Cerebral small vessel diseases: manifestations in young women

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.

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.

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.