Movement disorders that are infrequently seen in clinical practice can be difficult to recognize and accurately diagnose. Familiarity with these disorders can help the clinician distinguish them from more common movement disorders, such as Parkinson disease, which is associated with a significantly different prognosis and treatment approach. Rare disorders with predominate parkinsonism include multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration, whereas Huntington disease is the primary movement disorder characterized by chorea. This chapter will provide an overview of the diagnostic, pathologic, and imaging advances from recent literature as well as discuss current treatments for these rare movement disorders.
Relationship Disclosure: Dr Poston has nothing to disclose.
Unlabeled Use of Products/Investigational Use Disclosure: Dr Poston discusses the unlabeled use of olanzapine for the treatment of behavioral symptoms in Huntington disease chorea; fludrocortisone acetate for the treatment of orthostatic hypotension in multiple system atrophy; clonazepam for the treatment of anxiety and myoclonus in Huntington disease and corticobasal degeneration; carbamazepine, lamotrigine, and sodium valproate for depression; and pedunculopontine nucleus deep brain stimulation in progressive supranuclear palsy and multiple system atrophy.