Glutaric aciduria type I is a rare metabolic disorder caused by deficiency of glutaryl-coenzyme A dehydrogenase. Chronic subdural hematomas have been reported in glutaric aciduria type I and are considered as important differential diagnosis of nonaccidental head trauma. However, chronic subdural hematomas are usually thought to remain clinically silent in these patients. Here we report on a hitherto asymptomatic glutaric aciduria type I patient who developed severe, acute subdural hemorrhage after minor accidental head injury at age 23 months. Computed tomography confirmed significant mass effect on the brain necessitating decompressive hemicraniectomy. Subdural hemorrhage caused large hypoxic lesions of the cerebral cortex and subcortical regions resulting in spastic tetraplegia, dystonia, and loss of developmental milestones. This report emphasizes that acute subdural hemorrhage may be a life-threatening complication in glutaric aciduria type I patients after minor head trauma and should be considered in those patients presenting with neurologic deterioration after accidental head injury.
Keywords: acute subdural hemorrhage; glutaric aciduria type 1; hemicraniectomy; minor accidental head trauma; tetraspasticity.
© The Author(s) 2014.