Severe and rapidly progressive visual loss suggests "fulminant idiopathic intracranial hypertension" and should prompt aggressive management. Urgent surgery may be required in these patients, and temporizing measures such as repeat lumbar punctures, lumbar drainage, and IV steroids considered.
Purpose:
Central retinal artery occlusion (CRAO) is a form of acute ischemic stroke that causes severe visual loss and is a harbinger of further cerebrovascular and cardiovascular events. There is a paucity of scientific information on the appropriate management of CRAO, with most strategies based on observational literature and expert opinion. In this scientific statement, we critically appraise the literature on CRAO and provide a framework within which to consider acute treatment and secondary prevention.
Methods:
We performed a literature review of randomized controlled clinical trials, prospective and retrospective cohort studies, case-control studies, case reports, clinical guidelines, review articles, basic science articles, and editorials concerning the management of CRAO. We assembled a panel comprising experts in the fields of vascular neurology, neuro-ophthalmology, vitreo-retinal surgery, immunology, endovascular neurosurgery, and cardiology, and document sections were divided among the writing group members. Each member received an assignment to perform a literature review, synthesize the data, and offer considerations for practice. Multiple drafts were circulated among the group until consensus was achieved.
Results:
Acute CRAO is a medical emergency. Systems of care should evolve to prioritize early recognition and triage of CRAO to emergency medical attention. There is considerable variability in management patterns among practitioners, institutions, and subspecialty groups. The current literature suggests that treatment with intravenous tissue plasminogen activator may be effective. Patients should undergo urgent screening and treatment of vascular risk factors. There is a need for high-quality, randomized clinical trials in this field.
There are differences in the shape and depth of the ipsilateral and the contralateral field defects not previously reported. These findings demonstrate that certain fibers from the ipsilateral eye travel more anteriorly and laterally in Meyer's loop, and support the hypothesis that visual field defects due to anterior retrogeniculate lesions are relatively incongruous because of anatomic differences in the afferent pathways. Automated perimetry is a sensitive method of evaluating and quantifying visual field defects.
Four patients each developed a reversible homonymous hemianopia caused by non-ketotic hyperglycemia. In two patients the homonymous hemianopia was the first manifestation of diabetes mellitus type 2. All four patients had abnormal MRI scans; in the three patients who had late follow-up scans the abnormalities resolved completely. In one patient the progressive visual field defect and unusual MRI findings initially caused concern for a tumor.
Using inductively coupled argon plasma emission spectroscopy, we measured 19 trace elements in cerebrospinal fluid of 265 patients who were undergoing diagnostic lumbar puncture. Thirty-three patients had Alzheimer-type dementia (ATD); 16 patients had other dementing illnesses; and 20 had no neurologic disease. There were seven cases of autopsy-proven Alzheimer's disease (AD) and eight autopsy controls. We found elevated CSF silicon in 24% of ATD and 71% of AD patients. We found no relationship between CSF aluminum, arsenic, lead, or manganese and ATD, AD, or other dementing illnesses.
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