Fahr病(FD)是一种罕见的疾病,以基底神经节钙化为特征,表现为运动障碍,言语障碍,认知缺陷,神经精神症状.与FD相关的精神障碍在文献中几乎没有描述,关于病理生理学的知识缺失,当然,和管理。这里,我们报道了一名患者的长期随访,该患者有三次FD-精神病急性发作,其特征是奇怪的妄想和行为紊乱,没有幻觉.排除了FD的遗传和代谢原因。在所有三集中,奥氮平单药治疗迅速和完全解决精神病,不诱发代谢综合征和锥体外系症状。除了急性代偿失调,病人提出了一个驯服,内向,勤劳,和完美主义人格,我们可以将其解释为许多其他基底神经节疾病所描述的“帕金森人格”。此外,怪异的外表,减少情感,Abulia,具体讲话,在轻度不对称锥体外系综合征的背景下,表现为精神状态。认知特征最初以执行困难和部分失认症为标志,智商为86。在十年的时间里,患者患有左颞上回缺血性中风,这导致了记忆力和执行功能的下降,对精神病患者的情况没有任何影响.抗磷脂抗体综合征是根本原因;因此,在文献中第一次,本文描述了FD和抗磷脂抗体综合征的重叠。此病例报告再次强调需要更好地整合精神病学和神经病学,以及研究迟发性精神病的次要原因。
Fahr\'s disease (FD) is a rare disorder, characterized by basal ganglia calcification and presenting with movement disorders, speech impairment, cognitive deficits, and neuropsychiatric symptoms. Psychotic disorders related to FD are barely described in the literature, and knowledge is missing concerning pathophysiology, course, and management. Here, we report on the long-term follow-up of a patient who had three acute episodes of FD-psychosis characterized by bizarre delusions and behavioral disorganization, without hallucinations. Genetic and metabolic causes of FD were ruled out. In all three episodes, olanzapine monotherapy rapidly and completely resolved psychosis, without inducing metabolic syndrome and extrapyramidal symptoms. In addition to the acute decompensations, the patient presented a tame, introverted, industrious, and perfectionistic personality, which we could interpret as the \"parkinsonian personality\" described for many other basal ganglia disorders. Moreover, bizarre appearance, reduced affectivity, abulia, concrete speech, and stiff motricity in the context of a mild asymmetric extrapyramidal syndrome characterized the mental status. The cognitive profile was initially marked by executive difficulties and partial agnosia, with an IQ of 86. In the course of 10 years, the patient suffered from an ischemic stroke in the left superior temporal gyrus, which provoked a decline in memory and executive functions, without any impact on the psychiatric picture. Antiphospholipid antibody syndrome emerged as the underlying cause; thus, for the first time in the literature, an overlap of FD and antiphospholipid antibody syndrome is described here. This case report stresses once more the need for better integration of psychiatry and neurology and for the investigation of secondary causes of late-onset psychosis.