Sonographic lenticulostriate vasculopathy in infancy with tic and other neuropsychiatric disorders developed after 7 to 9 years of follow-up

On gray-scale transfontanel sonography, the small arteries supplying the basal ganglia are indistinct from the brain parenchyma in normal infants. Bright linear ‘branched candlestick’ stripes in these regions, suggesting sonographic lenticulostriate vasculopathy, were reported in more than 200 infants in the English literature; including 34 our own patients. To identify its long-term outcome, a prospective study was accomplished on our 34 infants with sonographic lenticulostriate vasculopathy which included 13 cryptogenic cases and 21 with distinct etiologies. At the age of 7 to 9 years in the cryptogenic group, 7 in 13 patients developed tics, attention deficits, hyperactivity, and/or obsession/compulsion; while in the symptomatic group only 2 of 21 patients had tics. The rate of mortality (33% vs. 0%), developmental delay (24% vs. 8%), mental retardation (24% vs. 0%), and neurologic deficits (29% vs. 0%) were significantly higher in the symptomatic group than the cryptogenic group. Comparatively, the occurrence rate of attention deficit-hyperactivity disorder (10% vs. 54%), tics (10% vs. 38%), and obsessive-compulsive disorder (5% vs. 13%) were significantly lower in the symptomatic group than the cryptogenic group. The rates of these neuropsychiatric disorders were 10% in the symptomatic group and 54% in the cryptogenic group. We concluded that idiopathic sonographic lenticulostriate vasculopathy in infancy may predict development of neuropsychiatric disorders later in childhood.