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KMID : 1118520090060030226
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Psychiatry Investigation
2009 Volume.6 No. 3 p.226 ~ p.229
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Psychiatric Disorder in Two Siblings with Hallervorden-Spatz Disease
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Sunwoo Young-Kyung
Lee Jung-Sub
Kim Won-Hyung
Shin Yong-Bum
Lee Myung-Ji
Cho In-Hee
Ock Sun-Myeong
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Abstract
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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.
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KEYWORD
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Hallervorden-Spatz disease, Motor tics, Psychiatric symptoms
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