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The disturbance of gaze in progressive supranuclear palsy: implications for pathogenesis.

Chen AL, Riley DE, King SA, Joshi AC, Serra A, Liao K, Cohen ML, Otero-Millan J, Martinez-Conde S, Strupp M, Leigh RJ - Front Neurol (2010)

Bottom Line: These vertical and vergence eye movements habitually work in concert to enable visuomotor skills that are important during locomotion with the hands free.Taken with the prominent early feature of falls, these findings suggest that PSP tauopathy impairs a recently evolved neural system concerned with bipedal locomotion in an erect posture and frequent gaze shifts between the distant environment and proximate hands.This approach provides a conceptual framework that can be used to address the nosological challenge posed by overlapping clinical and neuropathological features of neurodegenerative tauopathies.

View Article: PubMed Central - PubMed

Affiliation: Veterans Affairs Medical Center, University Hospitals Case Medical Center Cleveland, OH, USA.

ABSTRACT
Progressive supranuclear palsy (PSP) is a disease of later life that is currently regarded as a form of neurodegenerative tauopathy. Disturbance of gaze is a cardinal clinical feature of PSP that often helps clinicians to establish the diagnosis. Since the neurobiology of gaze control is now well understood, it is possible to use eye movements as investigational tools to understand aspects of the pathogenesis of PSP. In this review, we summarize each disorder of gaze control that occurs in PSP, drawing on our studies of 50 patients, and on reports from other laboratories that have measured the disturbances of eye movements. When these gaze disorders are approached by considering each functional class of eye movements and its neurobiological basis, a distinct pattern of eye movement deficits emerges that provides insight into the pathogenesis of PSP. Although some aspects of all forms of eye movements are affected in PSP, the predominant defects concern vertical saccades (slow and hypometric, both up and down), impaired vergence, and inability to modulate the linear vestibulo-ocular reflex appropriately for viewing distance. These vertical and vergence eye movements habitually work in concert to enable visuomotor skills that are important during locomotion with the hands free. Taken with the prominent early feature of falls, these findings suggest that PSP tauopathy impairs a recently evolved neural system concerned with bipedal locomotion in an erect posture and frequent gaze shifts between the distant environment and proximate hands. This approach provides a conceptual framework that can be used to address the nosological challenge posed by overlapping clinical and neuropathological features of neurodegenerative tauopathies.

No MeSH data available.


Related in: MedlinePlus

Comparison of fixation behavior in a normal elderly subject (A) and in a patient with PSP (B). Both show saccadic intrusions (square-wave jerks), but they are larger and more frequent in the record from the PSP patient. The gray dashed line corresponds to the desired horizontal position of fixation. The vertical channel has been offset in both records to aid clarity. Positive values indicate rightward and upward movements.
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Figure 5: Comparison of fixation behavior in a normal elderly subject (A) and in a patient with PSP (B). Both show saccadic intrusions (square-wave jerks), but they are larger and more frequent in the record from the PSP patient. The gray dashed line corresponds to the desired horizontal position of fixation. The vertical channel has been offset in both records to aid clarity. Positive values indicate rightward and upward movements.

Mentions: In PSP, the prominent movements during attempted steady fixation are saccadic intrusions (Troost and Daroff, 1977), mainly to-and-fro SWJ (see Figure 5). SWJ also occur in normal subjects (Abadi and Gowen, 2004), however, and current evidence suggests that SWJ and microsaccades constitute points on a continuum (Gowen et al., 2007). In PSP, saccades occurring during attempted fixation are not only more frequent and larger than in normal subjects, but they also lack a vertical component (Otero-Millan et al., 2009).


The disturbance of gaze in progressive supranuclear palsy: implications for pathogenesis.

Chen AL, Riley DE, King SA, Joshi AC, Serra A, Liao K, Cohen ML, Otero-Millan J, Martinez-Conde S, Strupp M, Leigh RJ - Front Neurol (2010)

Comparison of fixation behavior in a normal elderly subject (A) and in a patient with PSP (B). Both show saccadic intrusions (square-wave jerks), but they are larger and more frequent in the record from the PSP patient. The gray dashed line corresponds to the desired horizontal position of fixation. The vertical channel has been offset in both records to aid clarity. Positive values indicate rightward and upward movements.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3008928&req=5

Figure 5: Comparison of fixation behavior in a normal elderly subject (A) and in a patient with PSP (B). Both show saccadic intrusions (square-wave jerks), but they are larger and more frequent in the record from the PSP patient. The gray dashed line corresponds to the desired horizontal position of fixation. The vertical channel has been offset in both records to aid clarity. Positive values indicate rightward and upward movements.
Mentions: In PSP, the prominent movements during attempted steady fixation are saccadic intrusions (Troost and Daroff, 1977), mainly to-and-fro SWJ (see Figure 5). SWJ also occur in normal subjects (Abadi and Gowen, 2004), however, and current evidence suggests that SWJ and microsaccades constitute points on a continuum (Gowen et al., 2007). In PSP, saccades occurring during attempted fixation are not only more frequent and larger than in normal subjects, but they also lack a vertical component (Otero-Millan et al., 2009).

Bottom Line: These vertical and vergence eye movements habitually work in concert to enable visuomotor skills that are important during locomotion with the hands free.Taken with the prominent early feature of falls, these findings suggest that PSP tauopathy impairs a recently evolved neural system concerned with bipedal locomotion in an erect posture and frequent gaze shifts between the distant environment and proximate hands.This approach provides a conceptual framework that can be used to address the nosological challenge posed by overlapping clinical and neuropathological features of neurodegenerative tauopathies.

View Article: PubMed Central - PubMed

Affiliation: Veterans Affairs Medical Center, University Hospitals Case Medical Center Cleveland, OH, USA.

ABSTRACT
Progressive supranuclear palsy (PSP) is a disease of later life that is currently regarded as a form of neurodegenerative tauopathy. Disturbance of gaze is a cardinal clinical feature of PSP that often helps clinicians to establish the diagnosis. Since the neurobiology of gaze control is now well understood, it is possible to use eye movements as investigational tools to understand aspects of the pathogenesis of PSP. In this review, we summarize each disorder of gaze control that occurs in PSP, drawing on our studies of 50 patients, and on reports from other laboratories that have measured the disturbances of eye movements. When these gaze disorders are approached by considering each functional class of eye movements and its neurobiological basis, a distinct pattern of eye movement deficits emerges that provides insight into the pathogenesis of PSP. Although some aspects of all forms of eye movements are affected in PSP, the predominant defects concern vertical saccades (slow and hypometric, both up and down), impaired vergence, and inability to modulate the linear vestibulo-ocular reflex appropriately for viewing distance. These vertical and vergence eye movements habitually work in concert to enable visuomotor skills that are important during locomotion with the hands free. Taken with the prominent early feature of falls, these findings suggest that PSP tauopathy impairs a recently evolved neural system concerned with bipedal locomotion in an erect posture and frequent gaze shifts between the distant environment and proximate hands. This approach provides a conceptual framework that can be used to address the nosological challenge posed by overlapping clinical and neuropathological features of neurodegenerative tauopathies.

No MeSH data available.


Related in: MedlinePlus