Case history
Case history
A 42-year-old school teacher presents with difficulty managing her classroom. She has become increasingly irritable with students and fails to complete assigned tasks on time. Her sister and husband report that she has become restless, pays less attention to her appearance and social obligations, and at times is anxious and upset. She has stumbled unexpectedly. Her symptoms resemble those of her mother when she was diagnosed with Huntington disease. On examination, her speech is somewhat uneven and she is inappropriately flippant. Subtracting serial 7s from 100, while seated with her eyes closed, brings out random "piano-playing" movements of the digits along with other movements of the limbs, torso, and face. Subtraction errors occur with this task. She is unable to keep her tongue fully protruded for 10 seconds. Finger tapping is slower than the examiner's, and tapping tempo is uneven. Tandem walking is impaired.
Other presentations
In children or young adults, Huntington disease may present as a decline in performance at school or work, associated with rigidity and incoordination but little or no chorea.[2] Generalized slowing and awkwardness to movement may be noted, and ocular motility examination often reveals slowed saccadic eye movements. Children with Huntington disease are more likely to inherit the disorder from an affected father because the pathogenetic trinucleotide repeat is less stable in spermatogenesis than oogenesis, making it more susceptible to expansion. Sometimes, because of divorce or denial, the family history of Huntington disease is not known, making the disorder particularly difficult to diagnose.
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