关键词: Alzheimer’s disease GRN-related frontotemporal dementia personality disorders phenotype psychiatric disorders

Mesh : Female Humans Frontotemporal Dementia / diagnostic imaging genetics Narcissistic Personality Disorder Brain / diagnostic imaging pathology Magnetic Resonance Imaging Neuropsychological Tests Progranulins

来  源:   DOI:10.3233/JAD-230779

Abstract:
UNASSIGNED: Behavioral variant frontotemporal dementia (bvFTD) typically involves subtle changes in personality that can delay a timely diagnosis.
UNASSIGNED: Here, we report the case of a patient diagnosed of GRN-positive bvFTD at the age of 52 presenting with a 7-year history of narcissistic personality disorder, accordingly to DSM-5 criteria.
UNASSIGNED: The patient was referred to neurological and neuropsychological examination. She underwent 3 Tesla magnetic resonance imaging (MRI) and genetic studies.
UNASSIGNED: The neuropsychological examination revealed profound deficits in all cognitive domains and 3T brain MRI showed marked fronto-temporal atrophy. A mutation in the GRN gene further confirmed the diagnosis.
UNASSIGNED: The present case documents an unusual onset of bvFTD and highlights the problematic nature of the differential diagnosis between prodromal psychiatric features of the disease and primary psychiatric disorders. Early recognition and diagnosis of bvFTD can lead to appropriate management and support for patients and their families. This case highlights the importance of considering neurodegenerative diseases, such as bvFTD, in the differential diagnosis of psychiatric disorders, especially when exacerbations of behavioral traits manifest in adults.
摘要:
行为变异额颞叶痴呆(bvFTD)通常涉及人格的细微变化,可能会延迟及时诊断。
这里,我们报道了一例52岁时诊断为GRN阳性bvFTD的患者,有7年的自恋型人格障碍病史,根据DSM-5标准。
患者接受神经和神经心理学检查。她接受了3次特斯拉磁共振成像(MRI)和遗传研究。
神经心理学检查显示所有认知领域都存在严重缺陷,3T脑MRI显示出明显的额颞叶萎缩。GRN基因的突变进一步证实了该诊断。
本病例记录了bvFTD的异常发作,并强调了该疾病的前驱精神病特征与原发性精神疾病之间的鉴别诊断的问题。早期识别和诊断bvFTD可以为患者及其家人提供适当的管理和支持。这个案例突出了考虑神经退行性疾病的重要性,例如bvFTD,在精神疾病的鉴别诊断中,特别是当成年人的行为特征恶化时。
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