Psychiatric Disorder in Two Siblings with Hallervorden-Spatz Disease.
- Author:
Young Kyung SUNWOO
1
;
Jeong Seop LEE
;
Won Hyoung KIM
;
Yong Bum SHIN
;
Myung Ji LEE
;
In Hee CHO
;
Sun Myeong OCK
Author Information
1. Department of Psychiatry, Inha University College of Medicine, Incheon, Korea.
- Publication Type:Case Report
- Keywords:
Hallervorden-Spatz disease;
Motor tics;
Psychiatric symptoms
- MeSH:
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
- From:Psychiatry Investigation
2009;6(3):226-229
- CountryRepublic 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.