A clinical classification acknowledging XXXX in @entity2033 .

@entity1 @entity146 @entity2358 @entity73 @entity2033
BACKGROUND: @entity2358 , @entity146 , and @entity73 are all part of the symptom triad in @entity2033 ( @entity2033 ). Despite the fact that @entity146 and @entity73 may be early manifestations of @entity2033 , the clinical diagnosis is conventionally based on the presence of @entity2358 and a positive genetic test for the @entity2033 CAG repeat expansion. After investigating the frequencies of the triad manifestations in a large outpatient clinical cohort of @entity2033 gene-expansion carriers, we propose a new clinical classification. METHODS: In this cross-sectional study, 107 gene-expansion carriers from a Danish outpatient clinic were recruited. All @entity1 underwent neurological examination, @entity146 evaluation and neuropsychological testing. @entity1 were categorised according to motor symptoms, @entity146 , the use of psychotropic medication, and @entity73 . RESULTS: Among the motor manifest @entity2033 gene-expansion carriers, 51.8% presented with the full symptom triad, 25.0% were defined as @entity73 in addition to motor symptoms, and 14.3% had @entity146 along with motor symptoms. Only 8.9% had isolated motor symptoms. Among gene-expansion carriers without motor symptoms, 39.2% had @entity146 , were @entity73 , or had a combination of the two. CONCLUSION: This is the first study to report the frequencies of both motor symptoms, @entity73 , and @entity146 in @entity2033 gene-expansion carriers in a national outpatient @entity2033 clinical cohort. We found that almost 40% of the gene-expansion carriers without motor symptoms had either @entity146 , @entity73 or both, emphasising that these @entity1 are not premanifest in @entity146 and cognitive terms, suggesting that the current clinical classification is neither necessarily suitable nor helpful for this @entity1 group. Some premanifest gene-expansion carriers may have @entity146 and/or cognitive symptoms caused by reactive stress or other pathology than @entity2033 . Acknowledging this fact we, however, suggest classifying all @entity2033 gene-expansion carriers into three clinical categories: premanifest, non-motor manifest, and motor manifest.
