Abstract
BackgroundThe neuropathological process underlying amyotrophic lateral sclerosis (ALS) can be traced as a four-stage progression scheme of sequential corticofugal axonal spread. The examination of eye movement control gains deep insights into brain network pathology and provides the opportunity to detect both disturbance of the brainstem oculomotor circuitry as well as executive deficits of oculomotor function associated with higher brain networks.ObjectiveTo study systematically oculomotor characteristics in ALS and its underlying network pathology in order to determine whether eye movement deterioration can be categorized within a staging system of oculomotor decline that corresponds to the neuropathological model.MethodsSixty-eight ALS patients and 31 controls underwent video-oculographic, clinical and neuropsychological assessments.ResultsOculomotor examinations revealed increased anti- and delayed saccades’ errors, gaze-palsy and a cerebellary type of smooth pursuit disturbance. The oculomotor disturbances occurred in a sequential manner: Stage 1, only executive control of eye movements was affected. Stage 2 indicates disturbed executive control plus ‘genuine’ oculomotor dysfunctions such as gaze-paly. We found high correlations (p<0.001) between the oculomotor stages and both, the clinical presentation as assessed by the ALS Functional Rating Scale (ALSFRS) score, and cognitive scores from the Edinburgh Cognitive and Behavioral ALS Screen (ECAS).ConclusionsDysfunction of eye movement control in ALS can be characterized by a two-staged sequential pattern comprising executive deficits in Stage 1 and additional impaired infratentorial oculomotor control pathways in Stage 2. This pattern parallels the neuropathological staging of ALS and may serve as a technical marker of the neuropathological spreading.
Highlights
Amyotrophic lateral sclerosis (ALS) is the most common age-related motorneuron disease characterized by a rapidly progressive neurodegenerative disorder with involvement of the upper and lower motoneurons, leading to death with a mean survival of approximately 3 years [1]
Dysfunction of eye movement control in amyotrophic lateral sclerosis (ALS) can be characterized by a two-staged sequential pattern comprising executive deficits in Stage 1 and additional impaired infratentorial oculomotor control pathways in Stage 2
This pattern parallels the neuropathological staging of ALS and may serve as a technical marker of the neuropathological spreading
Summary
Amyotrophic lateral sclerosis (ALS) is the most common age-related motorneuron disease characterized by a rapidly progressive neurodegenerative disorder with involvement of the upper and lower motoneurons, leading to death with a mean survival of approximately 3 years [1]. With predominant involvement of executive function, are more common in ALS than previously thought [1] even in nondemented ALS patients. As previously described [2], ALS progresses according to a sequential regional pattern that can be defined by four neuropathological stages with primary affection of the cerebral cortex and dissemination by involved cortical projection neurons, along corticofugal axons. These findings raise the issue of whether the apparent neuropathological progression can be paralleled by clinical staging. The neuropathological process underlying amyotrophic lateral sclerosis (ALS) can be traced as a four-stage progression scheme of sequential corticofugal axonal spread. The examination of eye movement control gains deep insights into brain network pathology and provides the opportunity to detect both disturbance of the brainstem oculomotor circuitry as well as executive deficits of oculomotor function associated with higher brain networks
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