Robert L. Lesser

Neuro-ophthalmologic manifestations of lyme disease

Lyme disease is a tick-borne spirochetal infection characterized by skin rash, neurologic, cardiac, and arthritic findings. The authors report six patients with Lyme disease who had neuro-ophthalmologic manifestations. One patient had meningitis with papilledema, two had optic neuritis, and one had neuroretinitis. Three patients had sixth nerve paresis, two of whom cleared quickly, whereas multiple cranial nerve palsies and subsequent optic neuropathy developed in another. Early recognition of neuro-ophthalmologic findings can help in the diagnosis and treatment of Lyme disease.

The effect of prednisone on the progression from ocular to generalized myasthenia gravis.

Fifty percent of ocular myasthenia gravis (OMG) patients will progress to generalized myasthenia, 90% within 3 years from the onset of ocular symptoms. This study was performed to determine whether treatment with oral prednisone initiated and completed within 2 years from the onset of ocular symptoms would affect the progression of ocular myasthenia to generalized myasthenia gravis (GMG). Fifty-six patients were included in this review, with 27 patients in the prednisone-treated group and 29 patients in the untreated group. The treated group was initiated on 60 mg of prednisone daily with a slow taper over 3-6 months. At 2 years, significantly fewer patients in the treated group (3 of 27) progressed to generalized myasthenia when compared to the untreated group (10 of 29) (chi(2), p=0.04). Our results suggest that the early use of steroids may decrease progression of ocular to generalized myasthenia gravis. The decision to use steroids should be considered early in the course of patients diagnosed with ocular myasthenia gravis. This study should be considered preliminary and a prospective trial is warranted to confirm our observations.

Bilateral Keratitis in Lyme Disease

Lyme disease, caused by the spirochete Borrelia burgdorferi, has ophthalmic manifestations. The authors describe two cases of Lyme keratitis characterized by multiple focal, nebular opacities at varying levels of the stroma which may progress to edema, neovascularization, and scarring. Close observation, in addition to systemic antibiotic therapy, may be sufficient if the visual axis is not involved, and the patient is asymptomatic.

Long-term response to fractionated radiotherapy of presumed optic nerve sheath meningioma

Background/aims To review the long-term results of treatment of optic nerve sheath meningiomas (ONSMs) with conformal radiotherapy. Methods Eleven patients with presumed ONSM were treated with fractionated conformal radiotherapy using 45–54 Gy in 25–30 fractions and followed for more than 5 years. Affected eye visual acuity in logMAR notation, colour vision, threshold perimetry, mean deviation (dB) and imaging were studied before and after treatment. Analysis Included adverse effects of treatment and the frequency of affected eye maintained or improved vision. Results There were nine women and two men with a mean age of 45.3. Vision or field loss was the most common presentation. All had abnormal MR imaging. The follow-up period after radiotherapy was 61–156 months (mean 89.6). Visual acuity was unchanged or improved in 10 patients (91%). The average difference between pretreatment and final logMAR visual acuity was 0.08, while the average difference in visual-field mean deviation was −4.63 dB. The radiographic appearance of the tumours was stable in size in nine patients and decreased in two. No major side effects from radiation were seen. Conclusions Vision, MRI and complication outcomes are favourable for using fractionated conformal radiotherapy for ONSM.

Retinoblastoma and chromosome abnormality. Partial deletion of the long arm of chromosome 13.

A 7-year-old girl with retarded physical and mental development was found, on chromosome determination, to have deletion of a segment of the long arm of chromosome 13 (13 q—). A precise chromosome identification was made on peripheral leukocytes by using a quinacrine banding technique. Because of previous reports of an association of the 13 q—chromosomal abnormality with retinoblastoma, an ophthalmologic examination was carried out. The patient was found to have bilateral microphthalmia, and multifocal, moderately well-differentiated retinoblastoma in the left eye.

The Heidenhain variant of Creutzfeldt-Jakob disease: clinical, pathologic, and neuroimaging findings.

We report two patients who developed isolated visual symptoms and signs as initial manifestations of Creutzfeldt–Jakob disease (CJD). Both patients had normal conventional T1-and T2-weighted brain magnetic resonance (MR) images; in one patient, early cortical abnormalities were detected by diffusion-weighted and fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI). Results from the cerebrospinal fluid assay for the 14-3-3 brain protein were also negative in one patient, despite pathologic confirmation of CJD at autopsy. The Heidenhain variant of CJD should be considered in all patients who present with isolated visual manifestations, including homonymous hemianopsia and normal conventional brain MRI. Diffusion-weighted and FLAIR MRI may demonstrate early cortical abnormalities in patients with CJD. The CSF assay for the 14-3-3 protein may be normal, even in pathologically confirmed cases.

Creutzfeldt-Jakob disease and optic atrophy.

A 49-year-old man developed ataxia, myoclonic jerks, cortical blindness, and dementia. In 3 1/2 months, he rapidly deteriorated and died. Clinical and autopsy diagnosis confirmed Creutzfeldt-Jakob disease. The eyes were examined and bilateral optic atrophy was noted. No other ocular changes were noted. Optic atrophy had not been noted before death.

Ocular myasthenia gravis

Purpose of review To review ocular myasthenia gravis (OMG), a localized form of myasthenia gravis clinically involving only the extraocular, levator palpebrae superioris, and orbicularis oculi muscles. Recent findings Ocular manifestations can masquerade as a variety of ocular motility disorders, including central nervous system disorders and peripheral cranial nerve palsies. While sparing the pupils, the diagnosis and management can be challenging. Summary Because several diagnostic and treatment options are available for OMG, clinicians must decide the sequence and combination based on the level of disease activity and patient disability.

Is intravitreal bevacizumab an effective treatment option for nonarteritic anterior ischemic optic neuropathy

Abstract Nonarteritic anterior ischemic optic neuropathy (NAION) causes sudden profound loss of vision with no known cause or cure. Various treatment modalities, both surgical and pharmacologic, have been tried without success. The purpose of our retrospective study was to evaluate the effect of intravitreal bevacizumab (Avastin) as a treatment option for NAION. We evaluated demographics of 5 patients and compared visual acuity and automated visual fields prior to and following intravitreal bevacizumab injection. Visual acuity at presentation was 20/20 in 4 of 5 patients and 20/150 in 1. Visual acuity improved to 20/40 in the patient who presented with decreased acuity and decreased slowly in 3 patients and rapidly in 1. All patients presented with variable visual field defects: 1 improved slightly, 3 progressed, and 1 remained stable. One patient subsequently developed NAION in the fellow eye. These results are consistent with the natural course of the disease, and bevacizumab did not appear to have a dramatic effect on the clinical outcome in this small series of patients with NAION.

Cryptococcal meningitis and internal ophthalmoplegia.

A 17-year old girl received prednisone and azathioprine for the treatment of systemic lupus erythematosus. She developed a fever and hallucinations 18 months later; cryptococcal meningitis was diagnosed. An internal ophthalmoplegia with loss of accommodation and dilation of the pupils developed together with bilateral lateral rectus palsy. Treatment with intravenous amphotericin resulted in disappearance of papilledema, muscle palsy, and internal ophthalmoplegia. We believe that the internal ophthalmoplegia was secondary to involvement of the accommodative and pupillary fibers of both third nerves at the base of the brain.