Selected Neuro-Ophthalmic Disorders Associated With Pregnancy
During pregnancy or the postpartum period, several neuro-ophthalmic abnormalities can be exacerbated or occur with greater frequency, including cerebral venous thrombosis (CVT), idiopathic intracranial hypertension (IIH), pituitary apoplexy, and lymphocytic hypophysitis. CVT is discussed later in this chapter (see the section Cerebrovascular Disorders), and IIH and pituitary apoplexy are discussed in Chapter 4. Preexisting pituitary macroadenomas, meningiomas, schwannomas, and orbital and choroidal hemangiomas can also undergo rapid expansion during pregnancy.
A number of cranial neuropathies have been reported during pregnancy, most frequently involving the CN VII followed by the CN VI. These cranial neuropathies are thought to result from an increase in interstitial fluid around the nerve, causing compression. Hypercoagulopathy and hypertension have also been proposed as potential causes.
Severe preeclampsia/eclampsia can lead to various ophthalmic manifestations, including serous retinal detachment, choroidal infarction, retinal vascular narrowing, and retinal artery vasospasm or occlusion. The optic nerve may become edematous secondary to systemic hypertension, intracranial hypertension, or ischemic optic neuropathy. Disorders of higher cortical function, such as alexia, simultagnosia, and cortical blindness have also been reported. In addition, PRES may develop in the context of preeclampsia/ eclampsia (see the following section).
Digre KB. Neuro-ophthalmology and pregnancy: what does a neuro-ophthalmologist need to know? J Neuroopthalmol. 2011;31(4):381–387.
Posterior Reversible Encephalopathy Syndrome
PRES is characterized by headache, altered mental status, seizures, and visual disturbances (blurred vision, homonymous visual field loss, cortical blindness, photopsias, and visual hallucinations). MRI shows T2-hyperintense vasogenic edema involving the white matter of the cerebral posterior regions, especially the parieto-occipital lobes; however, the frontal and temporal lobes, basal ganglia, and brainstem may be involved as well (Fig 14-12). A variety of conditions can predispose a patient to PRES; common causes include acute hypertension, preeclampsia/eclampsia, immunosuppressive drugs (eg, cyclosporine, tacrolimus), renal disease, sepsis, and multiorgan dysfunction syndrome. It has been less commonly reported in patients with autoimmune disease. The abnormalities visible on neuroimaging are reversible, and the visual prognosis is usually excellent. The underlying pathophysiology of PRES remains elusive.
Table 14-2 Neurocutaneous Syndromes
Figure 14-12 Axial FLAIR MRI scan shows reversible bilateral posterior circulation signal abnormalities in a patient with transient cortical blindness and preeclampsia.
(Courtesy of Lanning B. Kline, MD.)
Fugate JE, Claassen DO, Cloft HJ, Kallmes DF, Kozak OS, Rabinstein AA. Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin Proc. 2010; 85(5):427–432.
Excerpted from BCSC 2020-2021 series: Section 5 - Neuro-Ophthalmology. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.