Motor Changes in Presymptomatic Huntington Disease Gene Carriers

Abstract

These results suggest that subtle subclinical changes in motor function are present in presymptomatic individuals who have inherited the HD allele.

Confirmation of Subtle Motor Changes Among Presymptomatic Carriers of the Huntington Disease Gene

“…These findings confirm those of previous analyses 16 showing that subtle changes in motor function are present in individuals at risk for HD who are considered to be presymptomatic on results of clinical examination. In addition to abnormalities that were present even among PSGCs with an overall score of 0, we noted a trend toward more extensive physiological abnormalities as neurologic symptoms increase.…”

“…In addition to the subtle changes found on results of neurologic examination, this PSGC sample and the previously reported sample 16 demonstrated lower performance for several physiological measures of central nervous system function. In both samples, movement time, movement time with decision, and auditory reaction time were significantly slower for the PSGCs.…”

Confirmation of Subtle Motor Changes Among Presymptomatic Carriers of the Huntington Disease Gene

“…These findings confirm those of previous analyses 16 showing that subtle changes in motor function are present in individuals at risk for HD who are considered to be presymptomatic on results of clinical examination. In addition to abnormalities that were present even among PSGCs with an overall score of 0, we noted a trend toward more extensive physiological abnormalities as neurologic symptoms increase.…”

“…In addition to the subtle changes found on results of neurologic examination, this PSGC sample and the previously reported sample 16 demonstrated lower performance for several physiological measures of central nervous system function. In both samples, movement time, movement time with decision, and auditory reaction time were significantly slower for the PSGCs.…”

Objective assessment of motor slowness in Huntington's disease: Clinical correlates and 2‐year follow‐up

“…The slowness of movement initiation and execution corroborates previous findings of akinesia and bradykinesia in HD 1, 2, 5–7, 10, 17–22. That these parkinsonian features were not only observed in late stage disease (as once thought, e.g., Denny‐Brown45) but also in the earliest stages is in accordance with previous reports on motor slowness in early HD,5, 10, 13 and can be seen as an extension of the subtle disturbances of voluntary movement described in presymptomatic gene carriers 2, 5, 7, 10, 29, 31, 34–36. Together with the present and previous observations of a strong relation between motor slowness and functional disability,2–8, 10–16 these findings support the view that impaired voluntary movement rather than chorea is the core (albeit nonspecific) movement disorder in HD.…”

Reflexive and voluntary saccadic eye movements as biomarker of Huntington’s Disease

“…This data is in contrast with those of other studies. Prolonged latency and increased latency variability of volitional saccades in presymptomatic carriers was previously noted [12][13][14]. Similar results were obtained by Golding et al, who showed prolonged latency of memory-guided saccades and increased variability of saccades latency [10].…”

Confirmation of Subtle Motor Changes Among Presymptomatic Carriers of the Huntington Disease Gene

“…These findings confirm those of previous analyses 16 showing that subtle changes in motor function are present in individuals at risk for HD who are considered to be presymptomatic on results of clinical examination. In addition to abnormalities that were present even among PSGCs with an overall score of 0, we noted a trend toward more extensive physiological abnormalities as neurologic symptoms increase.…”

“…In addition to the subtle changes found on results of neurologic examination, this PSGC sample and the previously reported sample 16 demonstrated lower performance for several physiological measures of central nervous system function. In both samples, movement time, movement time with decision, and auditory reaction time were significantly slower for the PSGCs.…”

Objective assessment of motor slowness in Huntington's disease: Clinical correlates and 2‐year follow‐up

“…The slowness of movement initiation and execution corroborates previous findings of akinesia and bradykinesia in HD 1, 2, 5–7, 10, 17–22. That these parkinsonian features were not only observed in late stage disease (as once thought, e.g., Denny‐Brown45) but also in the earliest stages is in accordance with previous reports on motor slowness in early HD,5, 10, 13 and can be seen as an extension of the subtle disturbances of voluntary movement described in presymptomatic gene carriers 2, 5, 7, 10, 29, 31, 34–36. Together with the present and previous observations of a strong relation between motor slowness and functional disability,2–8, 10–16 these findings support the view that impaired voluntary movement rather than chorea is the core (albeit nonspecific) movement disorder in HD.…”

Reflexive and voluntary saccadic eye movements as biomarker of Huntington’s Disease

“…This data is in contrast with those of other studies. Prolonged latency and increased latency variability of volitional saccades in presymptomatic carriers was previously noted [12][13][14]. Similar results were obtained by Golding et al, who showed prolonged latency of memory-guided saccades and increased variability of saccades latency [10].…”

Confirmation of Subtle Motor Changes Among Presymptomatic Carriers of the Huntington Disease Gene

“…These findings confirm those of previous analyses 16 showing that subtle changes in motor function are present in individuals at risk for HD who are considered to be presymptomatic on results of clinical examination. In addition to abnormalities that were present even among PSGCs with an overall score of 0, we noted a trend toward more extensive physiological abnormalities as neurologic symptoms increase.…”

“…In addition to the subtle changes found on results of neurologic examination, this PSGC sample and the previously reported sample 16 demonstrated lower performance for several physiological measures of central nervous system function. In both samples, movement time, movement time with decision, and auditory reaction time were significantly slower for the PSGCs.…”

Objective assessment of motor slowness in Huntington's disease: Clinical correlates and 2‐year follow‐up

“…The slowness of movement initiation and execution corroborates previous findings of akinesia and bradykinesia in HD 1, 2, 5–7, 10, 17–22. That these parkinsonian features were not only observed in late stage disease (as once thought, e.g., Denny‐Brown45) but also in the earliest stages is in accordance with previous reports on motor slowness in early HD,5, 10, 13 and can be seen as an extension of the subtle disturbances of voluntary movement described in presymptomatic gene carriers 2, 5, 7, 10, 29, 31, 34–36. Together with the present and previous observations of a strong relation between motor slowness and functional disability,2–8, 10–16 these findings support the view that impaired voluntary movement rather than chorea is the core (albeit nonspecific) movement disorder in HD.…”

Reflexive and voluntary saccadic eye movements as biomarker of Huntington’s Disease

“…This data is in contrast with those of other studies. Prolonged latency and increased latency variability of volitional saccades in presymptomatic carriers was previously noted [12][13][14]. Similar results were obtained by Golding et al, who showed prolonged latency of memory-guided saccades and increased variability of saccades latency [10].…”