Clinical Approach to the Differential Diagnosis Between Behavioral Variant Frontotemporal Dementia and Primary Psychiatric Disorders
Clinical Approach to the Differential Diagnosis Between Behavioral Variant Frontotemporal Dementia and Primary Psychiatric Disorders
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DOI：
10.1176/appi.ajp.2015.14101248
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发表时间：
2015-09-01
影响因子：
17.7
通讯作者：
Dickerson, Bradford C.
中科院分区：
文献类型：
--
作者：
Ducharme, Simon;Price, Bruce H.;Dickerson, Bradford C.
“A女士”，一名46岁的女性，被转介到麦克莱恩医院强迫症研究所（OCDI）接受住院治疗。她之前没有精神疾病史。两年前，她养成了清洁和检查的习惯，这些习惯逐渐变得严厉和侵扰，以至于失去了她的长期工作。她被一位社区精神科医生诊断为强迫症。医生开了舍曲林，但由于副作用，她很快就停了药。在OCDI呆了几天后，由于强迫症的非典型特征，包括发病年龄较晚和无法接受治疗，医生要求进行神经精神诊断评估。A女士否认有强迫症或焦虑症。她感觉很好，并不担心失去工作。从她丈夫处获得了间接病史。在强迫症发作后，他注意到她的性格发生了重大变化。A女士以前很有爱心，有条理，可靠，但在过去的2年里，她变得情感疏远和冷漠，现在她判断力差，忽视了她的女儿。她还表现出奇怪的饮食行为，比如一勺一勺地吃黄油。最近，她形成了双手摩擦的刻板印象。没有精神疾病、痴呆或肌萎缩侧索硬化症的家族史。在检查时，患者表面上是合作的，但对自己的行为没有洞察力、意识或担忧。她的感情是愉快而浅薄的。她对家里的事漠不关心。没有精神症状或语言异常。她在蒙特利尔认知评估中的得分为25/30（视觉空间和执行任务为3/5，系列7任务为4/5，回忆任务为3/5，有线索的12个单词），她在正面评估电池中的得分为17/18。在音位流畅性测试中，她列出了12个单词，在前15秒背诵的单词比在后45秒背诵的单词多（代际缺陷）。进行简短的社会认知测试。她在失礼的小插曲上没有犯错，但在“通过眼睛读心”测试中，她只识别出了17/36种情绪。她的基本神经学检查正常，除了难以消除的双侧手掌反射。虽然A女士的强迫行为是典型的强迫症，但临床表现与这种诊断不符，因为她没有与这些仪式相关的焦虑或痛苦。考虑到患者的移情能力丧失、冷漠、粗心行为、饮食改变、搓手习惯、生成缺陷和面部情绪识别受损等病史，她符合可能的行为变异性额颞叶痴呆（bvFTD）的临床标准。据报道，最近在一家私人机构进行的脑MRI检查正常（图像无法查阅）。然而，在我们医院的另一次脑部MRI显示双侧中度至重度局限性腹内侧前额叶和眶额皮质萎缩。一项[18F]氟脱氧葡萄糖正电子发射断层扫描研究显示，在前额叶内侧有明显的皮质代谢低下。血液学、生化和脑电图结果均在正常范围内。最终诊断可能为bvFTD。遗传评估显示没有常染色体显性突变与额颞叶痴呆相关（C9ORF72、前颗粒蛋白和MAPT）。在接下来的几个月里，他在家里的攻击性行为变得更加突出。双丙戊酸钠开始用于行为管理。尽管服用了药物，A女士的行为还是持续恶化，而且她…
“Ms. A,” a 46-year-old woman, was referred for inpatient treatment at the Obsessive-Compulsive Disorder Institute (OCDI) of McLean Hospital (Belmont, Mass.). She had no prior history of psychiatric disorders. Two years earlier she had developed cleaning and checking rituals that gradually became severe and intrusive to the point of losing her longterm employment. She had been diagnosed with obsessivecompulsive disorder (OCD) by a community psychiatrist. Sertraline was prescribed, but she soon stopped taking it because of side effects. After a few days at the OCDI, a diagnostic neuropsychiatric assessment was requested because of atypical features for OCD, including a late age at onset and an inability to engage in therapy. Ms. A denied obsessions or anxiety. She felt well and had no concerns about having lost her job. Collateral history was obtained from her husband. After the onset of compulsions, he noticed major changes in her personality. Ms. A had previously been caring, organized, and reliable, but over the past 2 years she had become emotionally detached and apathetic, and she now had poor judgment and neglected her daughter. She also exhibited odd eating behaviors, such as eating butter by the spoonful. Most recently she had developed bilateral hand-rubbing stereotypies. There was no family history of psychiatric disorders, dementia, or amyotrophic lateral sclerosis. On examination, the patient was superficially cooperative, but without insight, awareness, or concern about her behavior. Her affect was jovial and shallow. She was indifferent to her family’s concerns. There were no psychotic symptoms or language abnormalities. Her score on the Montreal Cognitive Assessment was 25/30 (3/5 in the visuospatial and executive tasks, 4/5 on the serial 7s task, 3/5 on the recall task, 12 words with cues), and her score on the Frontal Assessment Battery was 17/18. On phonemic fluency testing, she listed 12 words, reciting more words in the first 15 seconds than in the last 45 seconds (generation deficit). A brief social cognition battery was administered. She made no mistakes on faux-pas vignettes, but identified only 17/36 emotions on the Reading the Mind in the Eyes test. Her elemental neurological examination was normal except for inextinguishable bilateral palmomental reflexes. While Ms. A’s compulsions were typical of OCD, the clinical profile was not compatible with this diagnosis because of the absence of anxiety or distress related to the rituals. Given the history of loss of empathy, apathy, careless actions, dietary changes, hand-rubbing stereotypies, generation deficits, and impaired facial emotion recognition, she met clinical criteria for possible behavioral variant frontotemporal dementia (bvFTD). A recent brain MRI performed at a private facility was reported as normal (images were not available for review). However, another brain MRI, at our hospital, showed moderate to severe circumscribed ventromedial prefrontal and orbitofrontal cortical atrophy bilaterally. A [18F] fluorodeoxyglucose positron emission tomography study demonstrated marked cortical hypometabolism within the medial aspects of the anterior frontal lobes. Hematology, biochemistry, and EEG results were within normal limits. The final diagnosis was probable bvFTD. A genetic evaluation revealed none of the autosomal dominant mutations associated with frontotemporal dementia (C9ORF72, progranulin, and MAPT).Over the next few months, aggressive behaviors at home became more prominent. Divalproex sodium was initiated for behavioral management. Despite the medication, Ms. A’s behavior continued to deteriorate, and she …