Huntington’s disease is a type of neurodegenerative disorder that is primarily caused by a mutation in the HTT or huntingtin gene. While it is commonly an inherited condition, approximately ten percent of cases are the result of a new mutation. In the human body, every individual is born with two copies of each gene, one from their mother and one from their father. Interestingly, only one mutated copy of the huntingtin gene is required for an individual to develop Huntington’s disease.
This genetic characteristic means that if a person develops Huntington’s disease, each of their children has a 50% chance of inheriting the condition as well. Unfortunately, there is currently no known cure for Huntington’s disease. However, there are medications available that can help manage the symptoms associated with the condition.
Understanding Chorea in Huntington’s Disease
Chorea is a distinctive symptom of Huntington’s disease, a genetic condition that affects voluntary and involuntary movements. This article explores the nature and progression of chorea as a prominent manifestation of the disease.
Chorea presents as uncontrolled, dance-like movements that lack purpose or coordination. It is often one of the initial signs of Huntington’s disease, manifesting as involuntary facial twitches, jerky limb movements, clumsiness, and loss of coordination. Abnormal eye movements may also occur.
As the disease progresses, the muscles become increasingly rigid, leading to abnormal posture and writhing movements. In the advanced stages of Huntington’s disease, chorea becomes less common as muscle rigidity intensifies, making movement more challenging.
It is important to note that Huntington’s disease is the most common inherited cause of chorea. Understanding the nature of these involuntary movements can contribute to the diagnosis and management of the disease.
Abnormal Gait: Understanding Hyperkinetic Gait
A person diagnosed with Huntington’s disease may experience a unique gait abnormality known as hyperkinetic gait. This type of gait is characterized by jerky and involuntary movements that become more pronounced during walking.
The term “hyperkinetic” refers to an increase in movement, while “gait” simply refers to the way a person walks. People with Huntington’s disease often exhibit a wide-based gait, meaning their feet are positioned further apart than usual.
Hyperkinetic gait manifests as slow motion with involuntary flexion of the knee and raising of the leg. These movements can be erratic and are sometimes accompanied by additional involuntary movements of the upper or lower body.
Importantly, hyperkinetic gait is not exclusive to individuals with Huntington’s disease. It can also affect people with other basal ganglia disorders, which are conditions that involve the structures deep within the brain responsible for movement regulation.