The present review is aimed at providing practical assistance to the clinical neurologist in reaching a diagnosis, understanding the pathogenic mechanisms of movement disorders associated with systemic diseases, and determining appropriate therapy. Infectious disease by direct effect or as an acquired autoimmune neurological disease, stroke, hypoxia-ischemia, paraneoplastic syndromes, collagen disorders, endocrine, liver and kidney diseases that may cause hypokinetic or hyperkinetic abnormal movement are considered separately. The type and evolution of abnormal movement caused by systemic disease vary with age and underlying pathology. Therapy for abnormal movements should include a primary treatment for the systemic disease.