open access

Vol 48, No 4 (2014)
Original research articles
Submitted: 2014-03-15
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Saccadic eye movements in juvenile variant of Huntington disease

Natalia Grabska, Monika Rudzińska, Magdalena Wójcik-Pędziwiatr, Michał Michalski, Jarosław Sławek, Andrzej Szczudlik
DOI: 10.1016/j.pjnns.2014.06.003
·
Neurol Neurochir Pol 2014;48(4):236-241.

open access

Vol 48, No 4 (2014)
Original research articles
Submitted: 2014-03-15

Abstract

Background and purpose

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 methods

We 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.

Results

We 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.

Conclusion

Abnormalities 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

Background and purpose

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 methods

We 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.

Results

We 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.

Conclusion

Abnormalities 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.

Get Citation

Keywords

Huntington disease, Juvenile onset, Saccadic eye movements, Behavioral test, Neuropsychological tests

About this article
Title

Saccadic eye movements in juvenile variant of Huntington disease

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 48, No 4 (2014)

Pages

236-241

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

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