1962
DOI: 10.1001/archneur.1962.04210040027003
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Rigid and Akinetic Forms of Huntington's Chorea

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Cited by 99 publications
(37 citation statements)
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“…The motor nerves to these two types of muscle fibres can be distinguished from one another by their diameter (Pilar and Hess, 1966), as well as by their physiological properties (Bach-y-Rita and Ito, 1966), but the central nervous system structures controlling these two neuromuscular systems are not known. The loss of saccades in the patients with Huntington's chorea presented in this study and previous description of impaired or slowed conjugate gaze in this same illness (Bender, 1960;Bittenbender and Quadfasel, 1962) raises the possibility that the caudate nucleus which is predominantly affected in Huntington's chorea may be intimately related to the neural system regulating rapid eye movements. This interpretation receives indirect support from animal experiments in which "wide and vacuous eyes" with a "paucity of eye movements" were the consequence of bilateral caudate lesions (Mettler, 1964), while stimulation of the head of the caudate evoked orienting eye movements to the opposite side (Laursen, 1963).…”
Section: Discussionsupporting
confidence: 72%
See 1 more Smart Citation
“…The motor nerves to these two types of muscle fibres can be distinguished from one another by their diameter (Pilar and Hess, 1966), as well as by their physiological properties (Bach-y-Rita and Ito, 1966), but the central nervous system structures controlling these two neuromuscular systems are not known. The loss of saccades in the patients with Huntington's chorea presented in this study and previous description of impaired or slowed conjugate gaze in this same illness (Bender, 1960;Bittenbender and Quadfasel, 1962) raises the possibility that the caudate nucleus which is predominantly affected in Huntington's chorea may be intimately related to the neural system regulating rapid eye movements. This interpretation receives indirect support from animal experiments in which "wide and vacuous eyes" with a "paucity of eye movements" were the consequence of bilateral caudate lesions (Mettler, 1964), while stimulation of the head of the caudate evoked orienting eye movements to the opposite side (Laursen, 1963).…”
Section: Discussionsupporting
confidence: 72%
“…The difference between the six patients with normal eye movements and the three patients with an absence of saccades might be due to biotypes among individuals with Huntington's chorea, based on genetically determined variations in the central nervous system lesions. Such a concept has been proposed as the basis for the rigid and hypotonic forms of Huntington's chorea (Bittenbender and Quadfasel, 1962). Alternatively, since the motor manifestations of Huntington's chorea change during the course of the illness (Denny-Brown, 1962), the disorder of rapid eye movements may not yet have become manifest in these six patients and should become evident on subsequent examinations.…”
Section: Discussionmentioning
confidence: 99%
“…The resulting polyglutamine (polyQ) expansion in the 350 kDa cytosolic protein huntingtin (Htt) is associated with typical adult onset disease for repeat lengths of 40 -55, whereas more extreme expansions cause juvenile onset and a distinct clinical presentation (Bittenbender and Quadfasel, 1962;van Dijk et al, 1986;Brinkman et al, 1997). Neuronal loss is most severe among the medium-sized spiny neurons (MSNs) of the neostriatum (Vonsattel et al, 1985), whereas Htt expression is widespread (Strong et al, 1993).…”
Section: Introductionmentioning
confidence: 99%
“…These involuntary movements are seen first in the fingers and toes, then in the trunk. Approximately 10% of all patients with HD may, however, have the juvenile onset or Westphal variant of HD with symptoms of hypokinesia and rigidity similar to Parkinson's disease (Bittenbender & Quadfasel, 1962;Bruyn, 1962). Difficulties with balance occur, with exaggerated, fidgeting motor action and a tendency to violent involuntary movements.…”
Section: Motor Symptomsmentioning
confidence: 99%