Manic behavior and asymmetric right frontotemporal dementia from a novel progranulin mutation

Neuropsychiatric Disease and Treatment Dovepress open access to scientific and medical research C a s e r e p o rt Neuropsychiatric Disease and Treatment downloaded from https://www.dovepress.com/ by 37.59.46.207 on 12-Jul-2018 For personal use only. Open Access Full Text Article Manic behavior and asymmetric right frontotemporal dementia from a novel progranulin mutation This article was published in the following Dove Press journal: Neuropsychiatric Disease and Treatment Mario F Mendez 1–3 Department of Neurology, Department of Psychiatry & Biobehavioral Sciences, David Geffen School of Medicine, University of California at Los Angeles, Los Angeles, CA, USA; 3Neurology Service, VA Greater Los Angeles Healthcare System, Los Angeles, CA, USA 1 2 Abstract: Studies suggest a relationship of manic behavior and bipolar disorder (BD) with behavioral variant frontotemporal dementia (bvFTD). The nature of this relationship is unclear. This report presents a patient with initial manic behavior as the main manifestation of familial bvFTD from a novel progranulin (GRN) mutation. In contrast, there are other reports of a long background of BD preceding a diagnosis of bvFTD. A review of the literature and this patient suggest that manic symptoms result from damage to right frontotemporal neural structures from longstanding BD, as well as from bvFTD and other focal neurological disorders. In addition, there is a subgroup of patients with a probable genetic predisposition to both BD and bvFTD. Keywords: frontotemporal dementia, mania, bipolar disorder, progranulin mutation Introduction Behavioral variant frontotemporal dementia (bvFTD) is a neurodegenerative disorder that can present with subtle alterations of personality.1 The onset is usually insidious in mid-life, with progression to severe dementia caused by atrophy of the frontal and anterior temporal lobes. Most commonly, patients develop apathy or abulia, disinhibition or impulsivity, loss of sympathy or empathy, compulsions and stereotypies, and dietary changes, particularly carbohydrate cravings.1 Many, if not most, patients with bvFTD present to psychiatrists and other mental health specialists with concerns over depression, anxiety, mid-life crisis, and other conditions, before a neurodegenerative brain disorder is considered.2 The early diagnosis and management of bvFTD depends on recognizing the range of initial and presenting signs and symptoms of this disorder. This report presents a familial bvFTD patient who initially developed manic symptoms, and reviews the literature on the relationship of bipolar symptoms and bvFTD. Written informed consent was provided by the patient’s husband, who is the legal guardian for this case report and any accompanying images. Case report Correspondence: Mario F Mendez Neurobehavior (691/116AF), VA Greater Los Angeles Healthcare Center, 11301 Wilshire Blvd., Los Angeles, CA 90073, USA Tel +1 310 478 3711 ext 42696 Fax +1 310 268 4181 Email 657 submit your manuscript | www.dovepress.com Neuropsychiatric Disease and Treatment 2018:14 657–662 Dovepress © 2018 Mendez. This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php). http://dx.doi.org/10.2147/NDT.S156084 Powered by TCPDF (www.tcpdf.org) A 63-year-old right-handed woman with 16 years of education had a 2-year history of personality change, characterized as becoming increasing rude in conversation. Her husband reported that she would constantly interrupt and talk out of turn, speaking rapidly about topics tangential to the conversation. Her thoughts and speech would then go from one unrelated topic to another. This change was the husband’s main complaint, with an immediate precipitating event being the exclusion from her social group at her country club. Her husband described her as having an “acquired attention Dovepress Neuropsychiatric Disease and Treatment downloaded from https://www.dovepress.com/ by 37.59.46.207 on 12-Jul-2018 For personal use only. Mendez deficit hyperactivity behavior”, with decreased attention span and concentration, hyperactivity and fidgetiness, and restless inability to sit still for any prolonged period of time. He further described her as having diminished empathy or responsiveness to others’ feelings, and impulsive or inappropriate behavior, such as making offensive remarks and uncharacteristic arguments with peers. He observed her having difficulties with organization, multitasking, and following a sequence of steps to complete a task, such as cooking a meal. She was mostly independent in her routine and instrumental activities of daily living. The patient herself stated: “I am not aware of any changes. I just need to keep walking. I cannot sit still”. The review of psychiatric and neurological systems was negative for decreased need for sleep, increased goal-directed activity, risky behavior, compulsive-like acts, or dietary changes. Her past medical history was otherwise negative, but her family history was positive for an affected brother with an unknown “dementia” at age 68 and early dementia in her mother’s family (her mother died relatively young of colon cancer). On examination, she appeared restless, constantly moving, and hyperactive. Her speech was intrusive with tangential verbal output. She would quickly lapse into a totally unrelated story, going rapidly from one topic to another, appearing derailed by any transient idea or thought. Much of her rapid speech was imperfectly spoken with omitted words. Her mood and affect appeared euthymic, and there was no evidence of an expansive or euphoric mood, grandiosity, or inflated self-esteem. There was no evidence of a formal thought disorder or psychotic features. On her neurocognitive assessment, she was oriented in all spheres and had 26/30 on the Mini-Mental State Examination,3 missing memory items and the intersecting pentagons. When asked to slow down, however, she could do a good drawing, despite tending to rush through the tasks. Attention testing showed a digit span of 5 forward, but only 1 in reverse, and she was quickly derailed on any vigilance test. Her language was fluent, with normal comprehension, repetition, and confrontational naming, and she had word lists of 12 animals/min and nine “F” words/min. On an auditory verbal learning test, she recalled 7/10 words at 15 min, with recognition of 10/10. Her remote memory and knowledge of current events wer (...truncated)