关键词: Fanconi anemia cerebroretinal microangiopathy with calcifications and cysts (CRMCC) idiopathic basal ganglia calcification intracranial calcification psychosis

来  源:   DOI:10.1002/pcn5.10   PDF(Pubmed)

Abstract:
UNASSIGNED: Patients with Fanconi anemia (FA) are at high risk for the development of malignancies, and are often treated with radiation therapy. Radiation therapy during childhood can cause intracranial calcification after a latent period, which has been associated with psychiatric symptoms. Despite the high sensitivity of patients with FA to radiation, intracranial calcification has rarely been reported in these patients.
UNASSIGNED: A 17-year-old girl presented with psychiatric symptoms and cognitive impairment. She had been diagnosed with FA at 3 years old, and had received a bone marrow transplant at 5 years old with a preparative regimen that included total body irradiation. Results of IQ tests revealed a characteristic pattern of decline between the ages of 15 and 17 years. Computed tomography indicated multiple intracranial calcifications in regions associated with psychotic symptoms, including the frontal lobe and thalamus. The patient\'s psychiatric symptoms improved with the administration of blonanserin.
UNASSIGNED: The patient did not have regular intracranial imaging, making it difficult to confirm a direct relationship between intracranial calcification, psychiatric symptoms, and cognitive impairment. It is unclear whether the intracranial calcification in this case can be explained entirely by irradiation.
UNASSIGNED: This case suggests a link between FA, intracranial calcification, and psychosis, in which intracranial calcification may have caused psychiatric symptoms. At present, evidence regarding the characteristics of psychiatric symptoms of intracranial calcification and its treatment is lacking. The current case will be helpful for elucidating the pathogenesis of this disorder and developing appropriate treatment protocols.
摘要:
范可尼贫血(FA)患者发展为恶性肿瘤的高风险,经常接受放射治疗。儿童时期的放射治疗可在潜伏期后引起颅内钙化,这与精神症状有关。尽管FA患者对辐射的敏感性很高,这些患者很少有颅内钙化的报道.
一名17岁女孩出现精神症状和认知障碍。她在3岁时被诊断出患有FA,并且在5岁时接受了骨髓移植,并采用了包括全身照射的准备方案。智商测试的结果表明,在15至17岁之间出现了下降的特征模式。计算机断层扫描显示与精神病症状相关的区域有多个颅内钙化,包括额叶和丘脑.患者的精神症状在服用布兰色林后得到改善。
患者没有定期颅内成像,很难确认颅内钙化之间的直接关系,精神症状,和认知障碍。目前尚不清楚这种情况下的颅内钙化是否可以完全通过照射来解释。
这种情况表明FA,颅内钙化,和精神病,其中颅内钙化可能引起精神症状。目前,缺乏有关颅内钙化的精神症状特征及其治疗的证据。目前的病例将有助于阐明这种疾病的发病机制并制定适当的治疗方案。
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