Martha P. Schatz

Optic neuritis after anthrax vaccination

OBJECTIVE To report the occurrence of optic neuritis after anthrax vaccination in two patients. DESIGN Observational case reports, review of literature. METHODS Description of clinical history, examination, neuroimaging, and further studies in two patients experiencing optic neuritis in temporal association with anthrax vaccination. MAIN OUTCOME MEASURES Visual acuity, visual fields. RESULTS Two patients, 39 and 23 years of age, were seen with acute optic neuritis 1 month and 2 weeks, respectively, after anthrax booster vaccination and successfully treated with intravenous methylprednisolone. The first patient had a typical presentation and course of unilateral retrobulbar optic neuritis with excellent visual recovery. The second patient had a bilateral anterior optic neuritis and has required chronic immunosuppression to maintain his vision. Retinal and optic nerve autoantibodies were present in the second patient. No cross-reactive epitopes between anthrax vaccine and retina/optic nerve were identified. CONCLUSION Optic neuritis is a potential adverse reaction of anthrax vaccination.

Tetrasomy 18p: report of the molecular and clinical findings of 43 individuals.

Thus far, the phenotype of tetrasomy 18p has been primarily delineated by published case series and reports. Findings reported in more than 25% of these cases include neonatal feeding problems, growth retardation, microcephaly, strabismus, muscle tone abnormalities, scoliosis/kyphosis, and variants on brain MRI. Developmental delays and cognitive impairment are universally present. The purpose of this study was to more fully describe tetrasomy 18p at both the genotypic and the phenotypic levels. Array CGH was performed on 43 samples from individuals with tetrasomy 18p diagnosed via routine karyotype. The medical records of 42 of these 43 individuals were reviewed. In order to gain additional phenotypic data, 31 individuals with tetrasomy 18p underwent a series of clinical evaluations at the Chromosome 18 Clinical Research Center. Results from the molecular analysis indicated that 42 of 43 samples analyzed had 4 copies of the entire p arm of chromosome 18; one individual was also trisomic for a section of proximal 18q. The results of the medical records review and clinical evaluations expand the phenotypic description of tetrasomy 18p to include neonatal jaundice and respiratory distress; recurrent otitis media; hearing loss; seizures; refractive errors; constipation and gastroesophageal reflux; cryptorchidism; heart defects; and foot anomalies. Additional findings identified in a small number of individuals include hernias, myelomeningocele, kidney defects, short stature, and failure to respond to growth hormone stimulation testing. Additionally, a profile of dysmorphic features is described. Lastly, a series of clinical evaluations to be considered for individuals with tetrasomy 18p is suggested.

Occlusion properties of prosthetic contact lenses for the treatment of amblyopia.

BACKGROUND The efficacy of opaque contact lenses as occlusion therapy for amblyopia has been established in the literature. Prosthetic contact lenses use similar tints to improve cosmesis in scarred or deformed eyes and may be an alternative in occlusion therapy. To test this idea, we determined the degree of vision penalization elicited by prosthetic contact lenses and their effect on peripheral fusion. METHODS We tested 19 CIBA Vision DuraSoft 3 Prosthetic soft contact lenses with varying iris prints, underprints, and opaque pupil sizes in 10 volunteers with best-corrected Snellen distance visual acuity of 20/20 or better in each eye. Snellen visual acuity and peripheral fusion using the Worth 4-Dot test at near were measured on each subject wearing each of the 19 lenses. RESULTS Results were analyzed with 3-factor analysis of variance. Mean visual acuity through the various lenses ranged from 20/79 to 20/620. Eight lenses allowed preservation of peripheral fusion in 50% or more of the subjects tested. Iris print pattern and opaque pupil size were significant factors in determining visual acuity (p < 0.05). CONCLUSIONS Sufficient vision penalization can be achieved to make occlusion with prosthetic contact lenses a viable therapy for amblyopia. The degree of penalization can be varied and different iris print patterns and pupil sizes, using peripheral fusion, can be preserved with some lenses. Prosthetic contact lenses can be more cosmetically appealing and more tolerable than other amblyopia treatment modalities. These factors may improve compliance in occlusion therapy.

Topiramate-induced palinopsia: A case series and review of the literature

Background: To report palinopsia as a possible side effect of topiramate. Methods: Case series and review of the literature. Results: Nine patients in our series, and 4 previously reported patients, who developed palinopsia while on topiramate, are reviewed. All patients were women, and comorbidities included migraine, idiopathic intracranial hypertension, and bulimia nervosa. Palinopsia resolved in 8 patients after stopping or decreasing the dose of topiramate. The lowest dose of topiramate causing palinopsia was 25 mg twice a day. More than half of our patients reported exacerbation of visual disturbance in early morning or late evening. Conclusions: Topiramate-induced palinopsia may be underdiagnosed because physicians do not inquire about such visual symptoms.

Ophthalmic manifestations of tetrasomy 18p.

PURPOSE To characterize ophthalmic findings in patients with tetrasomy 18p, a rare chromosomal anomaly that has been previously associated with strabismus. METHODS All subjects underwent a complete eye examination to screen for ocular pathology. RESULTS A total of 25 subjects (13 female) were examined after they were diagnosed with tetrasomy 18p. The average age of subjects was 8.2 years (range, 13 months to 22 years). Of the 25 subjects, 18 (72% of examined subjects, 42% of the cohort) showed evidence of strabismus; 16 had esotropia (8 uncategorized, 5 infantile, and 3 accommodative), 1 had esophoria, and 1 was diagnosed with intermittent exotropia. CONCLUSIONS The coincidence of esotropia with tetrasomy 18p indicates the need to routinely screen these patients for strabismus at the time of diagnosis.

Scalp necrosis with giant cell arteritis11The views expressed in this article are those of the authors and do not reflect the official policy of the Department of Defense or other departments of the U.S. Government.

OBJECTIVE Giant cell arteritis (GCA) often presents with devastating visual loss in the elderly, yet early diagnosis and treatment can prevent visual loss. The authors report two cases of GCA in which scalp necrosis, a rare finding not reported previously in the ophthalmic literature, played an important role in diagnosis. DESIGN Observational case reports and literature review. PARTICIPANTS Two patients with GCA had scalp necrosis. INTERVENTION Intravenous steroid administration was performed. MAIN OUTCOME MEASURE Vision and healing of scalp necrosis were measured. RESULTS One patient had scalp necrosis that was diagnosed incorrectly preceding visual loss. Another patient had visual loss in one eye diagnosed as nonarteritic anterior ischemic optic neuropathy. He had scalp necrosis develop 2 weeks later, leading to the correct diagnosis of GCA. There are 21 previously reported cases of scalp necrosis with GCA, none in the ophthalmic literature, most of which were undiagnosed until the onset of visual loss. In addition, a higher rate of visual loss is seen in GCA with scalp necrosis. CONCLUSIONS Scalp necrosis is a valuable sign that frequently is misdiagnosed until visual loss occurs. In addition, it may indicate a more severe vasculitis. This finding should be added to the list of signs evaluated in patients for whom GCA is in the differential diagnosis.