Evoked potentials in patients with Huntington's disease and their offspring. I. Somatosensory evoked potentials

A patient with Huntington’s disease developed acute dystonia whilst treated with tiapride. Sulpiride and tetrabenazine also induced dystonia. The anticholinergic biperiden depressed the syndrome but worsened psycho-pathology. Finally a combination of tetrabenazine and clozapine was successful in treatment of both chorea and dystonia. According to this observation, acute dystonia may occur in Huntington’s disease as a consequence of neuroleptic treatment.

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“…The results of the CAT scan and evoked potentials were in good agreement with the diag nosis of HD [1][2][3].…”

Section: Discussionsupporting

confidence: 66%

Neuroleptically Induced Dystonia in Huntington’s Disease: A Case Report

Schott

Ried²,

Stevens³

et al. 1989

Eur Neurol

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“…In earlier SEP studies, reduction in size of the contralateral parietal NZdP25 response to median nerve stimulation has been described as the prominent feature in patients with manifest HD (21,41,42), while changes in latencies of the cortical responses were negligible. The decrease in the SEP amplitude can already be detected in subjects at risk for HD without any choreatic movements (41).…”

Section: Discussionmentioning

confidence: 95%

“…The decrease in the SEP amplitude can already be detected in subjects at risk for HD without any choreatic movements (41). Using a noncephalic reference instead of the commonly applied frontal reference, it is possible to discriminate between various parietal and frontal cortical generators in healthy human subjects (20).…”

Section: Discussionmentioning

confidence: 99%

Comparison of somatosensory evoked potentials with striatal glucose consumption measured by positron emission tomography in the early diagnosis of huntington's disease

et al. 1993

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Both somatosensory evoked potentials (SEP) and striatal glucose consumption (rCMRGlc) measured by positron emission tomography (PET) have been reported to be abnormal early in the course of Huntington's disease (HD). To compare their diagnostic value, SEP and rCMRGlc were measured in a group of 18 first degree off-spring of HD families: 6 had manifest HD with chorea and the remaining 12 individuals were chorea-free subjects at risk for HD. In five patients with manifest disease, both SEP and striatal rCMRGlc were significantly abnormal, defined in SEP as having either a bilaterally absent frontal N30 amplitude or a reduction of the parietal N20/P25 amplitude below 1 microV on at least one side; in PET as exhibiting a reduction of the cerebellar ratio (CR) of both caudate and lentiform rCMRGlc below the 99% confidence limits of these variables determined in 20 normal volunteers. The remaining patient with manifest HD had questionably abnormal SEP and significantly reduced indices of striatal rCMRGlc. The five persons at risk for HD who had normal SEP also had normal striatal rCMRGlc; those three at-risk patients with abnormal SEP also had a reduction of the CR of both caudate and lentiform rCMRGlc. Of the remaining four individuals at risk for HD who had questionably abnormal SEP, three had CR values of striatal rCMRGlc in the normal range and one a reduction of the CR of lentiform rCMRGlc. In at-risk patients, the SEP diagnosis correlated significantly with caudate (r = -0.8; p < 0.002) and lentiform (r = -0.76; p < 0.005) rCMRGlc. These data indicate a parallel deterioration of SEP and striatal rCMRGlc early in the course of HD even before the development of chorea.

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“…Alternatively, a reduced efficiency of sensorimotor processing may induce an increased force specification. Disturbed sensorimotor activity in HD has been suggested because of a total suppression or reduced activity of the reactive EMG response (Fellows et al 1997;Noth et al 1985), an abnormal (sub)cortical activation during passive sensory stimulation (Boecker et al 1999), changes in somatosensory evoked potentials (Noth et al 1984;Töpper et al 1993) and deficits in feedback control when external perturbations are introduced during reaching movements (Smith et al 2000).…”

Section: Discussionmentioning

confidence: 99%

Control of manipulative forces during unimanual and bimanual tasks in patients with Huntington’s disease

2002

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The aim of the study was to investigate gripload force regulation in Huntington's disease (HD) patients as compared to control subjects during the performance of a manipulative task that required rhythmical unimanual or bimanual isodirectional/non-isodirectional actions in the sagittal plane. Results showed that the profile of grip-load ratio force was characterized by maxima and minima that were attained at upward and downward hand positions, respectively. Minimum force ratio was higher in patients than in controls, which points to an elevated baseline that may be related to the inherent bradykinesia observed in HD. Maximum force ratio was also increased in patients, but this effect depended on the performance condition, with largest amplifications occurring during non-isodirectional movements. The latter rescaling may be associated with the complexity of the coordination mode and its asymmetrical load characteristics. In addition, the temporal delay between the grip and load force peaks was augmented in patients versus controls, indicating a disturbed coupled activation of both forces. Furthermore, the interval was largest during nonisodirectional movements followed by isodirectional and unimanual movements, which denotes that the grip-load force coupling deteriorated as a function of coordinative complexity. Together, these data indicate a deficit in the grip-load force constraint due to HD and illustrate the degrading effect of striatal dysfunction on (bi)manual manipulative function.

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Evoked potentials in patients with Huntington's disease and their offspring. I. Somatosensory evoked potentials

Cited by 82 publications

References 18 publications

Neuroleptically Induced Dystonia in Huntington’s Disease: A Case Report

Neuroleptically Induced Dystonia in Huntington’s Disease: A Case Report

Comparison of somatosensory evoked potentials with striatal glucose consumption measured by positron emission tomography in the early diagnosis of huntington's disease

Control of manipulative forces during unimanual and bimanual tasks in patients with Huntington’s disease

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