Cerebral small vessel diseases: manifestations in young women

Abstract

Purpose of review: Cerebral small vessel diseases are responsible for 20-30% of ischemic strokes as well as for a considerable proportion of cerebral hemorrhages and encephalopathies. Less known than the manifestations in old age are those in young women comprising posterior encephalopathy and Susac's syndrome.

Recent findings: Magnetic resonance imaging has allowed us to identify posterior encephalopathy, characterized by headache, seizures, visual disturbances and hypertension, as a frequent complication of preeclampsia/eclampsia syndrome in the perinatal and postpartum period. Magnetic resonance findings are pathognomonic with bilateral cortical-subcortical lesions in the posterior hemispheres typically sparing the calcarine fissure. Conversely, white matter lesions on magnetic resonance imaging particularly involving the corpus callosum, and a (subclinical) sensorineural hearing loss are diagnostic of the retinocochleocerebral vasculopathy called Susac's syndrome.

Summary: Posterior encephalopathy can also follow from a broad spectrum of endotheliotoxic conditions like chemotherapy, immunosuppression and sepsis. Early recognition of the disorder is decisive for a benign outcome since therapy consists of removal of precipitating factors, lowering of blood pressure and treatment with magnesium sulfate. The retinocochleocerebral vasculopathy appears to be underdiagnosed. An autoimmune-mediated arteriolopathy is presumed and the disease is nearly always monophasic. A variety of therapeutic approaches have been recommended, none of which, however, is based on anything other than anecdotal evidence.