Psychiatric disorder in two siblings with hallervorden-spatz disease

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 d...

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Veröffentlicht in:Psychiatry investigation 2009, 6(3), , pp.226-229
Hauptverfasser: Sunwoo, Young-Kyung, Lee, Jeong-Seop, Kim, Won-Hyoung, Shin, Yong-Bum, Lee, Myung-Ji, Cho, In-Hee, Ock, Sun-Myeong
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Sprache:eng
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Zusammenfassung: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.
ISSN:1738-3684
1976-3026
DOI:10.4306/pi.2009.6.3.226