Psychiatry Investigation  2009;6(3):226-229

Psychiatric Disorder in Two Siblings with Hallervorden-Spatz Disease.

Young Kyung SUNWOO 1 ; Jeong Seop LEE ; Won Hyoung KIM ; Yong Bum SHIN ; Myung Ji LEE ; In Hee CHO ; Sun Myeong OCK

Affiliations

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Keywords

Hallervorden-Spatz disease; Motor tics; Psychiatric symptoms

Country

Republic of Korea

Language

English

Abstract

Hallervorden-Spatz disease (HSD) is a rare autosomal-recessive hereditary disorder characterized by the early onset of progressive movement alterations, including dystonia, rigidity, choreoathetosis, and mental deterioration. HSD is also associated with a variety of psychiatric symptoms, primarily depression and mental deterioration. However, psychosis has rarely been reported as a major symptom of HSD. We report two siblings who presented psychiatric symptoms as major clinical presentations, accompanied by ataxic and spastic gait, dysarthria, and typical neuroimaging findings of HSD. A 14-year-old girl presented complex motor tics, stereotypic behavior and anxiety symptoms. Her older brother, a 16-year-old boy, presented prominent auditory hallucinations, persecutory delusions and social withdrawal symptoms. Psychiatric symptoms were improved after atypical antipsychotic treatment. HSD is a rare disease but should be carefully considered in the diagnosis of patients with both motor disorder and various psychiatric symptoms.