Cerebellar Cognitive Affective Syndrome-A Case Report

There are an increasing number of reports suggesting that cerebellum, apart from its significance in movement coordination, plays an important role in cognitive and emotional regulation. Cerebellar Cognitive Affective Syndrome (CCAS) also known as Schmahmann syndrome, refers to deficits in the cognitive domains of executive function, spatial cognition, language and affect resulting from damage to the cerebellum. There are only few case reports reflecting the affective features in CCAS. We present a 17-year-old girl brought to psychiatry Outpatient Department (OPD) with depressive features, who was subsequently diagnosed as a case of CCAS and started on fluoxetine 20 mg and propranolol 20 mg daily. There was improvement in the affective symptoms on follow up.

A 17-year-old female, studying in 12^th standard, presented to the psychiatry OPD with a history of persistent low mood, anxiety, decreased interest in pleasurable activities and disturbed sleep for the past two months. This was preceded by gradual onset of weakness in body leading to inability to walk steadily and had frequent falls. She had difficulty in writing and holding things steadily in her hands, all amounting to problems in her scholastic performance. There were no problems in speech. Family history of psychiatric disorder, history of chronic medical illness and substance abuse was absent.

A general physical examination revealed a conscious, cooperative well oriented young girl with normal vitals. On neurological examination, there were ataxic gait, tremors, impaired tandem walking and impaired tests for coordination (finger to nose test, heel-shin test, dysdiadochokinesia). Sensory and motor systems and cranial nerves examinations were normal. Mental state examination revealed poor eye contact, reduced psychomotor activity and speech, depressed affect, decreased self-confidence and hopelessness. Higher cognitive functions including digit span, serial subtraction, FAS test, proverb interpretation were impaired.

Results of routine blood investigations, liver and kidney function tests, iron and lipid profile, thyroid function tests, vitamin D and B12 levels, X-ray chest, ultrasound abdomen and electroencephalograph were within normal limits. MRI brain showed diffuse cerebellar atrophy with bilateral prominent cerebellar folia [Table/Fig-1-3]. Cerebellar cognitive affective syndrome was diagnosed based on impairment in executive functions, neuropsychiatric manifestations, impaired cerebellar functions and MRI changes [1,2]. Patient was given fluoxetine 20 mg and propranolol 20 mg daily. She reported improvement in anxiety, sleep, appetite and sad mood on fourth week follow-up. On follow-up after eight weeks, her affective symptoms remitted completely. Same medication was continued and regular follow up was advised.

[Table/Fig-1-3]:

MRI brain showing prominent folia on both sides indicating diffuse cerebellar atrophy.

Cerebellar cognitive affective syndrome also known as Schmahmann syndrome was first reported by Schmahmann JD and Sherman JC [1]. It refers to deficits in the cognitive domains of executive function, spatial cognition, language, and affect resulting from damage to the cerebellum. These cognitive impairments result in an overall lowering of intellectual function [2-4]. Although, certain features were prominent in each patient; however, all symptoms were not present in every patient. The behavioural changes were clinically prominent in patients with disease of the posterior lobe of the cerebellum and the vermis [5]. The cerebellum itself has largely been considered for regulation of motor functions. CCAS raises the possibility of cerebellar association with non-motor deficits due to dysfunction in its connections to the cortex and limbic system. Personality changes such as disinhibited and inappropriate behaviour has been reported in CCAS but in our patient it was absent. The severity of CCAS varies depending on the site and extent of the lesion. The behavioural symptoms are severe if the cerebellar lesion is bilateral or it lies in the posterior lobe, especially vermis [1,5]. Behavioural and emotional changes have been linked to dysfunctional circuits between cerebellum, cortex and limbic system [6].

There can be a number of causes for cerebellar damage such as cerebellar agenesis, dysplasia and hypoplasia, cerebellar stroke, tumour, cerebellitis, trauma, neurodegenerative diseases, viral infections and drugs [1,7]. Our patient was more likely to be sporadic as no specific cause could be detected.

Treatment of CCAS include cognitive behaviour therapy, reality orientation therapy, transcranial magnetic stimulation and medications such as bromocriptine (to treat deficit in executive functioning and learning), methylphenidate (to treat deficit in attention and inhibition)but both of these drugs have not been used in CCAS [8-10]. The previous case of CCAS reported by us suggested that Selective Serotonin Reuptake Inhibitor (SSRIs) may be useful in treating mood disturbances [11]. Clomipramine, a tricyclic antidepressant, had also been reported to be effective [12]. Our case responded to SSRI (fluoxetine).

The present case of CCAS presented with depressive features which responded to fluoxetine. So, it is important to screen the patients with CCAS for mood disturbances so that they can be timely treated.

There is need of future studies to find out the treatment modalities and long-term effects of CCAS.

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