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Summary
The purpose of this study was to test the usefulness of the Unified Huntington's Disease Rating Scale (UHDRS) in clinical practice.
The UHDRS was used to examine 45 persons with genetically diagnosed Huntington's disease (HD) in various stages.
The rate of motor involvement, cognitive deficit and reliance on nursing care rose in linear proportion to HD duration. The severity of motor involvement correlated significantly with all UHDRS subscales except for that of behavioral disorders, the rate of these disorders being unrelated to any of the parameters under study. The number of CAG triplets was inversely correlated with the age at onset of HD.
Being considerably time consuming, administration of the whole UHDRS calls for interdisciplinary co-operation. For valid data acquisition, the participation of caregivers is also essential. In clinical practice it is advisable regularly to monitor the patient's conditions and the efficacy of treatment using the UHDRS motor, functional and behavioral subscales. Cognitive tests present difficulties but, in view of the progressive cognitive deterioration in HD, they are very useful in the early stage of the disease. The UHDRS does not assess impaired voluntary motor activity, or furnish information relating to therapy, dysphagia, weight loss, sexual problems or drug abuse.
KEY WORDS: behavioral disorders, CAG triplets, cognitive deficit, executive dysfunction, Huntington's disease, Unified Hungington's disease rating scale.
Introduction
Huntington's disease (HD) is an autosomal dominant inherited neurodegenerative disease. The main symptoms of HD are choreatic movements with impaired voluntary motor activity, behavioral disorders, and progressive cognitive deterioration leading to dementia.
A number of scales and batteries of tests designed to evaluate the HD patient's motor and cognitive deficits, behavioral disturbances, and daily activities are in use worldwide (1-4).
In 1996, an international Huntington's Disease Study Group (5) came forward with their Unified Huntington's Disease Rating Scale (UHDRS). This is a collection of scales, tests and questionnaires allowing comprehensive clinical rating of HD severity. The UHDRS assesses the severity of motor impairment (UHDRS-motor assessment, UHDRS-M), as well as the degree of cognitive deterioration (cognitive assessement, UHDRS-psychology UHDRS-P). Disorders of behavior and of mentation are investigated by means of the UHDRS-behavioral assessment (UHDRS-B). Questionnaires such as UHDRS-functional assessment (UHDRS-F), UHDRS-independence (UHDRS-I) and UHDRS-functional capacity (UHDRS-C) are designed to evaluate HD patients' self-care, and social...