Faculty Bibliography

Several disease-modifying agents (DMAs) are approved for the treatment of multiple sclerosis, including three interferon (IFN)-beta products, glatiramer acetate and mitoxantrone. This article reviews the adverse event profiles of these DMAs based on the pivotal phase III trials, and provides practical guidelines for managing adverse effects. In general, the most common adverse events associated with IFN beta therapy are flu-like symptoms, including fever, chills and myalgias, and headache. The flu-like symptoms typically resolve within 24 hours and may be mitigated by over-the-counter anti-inflammatory agents. Adverse events related to glatiramer acetate therapy include injection-site reactions and a systemic reaction consisting of flushing, chest tightness, palpitation, anxiety or dyspnoea. The systemic reaction is transient (30 seconds to 30 minutes) and self-limited. Mitoxantrone may cause nausea, vomiting, alopecia, amenorrhoea and myelosuppression; isolated cases of acute leukaemia and dose-related cardiotoxicity have been reported in the literature. Longer-term tolerability data on mitoxantrone as a treatment for multiple sclerosis are needed. It is important for physicians to counsel patients on DMA-related adverse effects, most of which are transient and of mild-to-moderate severity. Various strategies that can be employed to prevent or manage these adverse effects and lessen their impact on the patient are discussed.

Multiple sclerosis (MS) is the most common disabling neurological disease in young people. Most CNS lesions involve neuroanatomically non-eloquent zones that often do not result in symptomatic complaints. By contrast, tissue-injury mechanisms involving inflammatory demyelination can involve more eloquent sites, such as the optic nerve and brainstem, which can correspondingly produce the development of well recognised syndromes such as optic neuritis and internuclear ophthalmoplegia, respectively. In this review we discuss the broad landscape of abnormalities that affect the afferent visual system and the ocular motor apparatus, and emphasise relevant features, the recognition and treatment of which are of importance to general neurological practice. The commonness of visual sensory and eye movement abnormalities in MS highlights the importance of understanding the principles addressed in this review.

The role for immediate neuroimaging in patients 50 years of age or older with acute isolated third, fourth, and sixth nerve palsies is controversial. We prospectively evaluated 66 patients, aged 50 years and older (median 67 years, range 50-85), with acute isolated ocular motor mononeuropathies. Our purpose was to evaluate both the role of neuroimaging and the role of clinical assessment in determining etiology. We found that clinical features, including time to maximal diplopic symptoms, were not predictive of etiology (median 2 days to maximal diplopic symptoms for both peripheral microvascular and other etiologies). The presence of any common vascular risk factor, including diabetes mellitus, hypertension, hypercholesterolemia, or coronary artery disease, was significantly associated with peripheral microvascular etiology in this cohort (p=0.0004, Fisher's exact test). Despite the high prevalence of peripheral microvascular ischemia as an etiology in this age group, other causes were identified by magnetic resonance imaging (MRI) or computed tomography (CT) scanning in 14% of patients. Diagnoses included brainstem and skull base neoplasms, brainstem infarcts, aneurysms, demyelinating disease, and pituitary apoplexy. Neuroimaging procedures may have a role in the initial evaluation of patients 50 years of age or older with acute ocular motor mononeuropathies.

PURPOSE: To describe the neuro-ophthalmic presentations of a rare intracranial tumor, hemangiopericytoma. METHODS: Retrospective multicenter case series. RESULTS: The neuro-ophthalmic and radiographic features of hemangiopericytoma are reviewed. The clinical presentation may mimic meningioma and the pre-operative distinction between meningioma and hemangiopericytoma is important because the evaluation, management, treatment, and prognosis differ significantly for the two lesions. CONCLUSION: We report five cases of intracranial hemangiopericytoma and review the neuro-ophthalmic findings of this uncommon entity.

We report a woman with anti-Yo-associated cerebellar degeneration, severe eye movement abnormalities, and no evidence of malignancy after 3 years of extensive evaluation. The prominent neuro-ophthalmologic findings suggest brainstem involvement, which may be a novel manifestation of anti-Yo associated paraneoplastic syndrome.

Visual symptoms frequently complicate the course of multiple sclerosis (MS). The ophthalmologist must be familiar with the neuro-ophthalmologic manifestations of MS to facilitate the initial evaluation and treatment of patients. Magnetic Resonance Imaging has become an important tool to confirm the diagnosis of MS or to assess the risk of MS in patients with clinically isolated demyelinating syndromes. This article reviews the neuro-ophthalmologic manifestations of MS and the management issues that arise in early MS.

We report a patient who presented with Balint's syndrome as a manifestation of primary central nervous system angiitis. Clinical findings included simultanagnosia, optic ataxia, and optic apraxia. Pathologic evaluation demonstrated amyloid angiopathy and Alzheimer's plaques. The presence of primary central nervous system angiitis along with amyloid angiopathy and Alzheimer's plaques may not be coincidental. Angiitis may be a foreign body reaction to A4 amyloid deposition.

Patients who present with visual loss that cannot be explained by organic lesions represent a wide spectrum of patients from those with no physiologic problem to those patients who have a true underlying condition. Regardless of where a patient falls within this spectrum, all patients need to be approached with a clinical evaluation to ensure that no underlying physiologic deficit exists. After excluding organic causes with appropriate examination and testing, a patient's visual loss still should not be labeled as functional until it is proven that they can see better than they claim to see. Only after convincingly demonstrating better vision can the physician begin to consider treatment options to help the patient's vision recover. Although functional visual loss places the physician in an unusual adversarial position of refuting a patient's symptoms, exposing the patient in a confrontational manner rarely helps. Instead, an approach that allows patients to resolve the symptoms on their own through reassurance and support often leads to successful restoration of vision. Reassurance that their condition is not serious, and may recover with time, allows patients to slowly admit their vision is improving without ever suggesting that the concern and medical attention they sought was unwarranted.