Autonomic symptoms in patients and pre‐manifest mutation carriers of Huntington’s disease

Aziz, N. Ahmad; Anguelova, Galia V.; Marinus, Johan; Dijk, J. Gert van; Roos, Raymund A.C.

doi:10.1111/j.1468-1331.2010.02973.x

Cited by 116 publications

(90 citation statements)

References 25 publications

(41 reference statements)

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“…The autonomic nervous system exerts direct control over bodily states via its two major branches: the sympathetic system, associated with energy mobilization, and the parasympathetic system, whose peripheral efferent signals are conveyed chiefly via the vagus nerve and are associated with energy conservation at the whole organism level (8). Perturbations in one or both of these systems are associated with homeostatic instability and diverse pathological feeling states (1,(9)(10)(11)(12)(13)(14). Therefore, elucidating the neural mechanisms of autonomic integration represents an important biomedical research priority.…”

mentioning

confidence: 99%

Dominant hemisphere lateralization of cortical parasympathetic control as revealed by frontotemporal dementia

Guo

Sturm

Zhou

et al. 2016

Proc. Natl. Acad. Sci. U.S.A.

110

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The brain continuously influences and perceives the physiological condition of the body. Related cortical representations have been proposed to shape emotional experience and guide behavior. Although previous studies have identified brain regions recruited during autonomic processing, neurological lesion studies have yet to delineate the regions critical for maintaining autonomic outflow. Even greater controversy surrounds hemispheric lateralization along the parasympathetic–sympathetic axis. The behavioral variant of frontotemporal dementia (bvFTD), featuring progressive and often asymmetric degeneration that includes the frontoinsular and cingulate cortices, provides a unique lesion model for elucidating brain structures that control autonomic tone. Here, we show that bvFTD is associated with reduced baseline cardiac vagal tone and that this reduction correlates with left-lateralized functional and structural frontoinsular and cingulate cortex deficits and with reduced agreeableness. Our results suggest that networked brain regions in the dominant hemisphere are critical for maintaining an adaptive level of baseline parasympathetic outflow.

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mentioning

confidence: 99%

Dominant hemisphere lateralization of cortical parasympathetic control as revealed by frontotemporal dementia

Guo

Sturm

Zhou

et al. 2016

Proc. Natl. Acad. Sci. U.S.A.

110

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“…Symptoms suggestive of autonomic nervous system (ANS) dysfunction have been reported. Patients with HD experience significantly more gastrointestinal, urinary, cardiovascular and sexual problems [1]. Accordingly, clinical testing has revealed hypofunction of the ANS in mid and advanced HD patients [2][3][4].…”

Section: Introductionmentioning

confidence: 99%

Microcirculation response to local cooling in patients with Huntington’s disease

et al. 2011

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Altered autonomic nervous system (ANS) functioning in early stages of Huntington's disease (HD) has been suggested, presumably due to distorted high-order autonomic control. ANS functioning in the early stages of HD was further investigated. Laser-Doppler (LD) flux in the skin of the fingertips, heart rate (HR), HR variability, systolic and diastolic blood pressure were measured during rest and during a 6 min cooling of one hand at 15°C. Data of 15 presymptomatic gene mutation carriers (PHD), 15 early symptomatic HD patients (EHD), and two groups of 15 age- and sex-matched controls were compared. The area under the low frequency (LF) and high frequency (HF) bands of the HR variability spectrum were calculated. An augmented reduction of cutaneous LD flux was found in response to the direct cooling in the PHD group (37.5 ± 8.5% of resting value) compared to the PHD controls (67.27 ± 8.4%) (p < 0.05). In addition, the PHD group had higher (LF/(LF + HF) index of primary sympathetic modulation of the HR at rest (53.6 ± 3.3) compared to the EHD patients (39.7 ± 4.2) (p < 0.05). In the EHD group, a significantly smaller change of HR during cooling (100.26 ± 1.2%) was found compared to the EHD controls (95.9 ± 1.0%) (p < 0.05). The results are in line with the hypothesis that ANS dysfunction occurs even in PHD subjects. Further, they support the hypothesis that dysfunction of the high-order autonomic centres are involved in HD.

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“…The English and Portuguese versions of the SCOPA-AUT have been validated [2], but the Japanese version of the SCOPA-AUT has not been published nor tested for its reliability. The SCOPA-AUT can be applied to patients with various neurological disorders because it has recently been used to assess non-PD patients, such as those with multiple system atrophy (MSA) and Huntington's disease [3] [4]. Thus, we prepared the Japanese version of the SCOPA-AUT.…”

Section: Introductionmentioning

confidence: 99%

Reliability of the Japanese version of the scales for outcomes in Parkinson's disease-autonomic questionnaire

Matsushima

Yabe

Hirotani

et al. 2014

Clinical Neurology and Neurosurgery

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Methods: We translated the SCOPA-AUT from English to Japanese. Thirty-one patients with diseases involving autonomic symptoms completed the form twice. The reliability was assessed by Cronbach's coefficient alphas and intraclass correlation coefficients (ICCs).Results: The average (standard deviation, SD) total scores of the first and second assessments of the SCOPA-AUT were 15.7 (SD, 7.1) and 13.6 (SD, 6.5), respectively. The Cronbach's coefficient alphas were globally high, but the ICCs were moderately high. The valid response rates for the questions about sexual dysfunction were 36.7% in men and 26.6% in women.Conclusions: The Japanese version of the SCOPA-AUT had high internal consistency. However, the questions about sexual dysfunction showed less valid response rates.

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Autonomic symptoms in patients and pre‐manifest mutation carriers of Huntington’s disease

Abstract: Autonomic symptoms are highly prevalent in patients with HD and may even precede the onset of motor signs. Moreover, autonomic dysfunction is related to functional disability and depression in HD.

Cited by 116 publications

References 25 publications

Dominant hemisphere lateralization of cortical parasympathetic control as revealed by frontotemporal dementia

Dominant hemisphere lateralization of cortical parasympathetic control as revealed by frontotemporal dementia

Microcirculation response to local cooling in patients with Huntington’s disease

Reliability of the Japanese version of the scales for outcomes in Parkinson's disease-autonomic questionnaire

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