Detection of nerve fiber atrophy in apparently effectively treated papilledema in idiopathic intracranial hypertension.

BACKGROUND: Since papilledema in idiopathic intracranial hypertension is a passive event not primarily affecting the visual tract, resolution with restitution ad integrum is expected if intracranial pressure is rebalanced. Retinal nerve fiber swelling due to papilledema in the acute phase and possible axon loss after long-lasting elevated intracranial pressure was investigated in a controlled cross-sectional study by scanning laser polarimetry. METHODS: A cohort of 23 patients with idiopathic intracranial hypertension according to the modified Dandy diagnostic criteria, and 23 controls matched for age and gender were investigated. All patients received neurological and ophthalmologic examination including scanning laser polarimetry (GDx VCC). Patients were divided into groups depending on the presence of a papilledema (group 1) or the regression of the papilledema after initiation of therapy (group 2). Therapy was based on recommendations of the German Society of Neurology. RESULTS: Scanning laser polarimetry showed an increase of nerve fiber thickness in group 1, and a decrease of the nerve fiber thickness in group 2 compared to controls. Ten of 13 patients showed signs of a regional axon loss in the deviation map of the GDx report, and six had a Nerve Fiber Index above 30. All patients with regressive papilledema and coincidence of visual field damage and pale optic disc appearance had a pathologic result in the GDx examination, but only four of ten patients with a pathologic GDx examination showed coincidence of pale optic disc appearance and visual field damage as sign of underlying optic disc atrophy. CONCLUSION: In patients with apparently effective treatment of clinical symptoms and a regression of papilledema in idiopathic intracranial hypertension, a retinal axon loss was detected by scanning laser polarimetry. Axon loss was even present in patients without clinical evidence of optic nerve atrophy.

Neuromyelitis optica presenting with relapses under treatment with natalizumab: a case report.

INTRODUCTION: Neuromyelitis optica is an inflammatory demyelinating disease of the central nervous system. To date, optimal therapeutic approaches for neuromyelitis optica have yet to be defined. Natalizumab is highly effective in relapsing-remitting multiple sclerosis and might be considered as an option. CASE PRESENTATION: Here, we describe a 67-year-old Caucasian man with definite neuromyelitis optica with detection of anti-aquaporin-4 antibodies over the course of the disease. After initially discussing the diagnosis of multiple sclerosis at an outside hospital, our patient received interferon beta 1a as well as repeated corticosteroid pulses without success. Under subsequent therapy with natalizumab, he continued to present relapses. It was not until discontinuation of natalizumab, repeated cycles of plasma exchanges and initiation of therapy with rituxan that the disease course started to stabilize. Although B cells were completely depleted, our patient experienced another severe myelitis relapse during further follow-up and an additional immunosuppressive therapy with cyclophosphamide was started. Under this regimen, no further relapses occurred over the next 24 months. CONCLUSIONS: This case adds further evidence to the previously discussed notion that natalizumab, while highly effective in multiple sclerosis, may not work sufficiently in neuromyelitis optica. It further advocates for repetitive testing of anti-aquaporin-4 antibodies before and after treatment initiation.