Huntington's Disease and Dementia

  • Huntington's Disease (HD) is a progressive brain disorder caused by a defective gene.
  • This disease causes changes in the central area of the brain, which affect movement, mood and thinking skills.
  • Symptoms of Huntington's disease usually develop between ages 30 and 50, but they can appear as early as age 2 or as late as 80.

Signs & Symptoms:

  • Uncontrolled movements of the arms, legs, head, face and upper body
  • Huntington's disease also causes a decline in thinking and reasoning skills, including memory, concentration, judgment and ability to plan and organize.
  • Huntington's disease brain changes lead to alterations in mood, especially depression, anxiety, and uncharacteristic anger and irritability.
  • Another common symptom is obsessive-compulsive behavior, leading a person to repeat the same question or activity over and over.
  • Death most often results from dysphagia through aspiration pneumonia or suffocation, usually between 10 and 20 years after the onset of symptoms.

Tips for First Responders:

  • Turn off lights and sirens as soon as possible
  • Speak slowly and clearly
  • Use "adult" words, but ask questions one at a time, rather than asking multiple part questions
  • Ask the individual if s/he wears a medic alert device (this may tell you where they live)
  • Maintain a calm manner; this may help the individual to calm down if s/he is anxious

Source: Kalathur, R. K. R., Hernandez-Prieto, M. A., & Futschik, M. E. (2012). "Huntington's Disease and its therapeutic target genes: a global functional profile based on the HD Research Crossroads database, BMC Neurology. 12(1), 47-67.