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Huntington’s Disease and its Effect on Speech

Huntington's Disease is a neurodegenerative disorder characterised by the gradual degeneration or death of neurons in the brain. It affects people both mentally and physically, with motor control and cognitive ability both gradually weakening over time. Changes in behaviour and personality also occur, as a result of both the changes to the brain and the psychological effects of living with the condition.

Cause

As it is a genetic condition, the child of a person with Huntington's Disease has a 50% chance of inheriting it. Research from Oxford University suggests that a child of two parents with Huntington's Disease also has a 50% chance of inheriting it, although the rate at which the disease progresses will be accelerated. Only about 10% of cases become evident before the age of 20, with the majority of people first noticing symptoms between 30 and 50 years of age. Both diagnostic & predictive tests are available to those who are concerned that they may have inherited the condition.  

Symptoms

As the condition causes the gradual degeneration of the brain, it affects a person's speech in both mental and physical ways. Involuntary spams, difficulty moving due to loss of control, and stiffness are all ways in which the muscles will be affected. Cognitively speaking, Huntington's Disease will affect a person's ability to focus, absorb new information, process thoughts, and show self-restraint. All of these symptoms combined impact a person's ability to speak, which can then lead to further problems, such as depression or social withdrawal.

Effects on Speech

Over 100 muscles are used to create speech, meaning that Huntington's Disease has a severe impact on a person's ability to communicate even from a purely physical standpoint, although speech is affected in two ways. The first is known as Dysarthria, which describes when the brain sends out signals but the muscles are too weak or uncontrollable to react properly, resulting in slurred, unclear speech. The second is apraxia, where the damage dealt directly to the brain limits its abilities to send out the signals at all. Other consequences include difficulty breathing and speaking at the same time, difficulty finding the desired words, and difficulty controlling the speed and volume of speech.

Dysphagia, or difficulty swallowing, is also a result of Huntington's Disease. This can have very negative consequences, as people tend to eat less, which makes their muscles weaken and causes the disease to progress faster. Diet and weight management are extremely important parts of living with the condition.

Speech and Language Therapy

Speech & language therapy plays a crucial role in slowing down the degenerative effects of Huntington's Disease on the muscles. Due to the fact that the condition can affect speech in so many ways, the course of treatment will not only vary considerably from patient to patient, but also develop as the condition progresses. One of the primary goals of SLT is to identify the muscles that are worst affected, and exercise them regularly to keep them as strong as possible.

In addition to exercising the muscles required to speak and swallow, SLT also teaches people how to optimise their speaking ability. This can be done in a number of different ways, such as speaking more slowly and loudly, using shorter words and sentences, exaggerating certain sounds, using gestures, and learning breathing techniques.  

Because Huntington's Disease affects both a person's ability to speak and their ability to register what others are saying, SLT can also be useful for a patient's friends and family members. There are many ways that people can help ensure that a patient remains included in conversation, does not become isolated, and understands what is being said clearly. These include sticking to familiar topics, using short, simple, unambiguous sentences, asking yes/no questions, using gestures, speaking more slowly, and eliminating distractions such as music. Although there is no cure for Huntington's Disease, SLT can help to slow its progression and ensure that the patient leads as normal as life as possible.