Autonomic nervous function in Huntington's disease.

Autonomic nervous system function was studied by means of a series of standardized tests in 11 patients with Huntington's disease (HD) and in 11 age- and sex-matched controls. Two statistically significant differences were found. The blood pressure response to sustained handgrip was diminished and the pupillary light reflex latency was increased in the HD group. The former probably reflects diminished input from higher centers on an otherwise intact sympathetic nervous system. The latter may indicate involvement of the pretectal or Edinger-Westphal nuclei in HD. No further signs of autonomic nervous system dysfunction were found.

Respiration during sleep in Huntington's chorea.

In view of recent reports on lower brainstem dysfunction in Huntington's chorea, we studied respiration during sleep in 12 patients with Huntington's chorea (HC) and in controls. There were no statistically significant differences between patients and controls with respect to apnea periods, respiratory frequency and time elapsed between minimal and maximal value of the respiratory curve. No statistically significant differences in respiratory variability were observed between patients and controls. In the present study, no indication was found for dysfunction of lower brainstem structures involved in respiration in HC.

Non-invasive assessment of autonomic nervous function in Gilles de la Tourette syndrome.

Autonomic nervous function was investigated in 18 Gilles de la Tourette (GTS) patients and in 23 controls, who matched the patients in age, sex, baseline blood pressure and baseline heart rate. Four heart rate tests were used (variation at rest, during deep breathing, following standing up and during a Valsalva manoeuvre), and two blood pressure tests (standing up and sustained handgrip). The only significant difference between the groups was found in the Valsalva test. This was due to the initial heart rate increase which was higher in the GTS group; the subsequent decrease did not differ between the groups. Increased sympathetic activity is a possible explanation for this finding, but no additional evidence in its favour was found. No signs of autonomic failure were found.

Nerve conduction velocity to different muscles in peroneal pressure neuropathy.

The variability inherent in nerve conduction velocity (NCV) studies suggests that multiple measurements may yield more information than a single measurement. To test this hypothesis, three motor nerve conduction velocities (NCVs) of the peroneal nerve were measured: to the extensor digitorum brevis muscle over the fibular head and in the lower leg, and to the tibialis anterior muscle over the fibular head. Three groups were studied: controls, polyneuropathy cases and peroneal pressure neuropathy cases. Over the fibular head, NCVs recorded from the tibialis anterior were consistently higher than those from the extensor digitorum brevis, and showed a higher number of abnormalities. Differences between NCVs, their Z-scores and Mahalanobis distances were compared to study the influence of variance differences and covariances. The best compromise between true and false positives was found to be a comparison between NCVs over the fibular head recorded from the tibialis anterior and the leg segment recorded from the extensor digitorum muscle. Z-scores are more useful than Mahalanobis distances in this respect.

Autonomic nervous system tests depend on resting heart rate and blood pressure.

In order to study the effects of baseline blood pressure and heart frequency on autonomic function tests, 75 normal subjects (aged 8-96 years) were investigated. Heart rate variability was studied at rest, during deep breathing, following standing up and during a Valsalva manoeuvre. Blood pressure changes were recorded during standing up and during sustained handgrip. Responses were described as ratios and as differences to study the efficacy of both methods. Multiple regression analysis showed that significant relationships with the resting heart rate existed for ratios but not for differences. The blood pressure rise in the sustained handgrip test showed a significant relationship with resting blood pressure regardless of the description method. As expected, relationships with age existed for all four heart rate tests regardless of the description method. The blood pressure responses were not significantly related to age. We advise that heart rate test results should be presented as differences, as this avoids the need for correction for the resting heart rate. Correction for the resting blood pressure improves the accuracy of the standing up blood pressure test. Correction for age remains necessary for heart rate tests but not for blood pressure tests.

Assessment of autonomic function in myotonic dystrophy.

Published reports suggested autonomic nervous system dysfunction in myotonic dystrophy but were inconclusive partly due to small patient numbers and because only a limited number of tests was used. Autonomic nervous system function with noninvasive tests was assessed in ten myotonic dystrophy patients and age and sex matched healthy controls. Statistically significant differences included reduction in the heart rate response to standing and in the blood pressure response to sustained handgrip. Latency of the pupillary light reflex was not different from controls but the time to reach peak velocity of contraction was significantly longer in the myotonic dystrophy group. It is argued that these results reflect dysfunction of voluntary and iris smooth muscle rather than autonomic nervous system dysfunction.