Apical Orbital Aspergillosis Complicating Giant Cell Arteritis.

A 75-year-old woman with new onset headaches and left vision loss, temporal scalp tenderness, and jaw claudication was found to have biopsy-proven giant cell arteritis (GCA). Despite treatment and improvement with prednisone, she later developed left orbital apex syndrome, and an orbital biopsy revealed aspergillosis. After antifungal treatment, extraocular motility improved although vision in the left eye remained no light perception. Clinicians should be aware that fungal orbital apex disease may mimic or complicate steroid-treated GCA.

Optic Neuropathy Due to Chronic Lymphocytic Leukemia Proven With Optic Nerve Sheath Biopsy.

Central nervous system involvement from chronic lymphocytic leukemia (CLL) occurs infrequently, and manifestations include cognitive and cerebellar dysfunction and cranial nerve palsies. We report a 45-year-old man with CLL believed to be in clinical remission, who presented with vision loss and bilateral optic disc edema. His optic neuropathy due to CLL was proven by optic nerve sheath biopsy, and he experienced visual recovery after treatment with ibrutinib and intrathecal methotrexate.

What's in a name?

A 51-year-old man with rheumatoid arthritis and diabetes mellitus presented with new onset left-sided hemiparesis, left-sided neglect, and left-sided incongruous, denser inferiorly, homonymous hemianopsia. Magnetic resonance image of the brain showed prominent swelling of the right frontal, parietal, and occipital lobes greater than on the left with significant change in the fluid-attenuated inversion recovery signal in the gray matter. Perinuclear antineutrophil cytoplasmic antibodies titers were elevated, and skin biopsy demonstrating leukocytoclastic vasculitis. He showed marked clinical and radiographic improvement in association with recovery of vascular abnormalities after the initiation of prednisone, pulse therapy with methylprednisolone, and methotrexate. Clinicians should be aware of the possibility of neuro-ophthalmic manifestations of rheumatoid arthritis, including rheumatoid arthritis-related vasculitis causing homonymous hemianopsia.

Treatment of giant cell arteritis.

PURPOSE OF REVIEW: Giant cell arteritis (GCA) remains a potentially blinding inflammatory vasculitis of the elderly. Because prolonged doses of corticosteroids remain the best established treatment, side-effects during treatment are common and potentially serious. This review addresses the challenges clinicians face in managing this disease. RECENT FINDINGS: High-dose corticosteroids with slow tapering and close monitoring are the mainstay of treatment. Investigations into adjunctive treatment have yet to establish other agents as beneficial, but further research is ongoing with some promising results. SUMMARY: GCA represents a challenging illness to clinicians because of its potential for causing blindness and the need for prolonged high doses of corticosteroids with their many complications.

Idiopathic intracranial hypertension in the Middle East: A growing concern.

Idiopathic Intracranial Hypertension (IIH) is a disorder of increased intracranial pressure without any identifiable etiology. It is defined by elevated intracranial pressure (ICP) with normal neuroimaging and normal cerebrospinal fluid (CSF) contents. IIH typically affects young obese women and produces symptoms and signs related to high ICP. Headache and blurred vision are the most common symptoms, and papilledema is the major clinical sign. In this review we examine the epidemiology and demographic features of IIH in Middle Eastern countries and compare and contrast them with the published IIH literature from Western countries. The incidence of IIH in several Middle East countries has been estimated at 2.02-2.2/100,000 in the general population, which is higher than the Western rate. Obesity is a major risk factor globally and it is associated with an increased risk of severe vision loss due to IIH. There has been an increase in obesity prevalence in the Middle East countries mainly affecting the Gulf Council Countries (GCC), which parallels increased industrial development. This rise may be contributing to the increasing incidence of IIH in these countries. Other risk factors may also be contributing to IIH in Middle East countries and the differences and similarities to Western IIH merit further study.

Papilledema: epidemiology, etiology, and clinical management.

Papilledema is optic disc swelling due to high intracranial pressure. Possible conditions causing high intracranial pressure and papilledema include intracerebral mass lesions, cerebral hemorrhage, head trauma, meningitis, hydrocephalus, spinal cord lesions, impairment of cerebral sinus drainage, anomalies of the cranium, and idiopathic intracranial hypertension (IIH). Irrespective of the cause, visual loss is the feared morbidity of papilledema, and the main mechanism of optic nerve damage is intraneuronal ischemia secondary to axoplasmic flow stasis. Treatment is directed at correcting the underlying cause. In cases where there is no other identifiable cause for intracranial hypertension (ie, IIH) the available options include both medical and surgical modalities. Weight loss and diuretics remain the mainstays for treatment of IIH, and surgery is typically reserved for patients who fail, are intolerant to, or non-compliant with maximum medical therapy.

Autoimmune-Related Retinopathy and Optic Neuropathy Accompanied by Anti-GAD Antibodies.

Autoimmune-related retinopathy and optic neuropathy is characterized by visual loss in the presence of antibodies against retina or optic nerve antigens in the absence of neoplasia. We report a case with progressive central visual loss accompanied by latent autoimmune diabetes in an adult. Visual fields, multimodal imaging and electrophysiological testing showed characteristic changes without evidence of neoplasia on positron emission tomography. Immunologic testing revealed abnormal reactivity against a 20 kDa optic nerve antigen and 40 kDa and 62 kDa retina antigens along with antibodies against anti-glutamic acid decarboxylase, a constellation which we believe to be unique.