters in down gaze and 12 diopters in right head tilt. The hypertropia changed to a 6 diopter right hypertropia in left head tilt. His eyelid position was normal. He showed no evidence of fatiguing. His optic discs were pink and flat.


What is your next step with this patient and why?

Discussion of Case IX10 from October 2012 Newsletter


This patient has three concurrent issues all related to an infarct in the brainstem. Considering the history of proximity to his myocardial infarct, he most likely suffered an embolus.


The first issue is a right internuclear ophthalmoplegia. This complex results from an injury to one or both medial longitudinal fascicules. In the acute stage, patients will show a decrease or absence of adduction of the ipsilateral eye, a delayed adduction saccade in the ipsilateral eye, and abduction nystagmus of the opposing eye. Patients may have absent convergence of the ipsilateral eye but may have normal convergence. If convergence is normal, a third cranial nerve injury is impossible. Within a few weeks, the process starts to resolve and diplopia generally disappears as patients enter the subacute stage. At this point, alternate cover testing does not reveal much if any residual exotropia. The delay in adduction saccades persists, however, and the saccade may take a sweep superiorly or inferiorly rather than taking a straight course toward the nose. Abduction nystagmus persists. In the chronic stage, all that will likely remain will be a slight delay in adduction saccades. In this age group, an infarct is the probable culprit. In patients below the age of 40, think demyelinating disease (bilateral INO is a hallmark of multiple sclerosis).


We now know that the lesion is somewhere along the right medial longitudinal fasciculus. The next tip is the hypertropia. The constancy of the deviation in different directions of gaze eliminates a cranial nerve palsy with the default diagnosis, therefore, of a skew deviation. Skew deviation results from damage to the vertical gaze pathways just dorsal to the medial longitudinal fasciculus. The vertical misalignment resolves in the majority of patients within only a few weeks. Prisms often relieve symptoms during the recovery phase. INO and skew often occur together.


We’re almost there, now. The final piece of the puzzle is the up-beating nystagmus present in neutral position. This eye movement disorder does not occur as a rule with INO or skew deviation but is localizing. The preponderance of patients with acquired up-beating nystagmus have a lesion a the ponto-mesencephalic junction. Be aware that many sedative or anti-seizure medications may result in up-beating nystagmus as a side effect.


Putting all the points together, the lesion involves the right medial longitudinal fasciculus (INO) and adjacent right vertical gaze pathway (skew). It sits at the ponto-mesencephalic junction (up-beating nystagmus) and involves neural input from the occipital lobes to the right third cranial nerve nucleus (absent convergence). Sometimes you don’t need an MRI to find the problem!

Neuro-Ophthalmology Newsletter October 2012

Optic nerve gliomas in children present a significant problem in management. The best form of treatment and the appropriate modalities for measuring the effectiveness of therapy are still under review. Kelly, et al (Ophthalmology 2012; 119:1231-1237, monitored visual acuity, tumor volume, and visual evoked potentials over time in children with optic nerve gliomas. The authors identified 21 patients between the ages of 0.7 and 9 years who had undergone treatment. 18 patients had undergone chemotherapy, 3 radiation therapy. All patients had serial MRI, visual acuity measurements in logMAR units, and pattern VEP. Patients were followed for an average of 9 years. The authors had available estimated tumor volumes before and after treatment in 15 patients. 81% had reduced visual acuity and optic nerve pallor prior to treatment. All had abnormal visual evoked potentials in one or both eyes. After initial treatment, there was a 53% decrease in tumor volume while 20% continued to progress. Visual acuity tended to stabilize visual acuity for four to five years. Patients often held constant visual acuities even without shrinkage of the tumor. 62% needed addition treatment with chemotherapy or radiation when the tumor continued to grow or growth recurred. The final visual acuity was stable or improved in 33% but overall declined 0.4 logMAR units on average. Initial visual acuity and tumor volume estimates were the best factors for predicting final visual outcome. The authors concluded that chemotherapy was successful in stabilizing visual function for 5 years on average. VEP was the most sensitive parameter for estimating the amount of visual pathway damage. Unfortunately, the final visual outcome tended to be limited by the amount of damage that preceded the initiation of therapy.


Ophthalmic or retinal artery occlusion may occur with the injection of cosmetic filler materials to the face. Park, et al (Am J Ophthalmol 2012; 154:653-662), performed a retrospective analysis on twelve consecutive patients with this complication.  Seven had ophthalmic artery occlusion, two central retinal artery occlusion, and three branch retinal artery occlusion. Parameters examined included the type of filler, injection sites, visual acuity, fluorescein angiography, OCT, and any associated ocular and systemic symptoms. Injected materials included autologous fat, hyaluronic acid, and collagen. Injection sites were the glabellar region in seven patients, nasolabial fold in four patients, and both sites in one patient. Fat injection led to the greatest drops in final visual acuity. All patients with ophthalmic artery occlusion suffered pain and did not regain any vision. Patients with ophthalmic artery occlusion also showed significant damage to the choroid. Two patients with central retinal artery occlusion and two patients with ophthalmic artery occlusion suffered cerebral infarction as well. Ophthalmic artery occlusion led to phthisis in one patient. The authors wished to alert practitioners regarding the risk for this severe complication and recommended that any patient with ocular pain after injection should be evaluated immediately.



Neuro-Ophthalmology Newsletter

Volume IX, Number 10 – October 2012

Case Study


Patient IX10 is a 65-year-old man who suffered a heart attack six days before this examination. He had some periods of significant hypotension. For the past two days he has noted horizontal, constant, binocular double vision worse looking to his left. His vision seems blurred. His medical history was significant for sinus congestion, myocardial infarcts in 1996 and recently, shoulder surgery, neck surgery, and cutaneous actinic keratosis. His ocular history was significant for the problems listed above. A social history indicated cessation of smoking cigarettes in 2010 and social alcohol consumption. His family history was non-contributory. Current medications included Plavix, Aspirin, Zocor, Allegra, Zyrtec, and over the counter dietary supplements. He reported allergies to Morphine and ACE inhibitors.


At exam, his visual acuity with correction was 20/30 in each eye. He had normal pupillary function without a relative afferent pupillary defect. Confrontation testing was full in both eyes. There was a normal appearance of his eyelids, orbits, lacrimal glands, lacrimal drainage system, and regional lymph nodes. His right eye showed a slight decrease in adduction and delayed adduction saccades. His left eye showed abduction nystagmus. He demonstrated a low amplitude, high frequency up-beating nystagmus in neutral position. He had a 5 diopter right hypertropia in neutral position, right gaze, and down gaze that increased slightly in left gaze and up gaze. His right eye did not converge to an approaching target. His anterior segments were healthy. His optic discs were pink and flat. He was alert and oriented time four.


What are his diagnoses and where is the lesion?

Discussion of Case IX9 from September 2012 Newsletter


This patient has a right junctional scotoma. All practitioners should recognize this pattern since it pinpoints the site of the problem. A junctional scotoma consists of a central defect in one eye with a superior temporal defect in the opposite eye. This pattern is noted in patients with lesions at the anterior aspect of the optic chiasm. The usual culprits are at fault, including pituitary adenomas, meningiomas, and craniopharyngiomas.


A couple of hypotheses have been presented to explain the combination of visual changes seen. Traditionally, the central scotoma in one eye, in this case the right eye, results from compression of the optic nerve just anterior to the optic chiasm. Anatomical studies have shown the presence of Wilbrand’s knee related to optic nerve fibers crossing in the chiasm from the contralateral eye. The optic nerve fibers from the inferior nasal retina of the contralateral eye corresponding to the superior temporal field in that eye (in this case the left eye) swing forward for a short distance into the optic nerve as they cross. As a result, these crossing fibers are affected simultaneously with the optic nerve fibers when the nerve is compressed.


Dr. Jonathan Horton, however, performed studies on the visual pathways of primates that indicated that Wilbrand’s knee was an artifact of tissue preparation and really does not exist. His suspicions were based on the fact that patients who have surgical resection of the optic nerve just anterior to the optic chiasm did not develop junctional scotomas. Her used MRIs from patients with junctional scotomas and demonstrated that they had both optic nerve and anterior chiasmal compression.


Horton’s arguments certainly are convincing. No matter which hypothesis you believe, however, this defect always locates to the junction of an optic nerve and the anterior optic chiasm.

Neuro-Ophthalmology Newsletter September 2012

Non-arteritic anterior ischemic optic neuropathy can be frustrating to physician and patient alike since no treatment has been shown scientifically to affect the outcome of the disease. Some physicians will treat these patients with corticosteroids in the hope that they may have an impact on recovery. Rebolleda, et al (Graefes Arch Clin Exp Ophthalmol 2012; e-published March 24, 2012), looked at ten eyes in ten patients with AION and treated them with Prednisone 80 mg initially with taper. The results in these patients were compared with 27 randomly selected historical controls. Patients were examined six to eight weeks following onset and six months following onset. The authors found no difference between the two groups in improvement of visual acuity, visual field median deviation, or visual field pattern standard deviation. Nerve fiber layer loss was similar in both groups. Three patients on corticosteroids suffered complications of treatment and one had to discontinue therapy. Two of the patients on Prednisone taper developed AION in the fellow eye within three months of losing vision in the first eye. No complications were noted in the control group. The authors terminated the study early due to the increased risk of complications in the treatment arm. The authors concluded that corticosteroids provided no benefit in visual outcome in AION and that it was associated with a significant risk of serious complications.


Adult strabismus patients have a lower quality of life than the general population. Most patients receive appropriate surgical treatment but others may not be medically stable enough to undergo surgery. Hancox, et al (Br J Ophthalmol 2012; 96:838-840), noted that many of these patients receive Botox injections as therapy. The authors retrospectively identified 65 patients who had undergone over 25 Botox injections for long term management were studied using quality of life surveys. 48 patients completed the questionnaire. Analysis of the surveys indicated that patients receiving Botox injections had a similar quality of life score to patients without strabismus and statistically better quality of life compared to patients with strabismus. The authors concluded that Botox was a good choice for therapy in patients who are not candidates for surgrery.


Patients with significant carotid artery stenosis are at an increased risk for stroke. Physicians have been looking for screening techniques to noninvasively identify these individuals.  Knecht, et al (Ophthalmology 2012; 119:1244-1249), studied ocular pulse amplitude, the difference in intraocular pressure between diastolic and systolic heartbeat phase readings, as a possible alternative. 67 consecutive patients in for carotid Doppler studies were included in the cohort. The authors compared ocular pulse amplitude in patients with less than 50% occlusion with those with more than 70% occlusion. The authors found a significantly difference in the ocular pulse amplitude in patients with mild stenosis compared to those with severe stenosis. The authors concluded that ocular pulse amplitude shows promise as a safe and economical screening tool for identifying patients at risk for severe carotid artery stenosis.



Neuro-Ophthalmology Newsletter

Volume IX, Number 9 – September 2012

Case Study


Patient IX9 is a 62-year-old woman who noted progressive loss of vision in her right eye. She now could barely see at all out of her right eye. She did not note any problems with her left eye. She had some mild headaches that were frequent. 


Her medical history did not indicate any factors that might explain her symptoms.


Her ocular history is significant for the problems listed above.


Her review of systems is otherwise noncontributory.


Her family history was unrevealing.


Current medications include over the counter headache medication.


Her social history is significant for no tobacco or alcohol consumption.


She reported no allergies to medications.


On examination, her visual acuity at distance, with correction, was counting fingers only in the right eye and 20/20 in the left eye.  Both pupils measured 5 mm in the dark and 3 mm in the light.  A 4+ right relative afferent pupillary defect was noted.  Humphrey perimetry demonstrated a dense central defect in her right eye and a superior temporal defect outside 10 degrees in her left eye.  Examination of her extraocular motility showed normal motility and alignment.  Slit lamp examination revealed a quiet conjunctiva, clear corneas, deep anterior chambers, and normal irises in both eyes.  Both eyes contained posterior chamber intraocular lenses.  Intraocular pressure measurements were 15 mmHg on her right and 16 mmHg on her left.   Dilation was deferred, again, because of the sleep test.  Her right optic disc showed mild pallor. Her left optic disc was pink and flat.  There was a normal appearance of the macula, retinal blood vessels, and vitreous in both eyes.  A mental status examination revealed her to be alert and oriented x4.


Where is the lesion and why did the patient have this particular pattern of visual field changes?

Discussion of Case IX8 from Augusts 2012 Newsletter


This patient has diplopia. Whenever you are faced with double vision, a thorough history may point you in the right direction. First of all, she was quite certain that the diplopia was isolated to her right eye. I corroborated this fact when she reported diplopia during visual acuity measurement of her right eye. Since a pinhole alleviated the diplopia and improved her visual acuity to 20/25 from 20/40 in her right eye, her problem had to do with how her eye was focusing.


Determining the cause of the diplopia, however, does nothing to explain her motility issues. Although she had decreased ability to abduct, elevate, and adduct both eyes symmetrically, she was well aligned in all directions of gaze. The normal sleep test ruled out the possibility of myasthenia gravis. The pattern did not fit a lesion in the brainstem or cranial nerve. Whenever this particular situation arises, one serious consideration must be chronic progressive ophthalmoplegia (CPEO).


CPEO usually presents initially with ptosis. Abnormalities of extraocular movements generally kick in months to years later. The condition results from a mutation in mitochondrial DNA. Patients often have some degree of systemic weakness as well.


Early onset CPEO raises concerns about Kearns-Sayre syndrome (KSS). In addition to the usual changes in muscular strength seen with CPEO, these individuals also have a pigmentary retinopathy and cardiac conduction abnormalities, elevated CSF protein, and cerebellar degenerative signs. Unusual components include mental retardation, Babinski sign, hearing loss, seizures, short stature, delayed puberty, and endocrine disorders.


The traditional means of diagnosing CPEO and KSS is muscle biopsy to look for characteristic nerve fiber changes and abnormal mitochondria. Polymerase chain reaction testing is available as well to look for mitochondrial DNA mutations.


For the record, a muscle biopsy confirmed the diagnosis of CPEO.

Two Little Rock Eye Clinic Physicians Recognized At The “Night For Sight” Dinner, Benefitting Legend Treks, On November 17th 2012.

On November 17th, 2012, two Little Rock Eye Clinic Physicians were recognized for charitable mission work with the organization Legend Treks.  The “Night For Sight” benefit dinner took place at the Embassy Suites in Little Rock, Arkansas.  Both Drs Charles Henry and Dan Hennessey received awards from the organization for their service on mission trips during 2012. 

Legend Treks is a non-profit organization that serves communities abroad in Peru, Morocco and Mali. 

The Little Rock Eye Clinic wants to congratulate both Dr. Henry and Dr. Hennessey on their recognition and their contribution to the international community!