Vasoregulatory activity in patients with Huntington's chorea

Aminoff, M. J.; Gross, M.

doi:10.1016/0022-510x(74)90103-8

Cited by 19 publications

(11 citation statements)

References 7 publications

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“…In humans, similar studies have been conducted to test the response of the baroreceptor reflex. Results have been mixed, however recent studies report deficits during the Valsalva maneuver, hand-grip test, and the head up tilt test [4] [7] [31] [32]. Furthermore, patients complain of dizziness and light-headedness upon standing, which are symptoms of baroreceptor dysregulation resulting in orthostatic hypotension [6] [8].…”

Section: Discussionmentioning

confidence: 99%

Baroreceptor reflex dysfunction in the BACHD mouse model of Huntington’s disease.

et al. 2011

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Huntington’s disease is a progressive, neurodegenerative disorder that presents with a triad of clinical symptoms, which include movement abnormalities, emotional disturbance and cognitive impairment. Recent studies reported dysfunction of the autonomic nervous system in Huntington’s disease patients, which may contribute to the increased incidence of cardiovascular events in this patient population that often leads to death. We measured the baroreceptor reflex, a process dependent on proper autonomic function, in the BACHD mouse model of Huntington’s disease. We found a blunted response of the baroreceptor reflex as well as significantly higher daytime blood pressure in BACHD mice compared to WT controls, which are both indications of autonomic dysfunction. BACHD mice had increased heart weight to tibia length ratios at 7 and 12 mo of age suggesting hypertrophic changes of the heart, which we speculate is a response to the increased blood pressure and aberrant baroreceptor reflex. Despite these structural changes, the hearts of BACHD mice continue to function normally as assessed by echocardiographic analysis. Studies of autonomic and cardiovascular function in BACHD mice may help elucidate the pathophysiology of Huntington’s disease and aid in the development of clinical strategies to offset the incidence of fatal cardiovascular events in the Huntington’s disease patient population.

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Section: Discussionmentioning

confidence: 99%

Baroreceptor reflex dysfunction in the BACHD mouse model of Huntington’s disease.

et al. 2011

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“…Autonomic symptoms such as hyperhydrosis, micturition and swallowing difficulties [9–11], sexual dysfunction [12], as well as complaints suggestive of orthostatic intolerance [13,14], have been reported in small groups of patients with HD. Moreover, although autonomic symptoms are thought to be most prominent in the advanced stages of the disease [15,16], complaints of possible autonomic origin such as dizziness after standing up, excessive perspiration, and tachycardia have also been described in mildly disabled HD patients and even in otherwise asymptomatic mutation carriers [5].…”

Section: Introductionmentioning

confidence: 99%

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

Aziz

Anguelova

Marinus

et al. 2010

Euro J of Neurology

117

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“…1 Only scant and inconsistent data have as yet been reported on whether HD correlates with disruptions in the function of the autonomic nervous system (ANS). [2][3][4][5] Both the presence 5 and absence 4 of parasympathetic dysfunction have been postulated in patients with HD. With regard to the sympathetic nervous system, defects in the central autonomic network-for example, the hypothalamus and its connection to the neocortex, the limbic system, brainstem, and spinal cord-have consistently been postulated.…”

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confidence: 99%

Autonomic nervous system function in Huntington's disease

Andrich

2002

Journal of Neurology, Neurosurgery &Amp; Psychiatry

104

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Objective: To investigate whether Huntington's disease (HD) affects autonomic nervous system (ANS) functioning. Methods: Twenty patients with HD who had positive genetic test results underwent standardised ANS function tests including sympathetic skin responses (SSRs) of the hands and feet, measurements of heart rate variability (HRV), both during five minutes of resting and deep respiration, and an orthostatic blood pressure test. Patients were classified according to the motor subscale of the unified Huntington's disease rating scale (UHDRS; mean (SD) score 26.4 (13.6)) and divided into two subgroups: UHDRS <25 points (early stages, E-HD) and UHDRS >25 points (mid stages, M-HD). Autonomic indices were compared with those obtained for a group of well matched healthy controls (n=60). Results: Overall, patients showed lower HRV indices than controls. Multivariate analysis with the independent factor of "group" (controls, E-HD, M-HD) showed a significant group effect on both the high frequency power (F=4.32, p=0.017) and the coefficient of variation (F=4.23, p=0.018), indicating a significant reduction in vagal modulation in the M-HD group. There was a shift in autonomic neurocardiac balance towards sympathetic predominance in the M-HD group compared with controls (F=2.89, p=0.062). Moreover, we found an inverse correlation between the severity of clinical HD symptoms (assessed by the UHDRS) and the modulation of cardiovagal activity (p=0.028). Vagal dysregulation was present in two patients; one of them also showed a pathological blood pressure test and a latency prolongation in the SSRs of the hands. Two other patients had pathologically reduced SSR amplitudes. Only patients of the M-HD group were affected. Conclusion: Autonomic dysfunction is present even in the middle stages of HD and affects both the sympathetic and parasympathetic branch of the ANS.

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Vasoregulatory activity in patients with Huntington's chorea

Cited by 19 publications

References 7 publications

Baroreceptor reflex dysfunction in the BACHD mouse model of Huntington’s disease.

Baroreceptor reflex dysfunction in the BACHD mouse model of Huntington’s disease.

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

Autonomic nervous system function in Huntington's disease

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