Objective: Fahr's disease (FD), as known as bilateral striopallidodentate calcification, is a rare clinical entity characterized by movement disorders, psychiatric symptoms, and neuropsychological impairments. Though clinical findings in FD are heterogeneous, in terms of cognitive function, the impairments in psychomotor speed, attention, memory, executive function, and intelligence are more consistently reported in the literature. However, there is little literature on the social function of FD. Thus, this case report represents a detailed cognitive and social function evaluation result of a patient with FD in two-year follow-up, in order to add more information on the picture of FD.
Method: This 40-year-old unmarried male with 12 years of schooling had a history of delayed developmental milestones and seizure attacks. Poor appetite and bizarre behaviors had been observed at age 19. Then he was brought to armed forces hospital and diagnosed as major depression with psychosis. At age 26, he suffered from visual hallucination (VH), followed by auditory hallucination (AH), delusion of persecution, and abnormal behavior. He admitted to our acute psychiatric ward at age 28 with the diagnosis of schizophrenia, paranoid type, and then has been settled in our chronic ward for long-term rehabilitation. During the hospitalization, social withdraw, self-talking, delusion, AH, VH, illogical thinking, anxious, psychomotor slowing, and poor self-care have been observed continually. Clozaril, clopine, ativan, and sinzac have been used to ameliorate above symptoms. Rigidity of limbs, several falls, and gait disturbance have also been reported since his 30s. Brain CT taken in 2010 showed massive diffuse calcification over dentate nucleus, bilateral basal ganglia, thalamus, periventricle, and cerebral cortex, and therefore Fahr's disease has been suggested. Laboratory tests revealed a decreased level of serum calcium and potassium, but normal thyroid, parathyroid, and pituitary function. Based on the finding of Brain CT and laboratory tests, several psychological tests were administered in 2010. The result disclosed a borderline level of intelligence, and deficits in processing speed, visuospatial organization, memory, executive function, attention, and some social mental functions including facial recognition ability, empathetic ability, and motive. However, comparing these results with previous neuropsychological examination taken in 2008, a selective deterioration of working memory and verbal episodic memory, and an improvement of social mental function were found.
Conclusion: This case report provides a cognitive deficits pattern of FD which is consistently with the past literature. It has been known that subcortical system is associated with some social function (e.g., motivation), but there is limited study conducted in the social function of FD. Hence, this report provides an initial exploration on the picture of FD's social function. Future research is suggested to get more understanding on the social function of FD.