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A 32-year-old woman was admitted for mild gait difficulty and behavioural changes of 3 years’ duration. Familial and medical histories were unremarkable. Neurological examination revealed: mildly slowed gait, decreased synkinesias of the left arm (held abducted and flexed), increased deep tendon reflexes, Babinski signs, mild lower limb spasticity and dysmetria of left limbs. Depression and mild executive dysfunctions were also found.
Brain CT showed cerebral white matter (WM) calcifications (fig 1). MRI revealed T2 WM hyperintensities in the centra semiovalia, around the frontal horns and, to a lesser extent, in the posterior regions. Post-contrast MRI demonstrated points and stripes of enhancement in the periventricular WM, which are consistent with engorged medullary vessels (fig 2). Proton …