Autonomic Nervous Function in Huntington's Disease

Heijer, J. C. Den; Bollen, Ward L. E. M.; Reulen, J. P. H.; Dijk, J. Gert van; Kramer, C. G. S.; Roos, Raymund A.C.; Buruma, O. J. S.

doi:10.1001/archneur.1988.00520270087025

Cited by 48 publications

(37 citation statements)

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“…In advanced HD patients clinical testing of autonomic function has revealed a hypofunction of the parasympathetic and sympathetic parts of the ANS [2,3]. In addition, autonomic dysfunction in mid-stage patients has been found [4].…”

Section: Discussionmentioning

confidence: 99%

“…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]. Moreover, there are signs of ANS dysfunction even in early symptomatic HD patients (EHD) and presymptomatic HD gene mutation carriers (PHD).…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2011

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“…2) is the response of the pupil to a bright flash of light, involving rapid contraction followed by dilation back to original size. This reflex has been used as a neurological screening tool for disorders such as Parkinson's disease, Huntington's disease, schizophrenia, multiple sclerosis and trauma [95][96][97][98]. Since it is mainly a parasympathetic cholinergic response [99], the pupil light reflex could also possibly be affected if central cholinergic depletion in AD extends to the parasympathetic oculomotor system.…”

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confidence: 99%

Ocular Biomarkers for Early Detection of Alzheimer's Disease

Frost

Martins

Kanagasingam

2010

JAD

100

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Alzheimer's disease (AD) is the most common form of dementia and is characterized clinically by a progressive decline in memory, learning and executive function and neuropathologically by the presence of cerebral amyloid deposits. Despite a century of research, there is still no cure or conclusive pre-mortem diagnosis for the disease. A number of symptom-modifying drugs for AD have been developed, but their efficacy is minimal and short-lived. AD cognitive symptoms arise only after significant, irreversible neural deterioration has occurred, hence there is an urgent need to detect AD early, before the onset of cognitive symptoms. An accurate, early diagnostic test for AD would enable current and future treatments to be more effective, as well as contribute to the development of new treatments.While most AD related pathology occurs in the brain, the disease has also been reported to affect the eye, which is more accessible for imaging than the brain. AD-related proteins exist in the normal human eye and may produce ocular pathology in AD. There is some homology between the retinal and cerebral vasculatures and the retina also contains nerve cells and fibers that form a sensory extension of the brain. The eye is the only place in the body where vasculature or neural tissue is available for non-invasive optical imaging. This article presents a review of current literature on ocular morphology in AD and discusses the potential for an ocular based screening test for AD.

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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%

“…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. 3 5 However, considerable discrepancies in the results of autonomic tests were evident despite this apparent consistency; as an example, normal sympathetic skin response (SSR) findings were produced in one study, 3 whereas in another there were prolongations in latency, amplitude reductions, and even lacking potentials. 5 These sometimes highly divergent findings may be related to the considerable methodological differences between the studies quoted.…”

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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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Autonomic Nervous Function in Huntington's Disease

Cited by 48 publications

References 16 publications

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

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

Ocular Biomarkers for Early Detection of Alzheimer's Disease

Autonomic nervous system function in Huntington's disease

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