The cerebellar
cognitive affective syndrome: case report
Liao JX, Zhen YM, Wei JR, Xiao YH, Zhang YQ, Li
CR
Shenzhen Children’s Hospital, Shenzhen, China
518026
Objective: To discuss the new
function of human cerebellum----the participation of cognition, language
and affection.
Case Report: A previously healthy
11-year old boy admitted to hospital for not to be so active as usual and
sleep more time for four days. He had had cough for 18 days and had been
diagnosed as pneumonia, and only few cough left. He looked malaise, did not
like to answer to anybody. His regulation of affect was impaired including
disinhibition and liability of affect with poor attentional and behavioral
modulation. Deficits in expressive language were characterized by brief responses,
lack of elaboration, reluctance to engage in conversation, long response
latencies and word-finding difficulties. In addition, on physical
examination he had all the manifestations of acute cerebellar ataxia. The
cerebral spinal fluid was normal. CT scan and MRI of the head were no
abnormal findings. On day 15, with 99mTc-ECD single photon
emission computed tomography (SPECT), the right cerebellum, bilateral
frontal pole, bilateral temporal area 38 and area 39 showed low perfusion.
The blood perfusion of right basal ganglia was higher than the left side.
Treated with corticosteroids for three days the condition modulated
progressively. One and half month later he got well completely.
Conclusions: This case suggested the
human cerebellum took part in language, cognition and affection of cerebral
advanced function. The cerebellar cognitive affective syndrome is
characterized by the following: 1. Disturbances of executive function. This
includes deficient planning, set-shifting, abstract reasoning, working
memory and decreased verbal fluency. 2. Impaired spatial cognition,
including visuospatial organization and impaired visuospatial memory. 3.
Personality change, characterized by flattening or blunting affect, and
disinhibited or inappropriate behavior. 4. Linguistic difficulties,
including dysprosodia, agrammatism and anomia. The net effect of these
disturbances in cognitive is a general lowering of overall intellectual
function.