目的:严重的神经认知障碍(NCD)是一种具有多种病因的异质性疾病。大多数确诊病例的来源不明。因此,Fahr综合征(FS)是一种罕见的神经退行性疾病,临床上表现为不同的神经系统,神经精神病学,和运动障碍特征。FS的特征是继发于其他疾病的双侧基底神经节钙化(例如,内分泌和代谢紊乱,传染病)。此案将扩展到NCD的罕见和多病因表现的文献。
方法:一名75岁的白人男性因妻子担心突然出现情绪减退而接受了神经心理学评估,快感缺失,失眠症,去抑制,和两年前的特殊运动,在感染COVID-19后恶化。患者患有多种共病,包括甲状腺功能减退,高胆固醇血症,中度高血糖,前列腺癌(缓解期),和晕厥事件的历史。双侧基底神经节钙化和白质病变的神经影像学检查显着。
结果:在考试中,患者表现为低omimia,共济失调步态,alogia,零星的运动刻板印象,和电机溢出。迈尔斯神经心理电池的整体表现低于平均水平,注意力相对较弱,记忆,处理速度,心理灵活性,和电机功能。患者被诊断为病因不明的主要NCD,并被指示继续与神经科会诊。
结论:该患者的认知困难表现为多病因,可能归因于基底节钙化的组合,白质变化,甲状腺功能减退,和COVID-19,这表明存在FS。这个案例说明了考虑多种病因的重要性,包括COVID-19在诊断过程中的影响,因为仍在发现更多的COVID-19的神经系统症状。
OBJECTIVE: Major neurocognitive Disorder (NCD) is a largely heterogeneous condition with numerous etiological possibilities. Most diagnosed cases have unspecified origins. As such, Fahr\'s syndrome (FS) is a rare neurodegenerative condition that clinically presents with varying neurologic, neuropsychiatric, and movement disorder features. FS is characterized by bilateral basal ganglia calcifications secondary to other conditions (e.g., endocrine and metabolic disorders, infectious diseases). This
case will expand upon the literature of rare and poly-etiological presentations of NCD.
METHODS: A 75-year-old White male underwent neuropsychological evaluation due to concerns from his wife regarding sudden onset of diminished mood, anhedonia, hypersomnia, disinhibition, and idiosyncratic motor movements two years prior which worsened after contracting COVID-19. Patient has multiple co-morbid medical conditions including hypothyroidism, hypercholesterolemia, intermediate hyperglycemia, prostate cancer (in remission), and history of a syncopal episode. Neuroimaging was remarkable for bilateral basal ganglia calcifications and white matter lesions.
RESULTS: At the exam, patient exhibited hypomimia, ataxic gait, alogia, sporadic motor stereotypies, and motor overflow. Overall performance on the Meyers Neuropsychological Battery was Below Average with relative weaknesses in attention, memory, processing speed, mental flexibility, and motor functioning. Patient was diagnosed with Major NCD of unspecified etiology and instructed to continue consultation with neurology.
CONCLUSIONS: This patient\'s cognitive difficulties appeared poly-etiological and are likely attributed to the combination of basal ganglia calcification, white matter changes, hypothyroidism, and COVID-19 which suggests the presence of FS. This
case exemplifies the importance of considering multiple etiologies, including the implications of COVID-19 during the diagnostic process as more neurologic symptoms of COVID-19 are still being discovered.