Cerebellar cognitive affective syndrome (Schmahmann syndrome) following isolated cerebellar infarction

Ramadhan, Mochamad Iskandarsyah Agung; Lastri, Diatri Nari; Ramli, Yetty; Prawiroharjo, Pukovisa

doi:10.1590/1980-5764-DN-2026-0500

Tabla de contenido Anterior Actual Siguiente

Tabla de contenido

Case Report • Dement. neuropsychol. 20 • 2026 • https://doi.org/10.1590/1980-5764-DN-2026-0500 linkcopy

Cerebellar cognitive affective syndrome (Schmahmann syndrome) following isolated cerebellar infarction

Síndrome cognitivo-afetiva cerebelar (síndrome de Schmahmann) após infarto cerebelar isolado

Authorship SCIMAGO INSTITUTIONS RANKINGS

The cerebellum plays an important role in higher-order cognition through cerebrocerebellar networks. Lesions affecting this structure may result in cerebellar cognitive affective syndrome (CCAS), which is frequently underrecognized by routine cognitive screening tools. We reported a 69-year-old right-handed man who presented with 18 months of forgetfulness, impaired concentration, and irritability following a documented left cerebellar infarction. Neurological examination revealed cerebellar signs without cortical deficits. Brain magnetic resonance imaging (MRI) demonstrated a left cerebellar infarct with hemorrhagic transformation. While the Mini-Mental State Examination (MMSE) was normal and the Montreal Cognitive Assessment (MoCA) showed mild impairment, detailed neuropsychological assessment using CCAS Scale (CCAS-S) confirmed definite CCAS. This case highlights the importance of cerebellum-specific cognitive assessment in patients with isolated cerebellar stroke, as conventional screening tools might underestimate multidomain cognitive and affective disturbances.

Keywords:
Cerebellar Cognitive Affective Syndrome; Brain Infarction; Cognitive Dysfunction

Instrument	Findings	Interpretation
MMSE	27/30 (orientation −1, calculation −2); other domains intact	Within normal limits; mild calculation deficit
MoCA-Ina	20/30; impaired visuospatial/executive (4/5), attention (1/2), letter vigilance (0/1), calculation (2/3), repetition (1/2), delayed recall (0/5); preserved naming (3/3), abstraction (2/2), orientation (6/6), verbal fluency (1/1)	Impairment with executive and memory predominance
CERAD	Impaired verbal fluency (10 words/min), immediate recall (2/6/5), delayed recall (0/10), recognition (4/10 hits; 8 false positives); other language functions relatively preserved	Verbal memory and fluency impairment
TMT-A/B	TMT-A 33 seconds; TMT-B in 123 seconds with three errors	Within normal limits
CCAS-S (v1A)	Failed: phonemic fluency (6/19), category switching (4/15), digit span forward (4/8), verbal recall (10/15); Preserved: semantic fluency (16/26), digit span backward (4/6), cube drawing (15/15), similarities (7/8), go/no-go (1/2), affect (5/6; irritability noted)	Definite CCAS (≥3 failed items)

location_on

Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento R. Vergueiro, 1353 sl.1404 - Ed. Top Towers Offices, Torre Norte, São Paulo, SP, Brazil, CEP 04101-000, Tel.: +55 11 5084-9463 | +55 11 5083-3876 - São Paulo - SP - Brazil
E-mail: revistadementia@abneuro.org.br | demneuropsy@uol.com.br

rss_feed Acompañe los números de esta revista en su lector de RSS

[1] Abbreviations: MMSE, Mini-Mental State Examination; MoCA-Ina, Montreal Cognitive Assessment — Indonesian version; CERAD, Consortium to Establish a Registry for Alzheimer’s Disease; TMT, Trail Making Test; CCAS-S, Cerebellar Cognitive Affective Syndrome Scale (version 1A).