Psychiatry Investig.  2009 Sep;6(3):226-229.

Psychiatric Disorder in Two Siblings with Hallervorden-Spatz Disease

Affiliations
  • 1Department of Psychiatry, Inha University College of Medicine, Incheon, Korea.
  • 2Department of Psychiatry, Gachon University of Medicine and Science, Incheon, Korea.
  • 3Department of Family Medicine, College of Medicine, The Catholic University of Korea, St. Mary's Hospital, Seoul, Korea. soulfree@catholic.ac.kr

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.

Keyword

Hallervorden-Spatz disease; Motor tics; Psychiatric symptoms

MeSH Terms

Adolescent
Anxiety
Delusions
Depression
Dysarthria
Dystonia
Gait Disorders, Neurologic
Hallucinations
Humans
Neuroimaging
Pantothenate Kinase-Associated Neurodegeneration
Psychotic Disorders
Rare Diseases
Siblings
Substance Withdrawal Syndrome
Tics
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