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Predictors of diagnosis in Huntington disease

From the Departments of Psychiatry (D.R.L., J.S.P.), Biostatistics (D.R.L.), Neurology (J.S.P.), and Psychology (J.S.P.), University of Iowa, Iowa City.

Address correspondence and reprint requests to Dr. Jane S. Paulsen, University of Iowa, Roy J. and Lucille A. Carver College of Medicine, Department of Psychiatry, 1-305 MEB, Iowa City, IA 52242-1000 jane-paulsen{at}uiowa.edu

Objective: Subtle signs and symptoms of Huntington disease (HD) are often present before impairments reach a point where the neurologic disease is manifest and a diagnosis must be considered. The objective is to examine the prognostic significance of these early clinical signs and symptoms regarding time until unequivocal clinical HD diagnosis.

Methods: We analyzed longitudinal data from 218 at-risk but healthy participants in the Huntington Study Group database who had either normal motor examination results or minimal soft motor signs at first observation. This group was followed periodically in HD clinics for up to 4.5 years. We used survival analysis to examine predictors of time until HD diagnosis.

Results: Diagnostic prediction was significantly improved using specific, nonredundant items from the Unified Huntington's Disease Rating Scale. When a movement disorder specialist initially had a global impression of "soft signs" present, cumulative relative risk of diagnosis was 4.68 times greater at 1.5 years of follow-up and 3.58 at 3 years. A neuropsychological test pattern with psychomotor speed 1 SD worse than a semantic knowledge measure increased cumulative risk by 1.99 times at 1.5 years and 1.81 at 3 years. Finally, reports of various subjective HD symptoms increased 3-year relative risk by 2.6 to 3.4.

Conclusions: Findings demonstrate that neuropsychological performance and both the clinician rating and the patient subjective perception of motor difficulties contribute nonredundantly to a prediction of Huntington disease diagnosis. These findings may have implications for prognostic assessment of persons at risk and eventually assist with early interventions.

*See the appendix for a list of members of the Huntington Study Group.

Supported by National Institute of Neurological Disorders and Stroke grant no. 40068, the National Institutes of Mental Health grant no. 01579, the Roy J. and Lucille Carver Trust, the Howard Hughes Medical Institute, and the High Q Foundation grants to Jane S. Paulsen; and the Huntington's Disease Society of America, the Huntington's Society of Canada, the Hereditary Disease Foundation, and the High Q Foundation grants to the Huntington Study Group.

Disclosure: The authors report no conflicts of interest.

Received September 16, 2004. Accepted in final form January 11, 2007.