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Psychiatry Investig. 2009 Sep;6(3):226-9. doi: 10.4306/pi.2009.6.3.226. Epub 2009 Aug 03.

Psychiatric disorder in two siblings with hallervorden-spatz disease.

Psychiatry investigation

Young-Kyung Sunwoo, Jeong-Seop Lee, Won-Hyoung Kim, Yong-Bum Shin, Myung-Ji Lee, In-Hee Cho, Sun-Myeong Ock

Affiliations

  1. Department of Psychiatry, Inha University College of Medicine, Incheon, Korea.

PMID: 20046400 PMCID: PMC2796072 DOI: 10.4306/pi.2009.6.3.226

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.

Keywords: Hallervorden-Spatz disease; Motor tics; Psychiatric symptoms

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