Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature.

OBJECTIVE: The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment options. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nine patients were identified with AMPPE and CNS involvement in addition to 22 patients reviewed in the literature. MAIN OUTCOME MEASURES: A review of nine patients with AMPPE and CNS involvement was performed. Charts were reviewed for age, gender, preceding viral prodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment. RESULTS: Thirty-one patients (nine new patients) were diagnosed with AMPPE and various degrees of CNS involvement. Ages ranged from 8 to 54 years, with an average of 27 years. Twenty-one males (68%) and 10 females (32%) were identified. Eleven patients (35%) had antecedent viral illnesses. Visual acuity was variable and ranged from 20/20 to count fingers. The spectrum of CNS findings ranged from headaches to sagittal sinus thrombosis. CONCLUSIONS: Acute multifocal placoid pigment epitheliopathy can be associated with CNS abnormalities and permanent visual deficits. Neuroimaging, lumbar puncture, and cerebral angiography analysis provide useful diagnostic tools when CNS involvement is suspected. Intravenous corticosteroids and collaboration with neurovascular colleagues should be considered in these situations. In cases complicated by CNS arteritis, immunosuppressive agents can be a beneficial adjunct to corticosteroids.

Levodopa may improve vision loss in recent-onset, nonarteritic anterior ischemic optic neuropathy.

OBJECTIVE: To study the effect of levodopa in improving visual function in patients treated within 45 days of onset of nonarteritic anterior ischemic optic neuropathy (NAION). DESIGN: Nonrandomized, retrospective, comparative trial. PARTICIPANTS: The study involved 37 patients with NAION of less than 45 days duration. METHODS: Eighteen patients who had been treated with levodopa were assigned to the case group, and 19 untreated patients were assigned to the control group. Snellen visual acuity converted to logMAR and mean deviation on Humphrey automated perimetry (Program 24-2, Humphrey Instruments, San Leardro, CA) were evaluated at the initial and 6-month visits. INTERVENTION: The 18 patients in the case group were administered a capsule of 100 mg levodopa/25 mg carbidopa (Sinemet 25-100) three times daily for 3 weeks. MAIN OUTCOME MEASURES: The primary outcome measures were changes in visual acuity and visual field at 6 months from baseline. Improvement in visual acuity was defined as a difference of -0.3 logMAR or less between the 6-month and initial visual acuities, whereas worsened visual acuity was a difference of +0.3 logMAR or more. Each 0.3 LogMar represented a doubling of the visual angle, i.e., a change by three lines on the eye chart. Improvement in visual field was defined as a difference in mean deviation of +3.0 dB or more between the 6-month and initial visual field tests, whereas worsened visual field was a difference in mean deviation of -3.0 dB or less. RESULTS: The proportions of patients with worsened, unchanged, and improved visual acuity at 6 months were compared for the levodopa and control groups. There was a significant difference (P = 0.012) between the groups. Examination of the proportions showed that a higher proportion of patients who received levodopa had improved visual acuity with a corresponding lower proportion having worsened visual acuity as compared with the control patients. Ten of 13 patients (76.9%) in the levodopa group with 20/40 visual acuity or worse at baseline had improved visual acuity at 6 months, and none of the 18 patients had worsened visual acuity. In contrast, 3 of 10 control patients (30%) with 20/40 visual acuity or worse at baseline had improved visual acuity at 6 months, and 3 of 19 control patients (16.3%) had worsened visual acuity. The proportions of patients with worsened, unchanged, and improved visual fields at 6 months were compared for the levodopa and control groups. There was no significant difference between the groups (P = 0.25). CONCLUSIONS: Patients treated with levodopa within 45 days of onset of NAION were more likely to experience improvement and less likely to have worsened visual acuity than untreated patients. Levodopa appears to be beneficial in the treatment of recent-onset NAION.

Aspirin use and the prevention of acute ischemic cranial nerve palsy.

PURPOSE: To assess the relationship of aspirin use and ischemic cranial nerve palsies among patients with diabetes mellitus and hypertension. METHODS: This retrospective case-control study involved 100 patients with ischemic cranial nerve palsies in association with diabetes, hypertension, or both (palsy cases) and 163 age-matched and sex-matched patients with diabetes, hypertension, or both but without ischemic cranial nerve palsies (nonpalsy control subjects). Comparisons were made with respect to duration of diabetes, dose and duration of aspirin use, dose and duration of tobacco use, and presence of cardiac or cerebrovascular disease. RESULTS: There were 20 oculomotor, 33 trochlear, 37 abducens, and 10 facial nerve palsy cases. The median duration of diabetes was 6 years for cases and 7 years for control subjects. There were 34 cases (34%) who had used aspirin for a mean duration of 5.5 years before the onset of the cranial nerve palsy and 49 control subjects (30.1%) who had used aspirin for a mean duration of 4.3 years. There were no significant differences between cases and control subjects for duration of diabetes (P =.94); aspirin use (P =.51), duration (P =.50), and dosage (P =.89); tobacco use (P =.73) and consumption (P =.45); and proportion of cardiac disease (P =.17). Cerebrovascular disease was significantly less common among palsy cases than nonpalsy control subjects (P<.001). There was no significant difference in the odds of a patient having cranial nerve palsy in the aspirin group compared with the nonaspirin group (odds ratio, 1.12; 95% confidence interval, 0.70-2.04). CONCLUSION: Aspirin use was not associated with a reduced rate of ischemic third, fourth, sixth, and seventh nerve palsies among patients with diabetes mellitus and hypertension. Aspirin appears to be ineffective in preventing ischemic third, fourth, sixth, and seventh cranial nerve palsies. Patients with ischemic cranial nerve palsy have a significantly lower rate of strokes and transient ischemic attacks than patients who have diabetes or hypertension but who do not have a history of cranial nerve palsy.

The role of weight loss and acetazolamide in the treatment of idiopathic intracranial hypertension (pseudotumor cerebri)

OBJECTIVE: To determine the weight loss associated with resolution of papilledema from idiopathic intracranial hypertension (IIH). DESIGN: A retrospective study. PARTICIPANTS: Fifteen consecutive female patients with IIH associated with obesity were studied. INTERVENTION: Patients underwent weight loss and treatment with acetazolamide during a 24-week period. MAIN OUTCOME MEASURES: The severity of papilledema was graded: absent (grade 0), mild (grade 1), moderate (grade 2), and marked (grade 3), based on a predetermined grading system ("gold standard") using stereoscopic photographs and the Frisén classification. RESULTS: The 15 patients, with mean age of 31.3+/-8.8 years, had a mean weight of 110.5+/-28.7 kg and mean body mass index of 40.7+/-13.0 kg/m2. Eleven (73.3%) patients had improved papilledema during the 24-week study period, of which 10 (66.7%) had complete resolution of papilledema within a median time of 8.5 weeks. An average of 3.3% weight loss (+/-0.5% standard error of the mean) was observed among patients having a one-grade change in papilledema. Weight loss of 6.2%+/-0.6% standard error of the mean was associated with a three-grade change in papilledema (i.e., complete resolution of marked papilledema). Nine of the ten patients with complete resolution of papilledema also took acetazolamide. However, none (26.7%) of the four patients without weight loss had improvement in papilledema despite similar treatment with acetazolamide. CONCLUSIONS: Approximately 6% weight loss was associated with resolution of marked papilledema in these authors' patients. The benefit of acetazolamide in IIH is questioned since weight loss, rather than acetazolamide, appeared to have been the catalyst for reducing the severity of papilledema.

Recurrent herpes labialis as a potential risk factor for nonarteritic anterior ischemic optic neuropathy.

This case-control study was undertaken to determine if recurrent herpes labialis posed an independent risk factor for the development of nonarteritic anterior ischemic optic neuropathy among individuals with small optic nerve cup:disk ratio. The study population was comprised of 43 nonarteritic anterior ischemic optic neuropathy cases and 30 consecutive control subjects with < or = 0.1 optic nerve cup:disk ratio. The two groups were compared with respect to the history of recurrent herpes labialis, cigarette smoking status, hypertension, diabetes mellitus, cardiovascular disease, and cerebrovascular disease. The nonarteritic anterior ischemic optic neuropathy cases were comparable to controls with respect to cigarette smoking status, hypertension, diabetes mellitus, cardiovascular disease, and cerebrovascular disease status. Nonarteritic anterior ischemic optic neuropathy cases were significantly more likely to have a history of recurrent herpes labialis than controls. Herpes simplex virus as identified by recurrent herpes labialis plays a role in the development of nonarteritic anterior ischemic optic neuropathy. However, a casual relationship cannot be established.

Effect of levodopa and carbidopa on recovery of visual function in patients with nonarteritic anterior ischemic optic neuropathy of longer than six months' duration.

PURPOSE: We conducted a pilot clinical trial to determine the efficacy of levodopa in promoting visual recovery in eyes with nonarteritic anterior ischemic optic neuropathy of greater than six months' duration. METHODS: This prospective, randomized, double-masked, placebo-controlled clinical trial involved 20 subjects with nonarteritic anterior ischemic optic neuropathy of 30 months' mean duration. Subjects were randomly assigned to receive either low-dose levodopa and carbidopa or a placebo for three weeks. At 12 weeks after the baseline visit, the levodopa group then was provided a higher, conventional dose of levodopa and carbidopa for three more weeks. Change in visual function was monitored at four, 12, 16, and 24 weeks after the baseline visit. RESULTS: At 12 weeks after the baseline visit, the levodopa group experienced a significant (P = .016) mean difference in improvement of visual acuity of 5.9 letters from the placebo group. At 24 weeks after the baseline visit, a significant treatment effect (P = .036) for visual acuity was still evident; the levodopa group had a mean gain in improvement of 7.5 letters difference from baseline from the placebo group. Three subjects in the levodopa group experienced a doubling of the visual angle as denoted by a gain of at least 15 letters. Significant improvement was not observed for color vision (P = .82) or mean deviation of visual field loss (P = .82). CONCLUSION: The study found significant improvement of visual acuity among subjects receiving levodopa and carbidopa despite long-standing visual loss from nonarteritic anterior ischemic neuropathy. Confirmation of our results is awaited from larger population studies and with a longer follow-up time interval regarding the efficacy of levodopa in reversing visual loss in this disease.

Adult periorbital fibromatosis.

Three patients aged 15 to 40 years with fibromatosis of the periorbital region were studied. A history of acute onset, preceding trauma, and pain associated with a palpable mass were typical features. Rapid recurrence followed surgical excision. Misdiagnosis is common in this benign disease and may lead to unnecessary examinations for malignancy. Surgical excision or debulking of these masses is indicated for diagnosis and pain relief, but long-term follow-up indicates that these lesions will often spontaneously resolve with time.

Branch retinal artery occlusion secondary to percutaneous transluminal coronary angioplasty.

We report on a patient suffering a branch retinal artery occlusion immediately following percutaneous transluminal coronary angioplasty. Balloon angioplasty may produce ocular embolic events consistent with previously reported neurological complaints.

Astigmatism associated with adnexal masses in infancy.

High degrees of astigmatism are common in infants with hemangiomas but have not been well documented with other adnexal masses. We reviewed records of 65 patients (69 eyes) with chalazions, epibulbar or orbital dermoids, hemangiomas, and dacryoceles. Astigmatism greater than +1.25 diopters (as high as +5.50 diopters) was most commonly associated with dacryoceles (eight of 12 eyes) and with hemangiomas (14 of 17 eyes). Plus cylinder axes were consistently oriented toward the lesion, and astigmatism tended to resolve with resolution of the lesions. Only one of the 17 eyes with dermoids and two of the 23 eyes with chalazions had astigmatism. Anisometropic amblyopia has been a prominent concern in the treatment of infants with hemangiomas. Patients with dacryoceles may be at similar risk. Repeated cycloplegic refractions are important in determining appropriate treatment of adnexal masses.

Ocular Munchausen's syndrome.

Patients with contrived histories and/or self-induced physical abnormalities (Munchausen's syndrome) are often successful in deceiving physicians. We recently cared for four patients with ocular Munchausen's syndrome. Self-induced ocular manifestations included voluntary nystagmus, subconjunctival hemorrhages, chronic orbital emphysema requiring exenteration, corneal alkali burns, erosions and ulcerations, and abscesses of the periorbital area. Correct diagnoses of ocular Munchausen's syndrome were made only after extensive medical and surgical investigations. Suggestions for evaluation and treatment will also be discussed.

Functional visual loss in children.

Twenty-three children (16 girls, 7 boys, aged 6-17 years) who presented with the specific complaint of blurred vision were diagnosed as having functional visual loss. Symptoms were intermittent in seven children. Associated signs and symptoms were common and included headaches, visual field loss, diplopia, micropsia, voluntary nystagmus, and spasm of the near reflex. Our treatment consisted of reassurance and follow-up. Resolution of symptoms occurred within 24 hours in one third and within two months in three-quarters of our patients. Parental support and encouragement were associated with more rapid resolution. Recurrence of symptoms and late onset of somatic complaints were rare. Conflicts related to family or school environment were common. Four children had been sexually or physically abused. Our experience suggests that, regardless of the duration or severity of symptoms, functional visual loss in children can usually be treated with reassurance. We believe that psychiatric referral is not necessary for most patients. Sexual or physical abuse should be considered as a possible predisposing factor.

Posttraumatic convergence insufficiency.

Convergence insufficiency was encountered following closed head trauma in 23 patients. The most common complaints included difficulty reading and diplopia at near. The severity of the head trauma varied. There was no correlation between the severity of the head trauma and the severity of the convergence insufficiency. A normal near point of convergence was encountered in six of the 23 patients; all six patients had abnormal convergence reserves when measured with prisms. Treatment consisted primarily of convergence exercises and prisms. Bilateral medial rectus resections were required in two patients. Response to therapy was variable and often incomplete. The anatomic localization of convergence insufficiency secondary to head trauma remains unknown, although lesions in the occipital lobe and upper midbrain both seem capable of producing this syndrome.