To the Editor: The cerebellum has traditionally been associated to the coordination of motor functions, however there is increasing evidence of a cerebellar contribution in higher cognitive functions.1–4
The cerebellar cognitive affective syndrome is clinically characterized by disturbances of executive functions, impairments in spatial cognition, personality changes and language difficulties.5 Since its description, there is a lack of reports of cases with pure cerebellar disorders to address its contribution in cognitive or affective impairments.
We report a case of volumetric reduction of cerebellum, with no extracerebellar abnormalities, associated with cognitive impairment and behavior change.
A 45-year-old male patient presented inappropriate behavior and personality changes: undressing in public places, talking loudly and quickly, and behaving aggressively with his wife. Eight months after the beginning of symptoms, the patient attempted suicide. He started psychiatric treatment but the symptomatology gradually worsened, despite pharmacologic treatment.
The patient presented progressive loss of interest, social withdrawal, impairments in intellectual and emotional functioning, impulsive behavior and inability to recover his daily activities. His first psychiatric admission was when he was 52, after physically assaulting his mother.
In March 2009, at age of 53 years old the patient was first admitted in our infirmary due to violent behavior. On admission he presented with limited spontaneous conversations and impoverishment of the content of thought, concrete interpretation, hypoprosody, inability to conduct simple calculations, diminished verbal fluency, apathy, blunted affect, facial hypomodulation, hypobulia, decreased psychomotor activity and poor fine motor coordination. His score at the MMSE was 22. The patient remained most of the time in his room, with a childish and impulsive behavior, verbally and physically attacking other inpatients.
He had normal hemogram, electrolytes, anti-HIV, VDRL, liver, renal and thyroid functions and vitamin B12. He underwent a MRI with volumetric reduction of cerebellum and enlargement of cerebellar sulci, basal cistern and IV ventricle. MRI showed no structural abnormalities outside the cerebellum.
We began quetiapine 600 mg/day, chlorpromazine 100 mg/day and clonazepam 1.5 mg/day, there was noticeable improvement of aggressiveness and impulsivity. The patient was discharged after 60 days of hospitalization. He was evaluated with the CAMCOG battery and scored 53, lower than expected for his age and education with impairments in working memory, verbal fluency and executive functions. Since then, he attends monthly to medical visits and his mental state is stabilized.
In 1998 the “cerebellar cognitive affective syndrome” was described and characterized by impairments of executive functions; visual-spatial disorganization and impairment of visual-spatial memory; personality changes and inappropriate behavior; and difficulties with linguistic abilities.5
In this case, impairments in executive function, linguistic abilities and personality changes are correlated to the neuropsychological and neuroimaging findings, and are consistent with the diagnosis of “cerebellar cognitive affective syndrome”. With pharmacologic treatment, aggressiveness and impulsivity improved considerably, although the cognitive impairments have remained. Further studies are still needed to elucidate the nature of the cerebellar mediation of cognitive process, their possibly link to motor functions and treatment alternatives to improve the cognitive impairment of these patients.