Augenbewegungsstörungen bei Amyotropher Lateralsklerose - Bericht über zwei Patienten

Palmowski, Anja M.; Jost, Wolfgang H.; Osterhage, J.; Prudlo, Johannes; Käsmann, B; Schimrigk, К.; Ruprecht, Klaus W.

doi:10.1055/s-2008-1035424

Cited by 8 publications

(4 citation statements)

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“…Although not commonly seen in neurological practice (because patients usually die before entering the complete locked-in state), eye muscles may become totally paralyzed in ALS rendering the patients completely locked in (complete locked-in state 5 CLIS) (Cohen & Caroscio, 1983;Harvey, Torack, & Rosenbaum, 1979;Kushner et al, 1984;Palmowski et al, 1995a); our own experience confirms these reports (Hill et al, 2006;Hinterberger, Birbaumer, & Flor, 2005;. For such patients, the visually based P300-BCI would no longer provide a reliable communication channel.…”

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confidence: 53%

“…Although sparing of neurodegeneration of nerves, which subserve eye movement, has been reported (Hayashi & Kato, 1989;Whitehouse, Wamsley, Zarbin, Price, & Kuhar, 1985), there are now numerous reports about impaired eye movement and slowing of saccades in ALS (Averbuch-Heller, Helmchen, Horn, Leigh, & Buttner-Ennerver, 1998;Jacobs, Bozian, Heffner, & Barron, 1981;Leveille, Kiernan, Goodwin, & Antel, 1982;Ohki et al, 1994;Palmowski et al, 1995a;Palmowski et al, 1995b;Szmidt-Salkowska & Rowinska-Marcinska, 2005). Although not commonly seen in neurological practice (because patients usually die before entering the complete locked-in state), eye muscles may become totally paralyzed in ALS rendering the patients completely locked in (complete locked-in state 5 CLIS) (Cohen & Caroscio, 1983;Harvey, Torack, & Rosenbaum, 1979;Kushner et al, 1984;Palmowski et al, 1995a); our own experience confirms these reports (Hill et al, 2006;Hinterberger, Birbaumer, & Flor, 2005;.…”

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confidence: 99%

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An auditory oddball (P300) spelling system for brain‐computer interfaces

et al. 2009

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This study was designed to develop and test an auditory event-related potential (ERP) based spelling system for a brain-computer interface (BCI) and to compare user's performance between the auditory and visual modality. The spelling system, where letters in a matrix were coded with acoustically presented numbers, was tested on a group of healthy volunteers. The results were compared with a visual spelling system. Nine of the 13 participants presented with the auditory ERP spelling system scored above a predefined criterion level control for communication. Compared to the visual spelling system, users' performance was lower and the peak latencies of the auditorily evoked ERPs were delayed. It was concluded that auditorily evoked ERPs from the majority of the users could be reliably classified. High accuracies were achieved in these users, rendering item selection with a BCI based on auditory stimulation feasible for communication.

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confidence: 53%

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confidence: 99%

An auditory oddball (P300) spelling system for brain‐computer interfaces

et al. 2009

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“…The two BIBD cases presented here were diagnosed with ALS-plus syndrome in which MND is associated with one or more clinical phenomena (i.e. dementia, parkinsonism) that have been considered by some to exclude the diagnosis of ALS [41–45]. However, it is now accepted that 10% of ALS patients manifest clinical features of FTD and up to 50% have measureable frontotemporal cognitive deficits [46].…”

Section: Discussionmentioning

confidence: 99%

Topography of FUS pathology distinguishes late-onset BIBD from aFTLD-U

Lee

Russ

Jung

et al. 2013

acta neuropathol commun

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BackgroundMultiple neurodegenerative diseases are characterized by the abnormal accumulation of FUS protein including various subtypes of frontotemporal lobar degeneration with FUS inclusions (FTLD-FUS). These subtypes include atypical frontotemporal lobar degeneration with ubiquitin-positive inclusions (aFTLD-U), basophilic inclusion body disease (BIBD) and neuronal intermediate filament inclusion disease (NIFID). Despite considerable overlap, certain pathologic features including differences in inclusion morphology, the subcellular localization of inclusions, and the relative paucity of subcortical FUS pathology in aFTLD-U indicate that these three entities represent related but distinct diseases. In this study, we report the clinical and pathologic features of three cases of aFTLD-U and two cases of late-onset BIBD with an emphasis on the anatomic distribution of FUS inclusions.ResultsThe aFTLD-U cases demonstrated FUS inclusions in cerebral cortex, subcortical grey matter and brainstem with a predilection for anterior forebrain and rostral brainstem. In contrast, the distribution of FUS pathology in late-onset BIBD cases demonstrated a predilection for pyramidal and extrapyramidal motor regions with relative sparing of cerebral cortex and limbic regions.ConclusionsThe topography of FUS pathology in these cases demonstrate the diversity of sporadic FUS inclusion body diseases and raises the possibility that late-onset motor neuron disease with BIBD neuropathology may exhibit unique clinical and pathologic features.

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“…Although oculomotor function is generally retained in ALS patients, they may manifest various oculomotor dysfunctions both at a relative early stage and in advanced stages of the disease. These include (i) a worsening of saccadic and pursuit eye movements ( Leveille et al, 1982 ; Cohen and Caroscio, 1983 ; Saito and Yamamoto, 1989 ; Gizzi et al, 1992 ; Marti-Fàbregas and Roig, 1993 ; Ohki et al, 1994 ; Abel et al, 1995 ; Shaunak et al, 1995 ; Okuda et al, 2009 ; Donaghy et al, 2010 ; Moss et al, 2012 ; Kang et al, 2018 ); (ii) a lack of suppression of the vestibulo-ocular reflex ( Ohki et al, 1994 ); (iii) a significant increase in error rates (distraction) and latency in anti-saccadic movements ( Shaunak et al, 1995 ; Donaghy et al, 2010 ) that may evoke saccadic paradigms, head shaking ( Kang et al, 2018 ), and positional nystagmus of central origin ( Saito and Yamamoto, 1989 ; Marti-Fàbregas and Roig, 1993 ; Ohki et al, 1994 ; Kang et al, 2018 ); (iv) gaze fixation instability ( Saito and Yamamoto, 1989 ; Palmowski et al, 1995 ; Shaunak et al, 1995 ; Donaghy et al, 2009 ; Moss et al, 2012 ; Kang et al, 2018 ); (v) eyelid opening apraxia ( Averbuch-Heller et al, 1998 ; Moss et al, 2012 ); and (vi) square wave jerks ( Gizzi et al, 1992 ; Shaunak et al, 1995 ; Kang et al, 2018 ), which may reflect the incidence of secondary abnormalities, such as parkinsonism ( Gizzi et al, 1992 ), and facilitate prefrontal dysfunction in these patients ( Shaunak et al, 1995 ). In bulbar onset compared with spinal onset, saccadic dysmetria and abnormal cogwheeling smooth pursuits are increased, which suggests neurodegeneration in ALS involving more than motor neurons (dysfunction of vestibule cerebellar connections), especially in bulbar-onset disease ( Kang et al, 2018 ).…”

Section: Amyotrophic Lateral Sclerosis and The Eyementioning

confidence: 99%

Amyotrophic Lateral Sclerosis: A Neurodegenerative Motor Neuron Disease With Ocular Involvement

et al. 2020

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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that causes degeneration of the lower and upper motor neurons and is the most prevalent motor neuron disease. This disease is characterized by muscle weakness, stiffness, and hyperreflexia. Patients survive for a short period from the onset of the disease. Most cases are sporadic, with only 10% of the cases being genetic. Many genes are now known to be involved in familial ALS cases, including some of the sporadic cases. It has also been observed that, in addition to genetic factors, there are numerous molecular mechanisms involved in these pathologies, such as excitotoxicity, mitochondrial disorders, alterations in axonal transport, oxidative stress, accumulation of misfolded proteins, and neuroinflammation. This pathology affects the motor neurons, the spinal cord, the cerebellum, and the brain, but recently, it has been shown that it also affects the visual system. This impact occurs not only at the level of the oculomotor system but also at the retinal level, which is why the retina is being proposed as a possible biomarker of this pathology. The current review discusses the main aspects mentioned above related to ALS, such as the main genes involved, the most important molecular mechanisms that affect this pathology, its ocular involvement, and the possible usefulness of the retina as a biomarker.

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Augenbewegungsstörungen bei Amyotropher Lateralsklerose - Bericht über zwei Patienten

Abstract: These findings provide further evidence of early oculomotor involvement in ALS, e.g. prior to respiratory failure and prior than previously suspected. Oculomotor impairment may occur in both the peripheral and the bulbar type of ALS.

Cited by 8 publications

References 1 publication

An auditory oddball (P300) spelling system for brain‐computer interfaces

An auditory oddball (P300) spelling system for brain‐computer interfaces

Topography of FUS pathology distinguishes late-onset BIBD from aFTLD-U

Amyotrophic Lateral Sclerosis: A Neurodegenerative Motor Neuron Disease With Ocular Involvement

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