Huntington's disease (HD) is a devastating neurodegenerative disorder that 
occurs in patients with a mutation in the huntingtin or IT15 gene. Patients are 
plagued by early cognitive signs, motor deficits, and psychiatric disturbances. 
Symptoms are attributed to cell death in the striatum and disruption of 
cortical-striatal circuitry. Mechanisms of cell death are unclear, but processes 
involving mitochondrial abnormalities, excitotoxicity, and abnormal protein 
degradation have been implicated. Many factors likely contribute to neuron death 
and dysfunction, and this has made it difficult to systematically address the 
pathology in HD. Pharmaceutical therapies are commonly used in patients to treat 
disease symptoms. These have limited benefit and do not address the inexorable 
disease progression. Several neuroprotective therapies are being evaluated in 
animal models of HD as well as in clinical trials. Similarly, cell replacement 
strategies such as fetal transplantation have been used in the clinic with 
minimal success, making future cell replacement strategies such as stem cell 
therapy uncertain. This review describes the disease pathology in HD and 
addresses many of the past and emerging therapeutic strategies.