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Cited by 3,230Vestibular and auditory disordersNeural and Behavioral Psychology StudiesMotor Control and Adaptation

The cerebellar cognitive affective syndrome

Schmahmann, J.·1998·Brain
DOI 10.1093/brain/121.4.561
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Abstract

Anatomical, physiological and functional neuroimaging studies suggest that the cerebellum participates in the organization of higher order function, but there are very few descriptions of clinically relevant cases that address this possibility. We performed neurological examinations, bedside mental state tests, neuropsychological studies and anatomical neuroimaging on 20 patients with diseases confined to the cerebellum, and evaluated the nature and severity of the changes in neurological and mental function. Behavioural changes were clinically prominent in patients with lesions involving the posterior lobe of the cerebellum and the vermis, and in some cases they were the most noticeable aspects of the presentation. These changes were characterized by: impairment of executive functions such as planning, set-shifting, verbal fluency, abstract reasoning and working memory; difficulties with spatial cognition including visual-spatial organization and memory; personality change with blunting of affect or disinhibited and inappropriate behaviour; and language deficits including agrammatism and dysprosodia. Lesions of the anterior lobe of the cerebellum produced only minor changes in executive and visual-spatial functions. We have called this newly defined clinical entity the 'cerebellar cognitive affective syndrome'. The constellation of deficits is suggestive of disruption of the cerebellar modulation of neural circuits that link prefrontal, posterior parietal, superior temporal and limbic cortices with the cerebellum.

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Crossref · 10.1093/brain/121.4.561

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  • Acquired cerebellar lesions involving the posterior lobe of the vermis, part of a reciprocal network with the anterior limbic system, are associated with mild cognitive impairment, executive function deficits, expressive language impairment, and affective blunting or disinhibition, behaviors recognized in autistic disorder.

    “Acquired cerebellar lesions involving the posterior lobe of the vermis, part of a reciprocal anatomical network with the anterior limbic system, have been associated with mild cognitive impairment, deficits of executive function, expressive language impairment, and affective blunting or disinhibition [31], all behaviors recognized in AD.”

    From Consensus Paper: Pathological Role of the Cerebellum in Autism (2012) · cites this paper as [31]

  • Schmahmann and Sherman documented similar cognitive impairments in patients with chronic cerebellar disease.

    “Schmahmann and Sherman [31] documented similar cognitive impairments in patients with chronic cerebellar disease.”

    From Consensus Paper: Pathological Role of the Cerebellum in Autism (2012) · cites this paper as [31]

  • Other researchers suggested terms 'cognitive dysmetria' or 'dysmetria of thought' to describe the effect of loss of cerebellar function on cognition, implying strong similarities between motor and cognitive cerebellar function.

    “It is interesting, that other researchers, based on clinical observations alone, suggested the terms “cognitive dysmetria” [227] or “dysmetria of thought” [31] to describe the effect of loss of cerebellar function on cognition, implying strong similarities between motor and cognitive cerebellar function.”

    From Consensus Paper: Pathological Role of the Cerebellum in Autism (2012) · cites this paper as [31]

  • The cerebellum has a broader role than previously recognized, including emotion, non-motor associative learning, working memory and mental rehearsal, verbal working memory and other language functions, spatial and episodic memory, event prediction, empathy and predicting others' actions, imitation, planning and decision-making, individual variation in cognitive performance, and cognitive-developm

    “However, in the past 10 years or so considerable evidence has accumulated that the cerebellum has a broader role than previously recognized, including emotion [75,76], non-motor associative learning [77], working memory and mental rehearsal [77,78], verbal working memory and other language functions [76,78–81], spatial and episodic memory [79,81,82], event prediction [83], empathy and predicting”

    From Embodied cognitive evolution and the cerebellum (2012) · cites this paper as [79]

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Authors
Schmahmann, J.
Affiliations
Massachusetts General Hospital (US)
Corresponding author
Schmahmann, J.
ORCID
Schmahmann, J.
Venue
Brain
Publisher
Oxford University Press (OUP)
Published
1998-04-01
Volume
121
Issue
4
Pages
561-579
ISSN
  • 0006-8950 (ISSN-L)
  • 1460-2156
DOI
10.1093/brain/121.4.561
OpenAlex
W2104284834
Cited by
3,230
References
141
Citations by year
2026: 131 · 2025: 168 · 2024: 149 · 2023: 170 · 2022: 175 · 2021: 177 · 2020: 158 · 2019: 155 · 2018: 168 · 2017: 126 · 2016: 128 · 2015: 127 · 2014: 138 · 2013: 127 · 2012: 113
FWCI
23.89
Type
journal article
Language
en
Open access
closed
Locations
  • Brain (publishedVersion)
  • PubMed (publishedVersion)
Funders
Massachusetts General Hospital
Topics
  • Life Sciences › Neuroscience › Neurology › Vestibular and auditory disorders
  • Life Sciences › Neuroscience › Cognitive Neuroscience › Neural and Behavioral Psychology Studies
  • Life Sciences › Neuroscience › Cognitive Neuroscience › Motor Control and Adaptation
Keywords
cerebellar cognitive affective syndrome, cerebellar lesions, cerebellar vermis, executive dysfunction, spatial cognition, personality change, affective blunting, language deficits, agrammatism, neuropsychological assessment
MeSH terms
Adult, Affect, Aged, Attention, Brain, Cerebellar Diseases, Cerebellar Neoplasms, Cerebral Infarction, Cerebrovascular Disorders, Cognition, Cognition Disorders, Female, Humans, Magnetic Resonance Imaging, Male, Middle Aged, Neurologic Examination, Neuropsychological Tests, Syndrome

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