Atypical onset of symptoms in Huntington disease: severe cognitive decline preceding chorea or other motor manifestations.
OBJECTIVE
To describe the onset of Huntington disease (HD) in a patient with atypical progression of symptoms.


BACKGROUND
The authors report the case of a 39-year-old man with severe cognitive impairment and diffuse cortical atrophy before the onset of motor manifestations or symptoms of an extrapyramidal movement disorder.


METHODS
Clinical examinations, neuropsychologic assessments, magnetic resonance imaging, electroencephalogram, and genetic testing were conducted.


CONCLUSIONS
Although HD was eventually confirmed through genetic testing, chorea was not part of the clinical picture until well after the patient had developed a frank dementia, with a decline in global intellectual functioning, memory deficits, slowed information processing speed, and executive dysfunction. This case indicates HD may present with atypical clinical features in the early course of the disease, and warrants diagnostic consideration in patients with early dementia of unknown etiology.
Cooper
Douglas B
DB
Department of Behavioral Medicine, Neuropsychology Service, Brooke Army Medical Center, Fort Sam Houston, TX 78234-6200, USA. douglas.cooper2@amedd.army.mil
Ales
Gregory
G
Lange
Christopher
C
Clement
Pamelia
P
D001284:Atrophy; D002540:Cerebral Cortex / Q000473:pathology*; D003072:Cognition Disorders / Q000175:diagnosis / Q000209:etiology*; D018450:Disease Progression; D006801:Humans; D006816:Huntington Disease / Q000150:complications / Q000175:diagnosis* / Q000523:psychology; D008297:Male