Pallidonigral Degeneration: A Recognizable Pattern of Secondary Degeneration Following Striatal Injury

Abstract

Primary brain injuries can give rise to specific, predictable patterns of secondary neuroaxonal degeneration. We report a novel imaging pattern of pallidonigral degeneration involving both the globus pallidus and the substantia nigra following ipsilateral striatal insult in children. In this clinical report, we present a series of 10 pediatric patients with secondary pallidonigral degeneration in three distinct temporospatial patterns. Pallidonigral degeneration manifested with cytotoxic injury in the globus pallidus around day 6-7 after symptomatic presentation of acute striatal injury, followed by additional involvement of the substantia nigra around day 9-10, with documented resolution in one patient by approximately one month. In patients with subacute or chronic progressive striatal injury, pallidonigral degeneration may be acute at the time of initial magnetic resonance evaluation. Recognition of these imaging patterns allows their distinction from ongoing damage linked to the underlying etiology of striatal insult and is crucial for directing appropriate management.