Dement Neuropsychol 2018 March;12(1):75-79 Case Report DOI: 10.1590/1980-57642018dn12-010011 Recognizing Late Onset Frontotemporal Dementia with the DAPHNE scale A case report Leonardo Tafarello Martins1, Ivan Abdalla Teixeira1,2, Jerson Laks1,3,5, Valeska Marinho1,4 ABSTRACT. Frontotemporal dementias are classically described as early onset dementias with personality and behavioral changes, however, late onset forms can also be found. Considering the paucity of information about late onset behavioral variant frontotemporal dementia and its challenging diagnosis, we present a case report of an 85-year-old woman with behavioral changes and slow progression to dementia who was first diagnosed as having bipolar disorder and then Alzheimer’s disease. The Daphne scale provided a structured means to improve clinical diagnosis, also supported by characteristic features on MRI and SPECT, while CSF biomarkers ruled out atypical Alzheimer’s disease. Key words: frontotemporal dementia, late onset, scale, DAPHNE, bipolar disorder. RECONHECENDO A DEMÊNCIA FRONTOTEMPORAL DE INÍCIO TARDIO COM A ESCALA DAPHNE: UM RELATO DE CASO RESUMO. As demências frontotemporais são classicamente descritas como demências de início precoce com mudanças de personalidade e comportamento, porém as formas de início tardio também podem ser encontradas. Considerando a escassez de informações sobre a demência frontotemporal – variante comportamental de início tardio e o diagnóstico desafiador, apresentamos um relato de caso de uma mulher de 85 anos com alterações comportamentais e progressão lenta para demência que foi diagnosticada pela primeira vez com transtorno bipolar e, em seguida, doença de Alzheimer. A escala DAPHNE foi utilizada permitindo a estruturação das características clínicas, aumentando a precisão do diagnóstico clínico, apoiado por características em RM e SPECT, enquanto os biomarcadores no líquor descartaram a doença de Alzheimer. Palavras-chave: demência frontotemporal, início tardio, escala, DAPHNE, transtorno bipolar. rontotemporal dementias (FTD) are clas- tive/compulsive behaviors, hyperorality and Fsically described as early onset dementias, dysexecutive neuropsychological profile) are however, late onset forms account for up to required. ‘Probable’ bvFTD criteria include 40% of all cases,1 manifested by behavioral functional disability and characteristic neu- and personality changes and language distur- roimaging, whereas bvFTD ‘with definite bances.2 FTD includes three clinical presen- frontotemporal lobar degeneration’ requires tations: behavioral variant frontotemporal histopathological confirmation or a patho- dementia, semantic dementia, and progres- genic mutation.2 sive nonfluent aphasia. New consensus crite- Due to the major changes in personality ria establish that, for a diagnosis of ‘possible’ and behavior, bvFTD is frequently misdi- behavioral variant frontotemporal dementia agnosed as a primary psychiatric disorder.3 (bvFTD), three out of six clinically discrimi- An accurate differential diagnosis between nating features (disinhibition, apathy/iner- bvFTD and psychiatric disorders must be tia, loss of sympathy/empathy, persevera- established given the dramatic differences This study was conducted at the Center for Alzheimer’s disease and Related Disorders, Institute of Psychiatry, Universidade Federal do Rio de Janeiro, RJ, Brazil. 1Center for Alzheimer’s disease and Related Disorders, Institute of Psychiatry, Universidade Federal do Rio de Janeiro, RJ, Brazil. 2MSc Student Center for Alzheimer’s Disease and Related Disorders, Institute of Psychiatry, Universidade Federal do Rio de Janeiro, RJ, Brazil. 3 Associate Professor, Universidade do Estado do Rio de Janeiro, RJ, Brazil. 4PhD. Center for Alzheimer’s Disease and Related Disorders, Institute of Psychiatry, Universidade Federal do Rio de Janeiro, RJ, Brazil. 5Invited Professor, Postgraduate Program in Translational Biomedicine, Universidade do Grande Rio (Biotrans – Unigranrio). Valeska Marinho. Rua Dois de Dezembro, 38/802 – 22220-040 Rio de Janeiro RJ – Brazil. E-mail: [email protected] Disclosure: The authors report no conflicts of interest. Received November 30, 2017. Accepted in final form January 08, 2018. Martins et al. DAPHNE scale in late-onset FTD 75 Dement Neuropsychol 2018 March;12(1):75-79 in prognosis, therapeutic options, and family orienta- Contrasting with her previous parsimonious per- tion.3 As bvFTD diagnosis relies mainly on its clinical sonality and good financial organization, she started features, a behavioral inventory can help differentiating spending excessively, buying jewelry and expensive it from other diseases. Many behavioral inventories have clothes, lending money to others indiscriminately, and been used since the first diagnostic criteria for FTD were taking out bank loans. Also, although she had always published, including the Frontal Behavioral Inventory been homosexual, she started to offer sex to unknown (FBI),4 the Middelheim Frontality Score (MFS),5 and a men in the neighborhood, manifesting hypersexuality recently proposed tool based on the new bvFTD criteria, and masturbating herself in front of others. Simultane- called the DAPHNE scale.6 ously, her speech became loud and fast, and she claimed The DAPHNE (an acronym for Disinhibition, Apathy, feeling a lot of energy to do several activities at the same Perseverations, Hyperorality, Personal Neglect and loss time. Later, she presented persecutory delusions, accom- of Empathy) is a six-domain, ten-item scale designed as panied by auditory hallucinations, in which she believed a semi-structured interview. The first five domains were that her neighbors were plotting to kill her. Over the proposed from bvFTD Rascovsky criteria and the last ensuing months, she had sudden mood swings, cycling from the FBI. Each item can be scored on a five-point from euphoria to periods of great hopelessness, apathy, scale (none, very mild, mild, moderate, severe). The and tearfulness. These changes often occurred from one scale validation process was successful in differentiat- day to the next or even during the same day. At this ing bvFTD from non-bvFTD patients, as well as from point, she had no memory, language, or visuospatial dis- Alzheimer’s Disease, Progressive Supranuclear Palsy and turbances. She was first seen by a psychiatrist and diag- Bipolar patients.6 nosed as having bipolar mood disorder. There was no Differences in FTD clinical presentation according response or reasonable symptom control following any to age of onset have also been described.1 An overall of the treatments prescribed, despite use of an exten- worse neuropsychological performance, impairment sive list of psychiatric medicines, including antipsychot- in memory and visuospatial function, as well as more ics (ziprasidone, olanzapine, quetiapine, risperidone, symptoms of depression, apathy, and impulsiveness, thioridazine, periciazine, haloperidol, chlorpromazine, have been described in early onset FTD.1 Late onset paliperidone, clozapine), mood stabilizers (lamotrigine, forms have not been extensively described and may be lithium, divalproex), benzodiazepines (alprazolam, bro- less frequently diagnosed on clinical grounds and often mazepam, diazepam, clonazepam, chlordiazepoxide) misdiagnosed as Alzheimer’s disease. and antidepressants (sertraline, paroxetine, citalopram, Considering the paucity of information about late trazodone, fluoxetine, venlafaxine, duloxetine, clomip- onset bvFTD, we present a case report of an 85-year- ramine, nortriptyline, amitriptyline, mirtazapine), pre- old woman with marked changes in behavior and slow scribed either as monotherapy or combined therapy. progression over a decade to cognitive and functional At the age of 80 years, her mood became predomi- impairment, proving non-responsive to many pharma- nantly sad, anxious, and apathetic, she developed ste- cological therapies. The Daphne scale provided a struc- reotypical behaviors and compulsions of self-harm tured means to improve clinical diagnosis, including the (such as biting and beating herself, nail-biting and hair- challenging differentiation between bipolar disorder pulling), and repeating words or phrases out of context. and bvFTD. Support to confirm bvFTD diagnosis was When upset, she either threatened to commit suicide obtained by using neuroimaging and cerebrospinal fluid or displayed catastrophic reactions and voluntary falls. biomarkers. She started to complain of forgetfulness, with limited self-orientation outside the home and progressive loss CASE REPORT of autonomy, remaining more restricted to the domestic We present the case of Mrs. G, an 85-year-old retired environment. She gradually developed dependency for lawyer, without any previous history of psychiatric instrumental and basic activities of daily living, needing disorder, who began to present a dramatic behavioral help with her self-hygiene, choosing clothes and dress- change at the age of 75. ing, feeding herself and handling money. Throughout She had a previous medical history of hypertension the course of the disease, Mrs. G’s sleep remained pre- and stage I breast cancer at 71years of age (considered served with the use of benzodiazepines and there was cured after quadrantectomy, radiation therapy, and no significant weight change. Often, she did not recog- hormone blockade treatment), and a family history of nize caregivers or relatives, mistaking them as thieves. bipolar disorder (her older sister). An AD diagnosis was suggested by another psychiatrist,