Abstract

We report a case of 30-year-old man presenting chorea in his legs. A brain computed tomography (CT) scan showed bilateral symmetric calcifications in the basal ganglia, thalamus, cerebellum and subcortical white matter. Laboratory studies showed no abnormalities of serum calcium, phosphate, PTH, lactic acid, pyruvic acid and cerebrospinal fluid. Under the diagnosis of Fahr's disease (FD), we treated with quetiapine (75 mg/day), which completely abolished his symptoms and he showed no other side effect. Our experience suggests that quetiapine is well tolerated in FD patients and effectively treats chorea without extrapyramidal movement.

Highlights

  • Fahr’s disease (FD) refers to a sporadic or familial idiopathic calcification of the basal ganglia that may lead to neurological, psychiatric, and cognitive abnormalities

  • We report a case of FD presenting chorea successfully treated by the use of quetiapine, atypical antipsychotic drug

  • FD is a rare clinical entity characterized by nonarteriosclerotic calcification of the striopallidodentate system bilaterally without blood calcium or hormone level abnormalities.[1]

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Summary

Introduction

Fahr’s disease (FD) refers to a sporadic or familial idiopathic calcification of the basal ganglia that may lead to neurological, psychiatric, and cognitive abnormalities. Patients with FD mostly present with movement disorders such as parkinsonism, chorea, tremor, dystonia, dysarthria, paresis, or speech impairment.[1,2] Other common neurological features are seizures, syncope, or stroke-like events, often combined with a frontal subcortical pattern of behavioral dysfunction and psychiatric symptoms such as psychosis, mood disorders, and dementia.[3,4] The origin and pathophysiology of this disorder are unknown, as is the reason why other cases with basal ganglia calcification remain asymptomatic.[5,6] Treatment of FD is not fully documented.

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