ABSTRACT

Choreic movement was recognized as a neurological entity in the 17th century by the English physician Thomas Sydenham. A hereditary pattern of chorea was observed by Charles Waters in 1842 in families in New York state. In 1872, George Sumner Huntington was the first to publish a description of the symptoms of chorea and dementia in families in Long Island and, as a result, the disease came to bear his name. Huntington’s disease (HD) is an autosomal dominant disorder. Typically, the disease initially manifests itself with subtle psychiatric symptoms such as personality changes and depression. These can last for many years before chorea begins. Dementia then follows and death inevitably occurs 15-20 years after the onset of motor symptoms. The eventual cause of death is usually aspiration pneumonia and other complications of immobility. There is widespread death of neurons in the striatum and in the cerebral cortex, and some neurons also form cytoplasmic and intranuclear protein inclusions suggesting that their functions may be impaired. No satisfactory treatment is available. The restoration of the dysfunctional or dead neurons by transplantation of normal neurons is a potentially interesting therapeutic approach. In this review, we discuss the attempts to restore striatal function in animal models of HD by cell transplantation or augmentation of adult neurogenesis (Fig. 1). We also describe recent clinical neural grafting trials in HD.