Department of Psychiatry, Inha University College of Medicine, Incheon, Korea.
Psychiatry Investig. 2009 Sep;6(3):226-9. doi: 10.4306/pi.2009.6.3.226. Epub 2009 Aug 3.
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.
Hallervorden-Spatz 病(HSD)是一种罕见的常染色体隐性遗传性疾病,其特征为早期出现进行性运动改变,包括肌张力障碍、僵硬、舞蹈手足徐动症和精神恶化。HSD还与各种精神症状相关,主要为抑郁和精神恶化。然而,精神病作为 HSD 的主要症状很少见。我们报告了两例以精神症状为主要临床表现的同胞,伴有共济失调和痉挛步态、构音障碍以及 HSD 的典型神经影像学表现。一名 14 岁女孩表现出复杂的运动抽搐、刻板行为和焦虑症状。她 16 岁的哥哥表现出明显的幻听、被害妄想和社会退缩症状。抗精神病药治疗后精神症状得到改善。HSD 是一种罕见疾病,但对于同时存在运动障碍和各种精神症状的患者,应仔细考虑该病的诊断。