The authors performed a systematic review and meta-analysis of studies evaluating the affective component of the cerebellar cognitive affective syndrome. Depressive and anxiety symptoms and personality changes were more frequent in patients with spinocerebellar ataxia. Patients with cerebellar lesions were more likely to have depression, deficits in the ability to experience emotions, and behavioral difficulties. A meta-analysis revealed modestly higher scores on the Hamilton Depression Rating Scale (HAM-D) among patients with cerebellar disease. This review highlights the need for cohort studies using noncerebellar comparison groups, more sensitive measures, and appropriate-sized populations with isolated cerebellar lesions to thoroughly assess the affective component of the cerebellar cognitive affective syndrome.

https://neuro.psychiatryonline.org/doi/pdf/10.1176/jnp.2009.21.3.245

Evaluating the Affective Component of the Cerebellar Cognitive Affective Syndrome

The role of the cerebellum in cognition, both in healthy subjects and in patients with cerebellar diseases, is debated. Neuropsychological studies in spinocerebellar ataxia type 1 (SCA1) and type 2 (SCA2) demonstrated impairments in executive functions, verbal memory, and visuospatial performances, but prospective evaluations are not available. Our aims were to assess progression of cognitive and psychiatric functions in patients with SCA1 and SCA2 in a longitudinal study. We evaluated at baseline 20 patients with SCA1, 22 patients with SCA2 and 17 matched controls. Two subgroups of patients (9 SCA1, 11 SCA2) were re-evaluated after 2 years. We tested cognitive functions (Mini Mental State Examination, digit span, Corsi span, verbal memory, attentional matrices, modified Wisconsin Card Sorting Test, Raven Progressive Matrices, Benton test, phonemic and semantic fluency), psychiatric status (Scales for Assessment of Negative and Positive Symptoms, Hamilton Depression and Anxiety Scales), neurological conditions (Scale for Assessment and Rating of Ataxia), and functional abilities (Unified Huntington Disease Rating Scale–part IV). At baseline, SCA1 and SCA2 patients had significant deficits compared to controls, mainly in executive functions (phonemic and semantic fluencies, attentional matrices); SCA2 showed further impairment in visuospatial and visuoperceptive tests (Raven matrices, Benton test, Corsi span). Both SCA groups had higher depression and negative symptoms, particularly apathy, compared to controls. After 2 years, motor and functional disability worsened, while only attentive performances deteriorated in SCA2. This longitudinal study showed dissociation in progression of motor disability and cognitive impairment, suggesting that in SCA1 and SCA2 motor and cognitive functions might be involved with different progression rates.

Longitudinal study of cognitive and psychiatric functions in spinocerebellar ataxia types 1 and 2.

Prediction, Psychosis, and the Cerebellum.

http://www.lib.kobe-u.ac.jp/repository/90000758.pdf

Characteristics of depression in Parkinson's disease: evaluating with Zung's Self-Rating Depression Scale.

Background Neurologic manifestations of primary Sjogren's syndrome (PSS) have been reported to vary from sensory polyneuropathy to encephalopathy or psychiatric problems. However, marked cerebellar degeneration associated with PSS has rarely been reported. Case report We describe a patient with Sjogren's syndrome who exhibited rapidly progressive cerebellar ataxia, nystagmus, cognitive decline, and psychiatric problems. Brain magnetic resonance imaging revealed marked atrophy of the cerebellum, and (18)F-fluorodeoxyglucose positron-emission tomography demonstrated glucose hypometabolism of the cerebellum. Conclusions Our PSS patient exhibited a progressive course of cerebellar syndrome, as evidenced by cerebellar atrophy on serial brain images.

/pdf/cerebellar-degeneration-associated-with-sjogren-s-syndrome-1j06diwuvl.pdf

Cerebellar Degeneration Associated with Sjögren's Syndrome

The authors performed a chart review to determine the frequency with which neurologists detect cognitive and psychiatric symptoms in patients with cerebellar degeneration. Psychopathology, including depression, personality change, cognitive impairment, anxiety, and psychosis was noted in 51% of 133 patients.

Christine M. Liszewski

Papers

Cognitive impairment and psychiatric symptoms in 133 Patients with diseases associated with Cerebellar Degeneration.