Adult unilateral periventricular pseudocysts with ipsilateral headache

Objectives Unilateral periventricular pseudocysts (PVPC), manifesting as ipsilateral headache have been only rarely reported in adults. Case report In a 48-year-old woman, right-sided, pulsating headache occurred in 1999. Headache exclusively extended over the right head and periorbitally; it was frequently associated with a feeling of cold, lasted maximally for 1 day, and occasionally went along with nausea and right-sided lacrimation. Since January 2003, headache occurred daily with varying intensity. For headache, she was regularly taking doxepin (25 mg). Ordinary analgesics and tryptanes were only of minimal effect. Since 2001, left-sided hemi-hypesthesia occurred. CT and MRI scans of the brain disclosed right-sided cysts in the white matter with a maximal diameter of 1 cm, partially grouped and partially disseminated and bilateral calcifications of the basal ganglia. Since hypoxemia/ischemia, subependymal hemorrhage, CNS infection, developmental defect of the mantle layer, chromosomal disorder, Zellweger syndrome, carbon monoxide intoxication, trauma, or mitochondriopathy were not causative, the etiology of PVPC remained questionable. Conclusions Rarely, unilateral PVPC become symptomatic in adulthood, manifesting as ipsilateral headache, contralateral hemi-hypesthesia, depression, collapses, and slight cognitive decline. Clinical progression of the disease is not necessarily related to the progression of the imaging findings. PVPC should be included in the differential diagnosis of unilateral headache.