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dc.contributor.authorAbel, LA
dc.contributor.authorBowman, EA
dc.contributor.authorVelakoulis, D
dc.contributor.authorFahey, MC
dc.contributor.authorDesmond, P
dc.contributor.authorMacfarlane, MD
dc.contributor.authorLooi, JCL
dc.contributor.authorAdamson, CL
dc.contributor.authorWalterfang, M
dc.date.available2014-05-22T07:23:21Z
dc.date.available2012-10-29
dc.date.available2012-10-29
dc.date.available2012-10-29
dc.date.available2012-10-29
dc.date.available2012-10-29
dc.date.available2012-10-29
dc.date.available2012-10-29
dc.date.available2012-10-29
dc.date.available2012-10-29
dc.date.available2012-10-29
dc.date.issued2012-11-30
dc.identifierpii: PONE-D-12-17892
dc.identifier.citationAbel, L. A., Bowman, E. A., Velakoulis, D., Fahey, M. C., Desmond, P., Macfarlane, M. D., Looi, J. C. L., Adamson, C. L. & Walterfang, M. (2012). Saccadic Eye Movement Characteristics in Adult Niemann-Pick Type C Disease: Relationships with Disease Severity and Brain Structural Measures. PLOS ONE, 7 (11), https://doi.org/10.1371/journal.pone.0050947.
dc.identifier.issn1932-6203
dc.identifier.urihttp://hdl.handle.net/11343/32893
dc.descriptionC1 - Journal Articles Refereed
dc.description.abstractNiemann-Pick Type C disease (NPC) is a rare genetic disorder of lipid metabolism. A parameter related to horizontal saccadic peak velocity was one of the primary outcome measures in the clinical trial assessing miglustat as a treatment for NPC. Neuropathology is widespread in NPC, however, and could be expected to affect other saccadic parameters. We compared horizontal saccadic velocity, latency, gain, antisaccade error percentage and self-paced saccade generation in 9 adult NPC patients to data from 10 age-matched controls. These saccadic measures were correlated with appropriate MRI-derived brain structural measures (e.g., dorsolateral prefrontal cortex, frontal eye fields, supplemental eye fields, parietal eye fields, pons, midbrain and cerebellar vermis) and with measures of disease severity and duration. The best discriminators between groups were reflexive saccade gain and the two volitional saccade measures. Gain was also the strongest correlate with disease severity and duration. Most of the saccadic measures showed strongly significant correlations with neurophysiologically appropriate brain regions. While our patient sample is small, the apparent specificity of these relationships suggests that as new diagnostic methods and treatments become available for NPC, a broader range of saccadic measures may be useful tools for the assessment of disease progression and treatment efficacy.
dc.formatapplication/pdf
dc.languageEnglish
dc.publisherPUBLIC LIBRARY SCIENCE
dc.subjectCentral Nervous System; Neurology and Neuromuscular Diseases; Human Pharmaceutical Treatments (e.g. Antibiotics); Nervous System and Disorders
dc.titleSaccadic Eye Movement Characteristics in Adult Niemann-Pick Type C Disease: Relationships with Disease Severity and Brain Structural Measures
dc.typeJournal Article
dc.identifier.doi10.1371/journal.pone.0050947
melbourne.peerreviewPeer Reviewed
melbourne.affiliationThe University of Melbourne
melbourne.affiliation.departmentOptometry And Vision Sciences
melbourne.source.titlePLOS ONE
melbourne.source.volume7
melbourne.source.issue11
dc.rights.licenseCC BY
melbourne.publicationid188905
melbourne.elementsid349856
melbourne.openaccess.pmchttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC3511378
melbourne.contributor.authorWalterfang, Mark
melbourne.contributor.authorAbel, Larry
melbourne.contributor.authorDesmond, Patricia
melbourne.contributor.authorVelakoulis, Dennis
melbourne.contributor.authorBOWMAN, ELIZABETH
melbourne.contributor.authorBowman, Elizabeth
dc.identifier.eissn1932-6203
melbourne.fieldofresearch320903 Central nervous system
melbourne.fieldofresearch320905 Neurology and neuromuscular diseases
melbourne.seocode240803 Human pharmaceutical treatments
melbourne.seocode200199 Clinical health not elsewhere classified
melbourne.accessrightsAccess this item via the Open Access location


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