The Neuropsychology of Degenerative Brain Diseases

By Robert G. Knight | Go to book overview

Chapter 7
HUNTINGTON'S DISEASE

Graham is a 47-year old accountant. Two months ago he was diagnosed as having Huntington's disease. Sitting and talking with him, the casual observer might notice nothing unusual in Graham's presentation. It is only in the light of his diagnosis that his behavior and symptoms take on any significance. The involuntary choreic movements that characterize the disease are barely perceptible. He appears restless when seated with his legs constantly changing position, and his thumbs moving rhythmically even when his hands are clasped. More apparent are his chewing jaw movements, which he reports have increased in frequency in the last few months. Graham also believes that his coordination is not as good as it ought to be and that he can feel a "tremble" in his upper body. There are signs of dysfluency in his speech. He has difficulty finding and pronouncing words, and he reports that he has problems modulating the volume and tone of his voice. All these symptoms are unobtrusive and might easily be mistaken for signs of general nervousness.

It is the cognitive changes that Graham finds most noticeable. He reports that he first observed these changes nearly three years previously. A broad range of abilities have been affected. He states that he has difficulty with short-term memory, rapidly forgetting telephone numbers after he has consulted a directory and names of people to whom he has just been introduced. He finds it difficult to concentrate and tends to lose track of conversations and to forget what has just been said. He also reports having problems with language. He notes that often he "jumbles" the words in a sentence and has difficulty finding the most appropriate words to express what he wants to say. He is not overtly depressed or emotionally distressed. He reports feeling apathetic and unmotivated and remarks how this contrasts sharply with his early life, when he was busy, active, and well-organized.

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