Unraveling corticobasal syndrome and alien limb syndrome with structural brain imaging

Albrecht, Franziska; Mueller, Karsten; Ballarini, Tommaso; Lampe, Leonie; Diehl‐Schmid, Janine; Faßbender, Klaus; Fließbach, Klaus; Jahn, Holger; Jech, Robert; Kassubek, Jan; Kornhuber, Johannes; Landwehrmeyer, G. Bernhard; Lauer, Martin; Ludolph, Albert C.; Lyros, Epameinondas; Prudlo, Johannes; Schneider, Anja; Synofzik, Matthis; Wiltfang, Jens; Danek, Adrian; Otto, Markus; FTLD-Consortium,; Schroeter, Matthias L.

doi:10.1016/j.cortex.2019.02.015

Cited by 19 publications

(31 citation statements)

References 25 publications

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“…The clinical and phenomenological dissociation between apraxia and alien limb phenomena might be surprising, as they both share underlying brain lesions, e.g. in the supplementary motor, prefrontal and parietal cortices or white matter connecting these regions [7,9,10,27,28]. What might explain this clinical dissociation, considering, despite the shared anatomical lesions?…”

Section: The Relationship Between Alien Limb Phenomena and Apraxiamentioning

confidence: 99%

“…The other is a syndrome seen following damage to the left frontomedial and callosal regions in which the patient's dominant hand exhibits unintentional reaching and grasping evoked by external stimuli [8]. Within these frontal regions, damage to the presupplementary motor area may play a central role in the development of alien limb [9,10].…”

Section: Introductionmentioning

confidence: 99%

“…Another disorder of the sensorimotor system that is included in the diagnostic criteria of, and is common in, the corticobasal syndrome is called 'apraxia' [7,9,10,22]. Apraxia may be defined as a disorder of "the execution of learned movement which cannot be accounted for either by weakness, incoordination or sensory loss, or by incomprehension of or inattention to commands" [23].…”

Section: Introductionmentioning

confidence: 99%

“…In addition to their co-occurrence in the corticobasal syndrome, limb apraxia and alien limb syndrome are both disorders of complex sensorimotor function with some similarities. First, shared anatomic substrates have been implicated through association with lesions either directly affecting the supplementary motor, prefrontal and parietal cortices or causing their disconnection [7,9,10,27,28]. Second, classical alien behaviours were phenomenologically described as 'diagonistic dyspraxia' [27] and 'magnetic apraxia' [29].…”

Section: Introductionmentioning

confidence: 99%

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Alien limb in the corticobasal syndrome: phenomenological characteristics and relationship to apraxia

et al. 2020

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Alien limb refers to movements that seem purposeful but are independent of patients' reported intentions. Alien limb often co-occurs with apraxia in the corticobasal syndrome, and anatomical and phenomenological comparisons have led to the suggestion that alien limb and apraxia may be causally related as failures of goal-directed movements. Here, we characterised the nature of alien limb symptoms in patients with the corticobasal syndrome (n = 30) and their relationship to limb apraxia. Twenty-five patients with progressive supranuclear palsy Richardson syndrome served as a disease control group. Structured examinations of praxis, motor function, cognition and alien limb were undertaken in patients attending a regional specialist clinic. Twenty-eight patients with corticobasal syndrome (93%) demonstrated significant apraxia and this was often asymmetrical, with the left hand preferentially affected in 23/30 (77%) patients. Moreover, 25/30 (83%) patients reported one or more symptoms consistent with alien limb. The range of these phenomena was broad, including changes in the sense of ownership and control as well as unwanted movements. Regression analyses showed no significant association between the severity of limb apraxia and either the occurrence of an alien limb or the number of alien limb phenomena reported. Bayesian estimation showed a low probability for a positive association between alien limb and apraxia, suggesting that alien limb phenomena are not likely to be related to severity apraxia. Our results shed light on the phenomenology of these disabling and as yet untreatable clinical features, with relevance to theoretical models of voluntary action.

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Section: The Relationship Between Alien Limb Phenomena and Apraxiamentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Alien limb in the corticobasal syndrome: phenomenological characteristics and relationship to apraxia

et al. 2020

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“…Within these frontal regions, damage to the pre-supplementary motor area may play a central role in the development of alien limb [9,10].…”

Section: Introductionmentioning

confidence: 99%

Alien limb in the corticobasal syndrome: phenomenological characteristics and relationship to apraxia

Lewis‐Smith

Wolpe

Ghosh

et al. 2019

Preprint

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Alien limb refers to movements that seem purposeful but are independent of patients' reported intentions. Alien limb often co-occurs with apraxia in the corticobasal syndrome, and anatomical and phenomenological comparisons have led to the suggestion that alien limb and apraxia may be causally related as failures of goal-directed movements. Here, we characterised the nature of alien limb symptoms in patients with the corticobasal syndrome (n=30) and their relationship to limb apraxia. Twenty-five patients with progressive supranuclear palsy Richardson syndrome served as a disease-control group. Structured examinations of praxis, motor function, cognition and alien limb were undertaken in patients attending a regional specialist clinic. Twenty-eight patients with corticobasal syndrome (93%) demonstrated significant apraxia and this was often asymmetrical, with the left hand preferentially affected in 77% of patients. Moreover, 83% reported one or more symptoms consistent with alien limb. The range of these phenomena was broad, including changes in the sense of ownership and control as well as unwanted movements. Regression analyses showed no significant association between the severity of limb apraxia and both the occurrence of an alien limb and the number of alien limb phenomena reported. Bayesian estimation showed a low probability for a positive association between alien limb and apraxia, suggesting that alien limb phenomena are not likely to be related to severe apraxia. Our results shed light on the phenomenology of these disabling and as yet untreatable clinical features, with relevance to theoretical models of voluntary action.

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Network Localization of Alien Limb in Patients with Corticobasal Syndrome

Tetreault

Phan

Petersen

et al. 2020

Annals of Neurology

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for the 4 Repeat Tau Neuroimaging Initiative † Objective: Perirolandic atrophy occurs in corticobasal syndrome (CBS) but is not specific versus progressive supranuclear palsy (PSP). There is heterogeneity in the locations of atrophy outside the perirolandic cortex and it remains unknown why atrophy in different locations would cause the same CBS-specific symptoms. In prior work, we used a wiring diagram of the brain called the human connectome to localize lesion-induced disorders to symptom-specific brain networks. Here, we use a similar technique termed "atrophy network mapping" to localize single-subject atrophy maps to symptom-specific brain networks. Methods: Single-subject atrophy maps were generated by comparing cortical thickness in patients with CBS versus controls. Next, we performed seed-based functional connectivity using a large normative connectome to determine brain regions functionally connected to each patient's atrophied locations. Results: Patients with CBS had perirolandic atrophy versus controls at the group level, but locations of atrophy in CBS were heterogeneous outside of the perirolandic cortex at the single-subject level (mean spatial correlation = 0.04). In contrast, atrophy occurred in locations functionally connected to the perirolandic cortex in all patients with CBS (spatial correlation = 0.66). Compared with PSP, patients with CBS had atrophy connected to a network of higher-order sensorimotor regions beyond perirolandic cortex, matching a CBS atrophy network from a recent meta-analysis. Finally, atrophy network mapping identified a symptom-specific network for alien limb, matching a lesion-induced alien limb network and a network associated with agency in healthy subjects. Interpretation: We identified a syndrome-specific network for CBS and symptom-specific network for alien limb using single-subject atrophy maps and the human connectome.

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Unraveling corticobasal syndrome and alien limb syndrome with structural brain imaging

Cited by 19 publications

References 25 publications

Alien limb in the corticobasal syndrome: phenomenological characteristics and relationship to apraxia

Alien limb in the corticobasal syndrome: phenomenological characteristics and relationship to apraxia

Alien limb in the corticobasal syndrome: phenomenological characteristics and relationship to apraxia

Network Localization of Alien Limb in Patients with Corticobasal Syndrome

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