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Saccadic eye movements in juvenile variant of Huntington disease
Affiliations- Department of Neurology, Jagiellonian University Medical College, Krakow, Poland
- Department of Neurology, Medical University of Silesia, Zabrze, Poland
- Department of Neurology, St. Adalbert Hospital, Gdansk, Poland
- Department of Neurological and Psychiatric Nursing, Medical University of Gdansk, Gdańsk, Poland
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Abstract
Huntington disease (HD) is a neurodegenerative disease leading to involuntary movements, cognitive and behavior decline. The juvenile variant of HD (JHD) manifests in people younger than 21 and is characterized by a different clinical presentation, i.e. rigidity and bradykinesia. Rapid eye movements were not extensively studied in patients with JHD. Aims of our study were to describe the saccadic eye movements in JHD patients and to find a correlation between the saccade abnormalities, severity of the disease and cognitive and behavior deterioration.
Materials and methodsWe studied 10 patients with JHD and 10 healthy subjects. Reflexive and volitional saccades were assessed with the Saccadometer Advanced. The battery of cognitive and behavior tests was performed as well.
ResultsWe found a prolonged latency, slowness and decreased velocity of reflexive and voluntary saccades and reduced amplitude of voluntary saccades. Moreover, patients with JHD executed a significantly lower number of volitional saccades and made more incorrect cued saccades than controls. We noted a significant correlation between prolonged latency of reflexive saccades with gap task and disease severity and significant inverse correlation between prolonged latency of reflexive saccades with overlap task, an increased number of incorrect saccades made on a cue and impairment in working memory.
ConclusionAbnormalities of saccade eye movements in patients with JHD were similar to those reported in patients with HD. Our findings did not confirm abnormalities previously reported in patients with early onset HD. Abnormal saccade parameters correlated also with a disease severity and cognitive deterioration.
Abstract
Huntington disease (HD) is a neurodegenerative disease leading to involuntary movements, cognitive and behavior decline. The juvenile variant of HD (JHD) manifests in people younger than 21 and is characterized by a different clinical presentation, i.e. rigidity and bradykinesia. Rapid eye movements were not extensively studied in patients with JHD. Aims of our study were to describe the saccadic eye movements in JHD patients and to find a correlation between the saccade abnormalities, severity of the disease and cognitive and behavior deterioration.
Materials and methodsWe studied 10 patients with JHD and 10 healthy subjects. Reflexive and volitional saccades were assessed with the Saccadometer Advanced. The battery of cognitive and behavior tests was performed as well.
ResultsWe found a prolonged latency, slowness and decreased velocity of reflexive and voluntary saccades and reduced amplitude of voluntary saccades. Moreover, patients with JHD executed a significantly lower number of volitional saccades and made more incorrect cued saccades than controls. We noted a significant correlation between prolonged latency of reflexive saccades with gap task and disease severity and significant inverse correlation between prolonged latency of reflexive saccades with overlap task, an increased number of incorrect saccades made on a cue and impairment in working memory.
ConclusionAbnormalities of saccade eye movements in patients with JHD were similar to those reported in patients with HD. Our findings did not confirm abnormalities previously reported in patients with early onset HD. Abnormal saccade parameters correlated also with a disease severity and cognitive deterioration.
Keywords
Huntington disease, Juvenile onset, Saccadic eye movements, Behavioral test, Neuropsychological tests
About this articleTitle
Saccadic eye movements in juvenile variant of Huntington disease
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
236-241
Page views
280
Article views/downloads
376
DOI
10.1016/j.pjnns.2014.06.003
Bibliographic record
Neurol Neurochir Pol 2014;48(4):236-241.
Keywords
Huntington disease
Juvenile onset
Saccadic eye movements
Behavioral test
Neuropsychological tests
Authors
Natalia Grabska
Monika Rudzińska
Magdalena Wójcik-Pędziwiatr
Michał Michalski
Jarosław Sławek
Andrzej Szczudlik
