Dystonia and chorea in acquired systemic disorders

Abstract

Dystonia and chorea are uncommon accompaniments, but sometimes the presenting features of certain acquired systemic disorders that presumably alter basal ganglia function. Hypoxia-ischaemia may injure the basal ganglia through hypoperfusion of subcortical vascular watershed regions and by altering striatal neurotransmitter systems. Toxins interfere with striatal mitochondrial function, resulting in cellular hypoxia. Infections may affect the basal ganglia by causing vasculitic ischaemia, through the development of antibodies to basal ganglia epitopes, by direct invasion of the basal ganglia by the organism, or through cytotoxins causing neuronal injury. Autoimmune disorders alter striatal function by causing a vasculopathy, by direct reaction of antibodies with basal ganglia epitopes, or by stimulating the generation of a cytotoxic or inflammatory reaction. Endocrine and electrolyte abnormalities influence neurotransmitter balance or affect ion channel function and signalling in the basal ganglia. In general, the production of chorea involves dysfunction of the indirect pathway from the caudate and putamen to the internal globus pallidus, whereas dystonia is generated by dysfunction of the direct pathway. The time of the onset of the movement disorder relative to the primary disease process, and course vary with the age of the patient and the underlying pathology. Treatment of dystonia or chorea associated with a systemic medical disorder must initially consider the systemic disorder.