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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 5  |  Issue : 1  |  Page : 71-74

Red herrings in the diagnosis of frontotemporal dementia: A case of probable bipolar disorder evolving into frontotemporal dementia


1 Department of Psychiatry, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India
2 Department of Psychiatry, Pondicherry Institute of Medical Sciences, Puducherry, India
3 Department of Psychiatry, K.G. Hospitals, Coimbatore, Tamil Nadu, India

Date of Web Publication27-Jun-2018

Correspondence Address:
Shiva Shanker Reddy Mukku
Department of Psychiatry, National Institute of Mental Health and Neurosciences, Bengaluru - 560 029, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jgmh.jgmh_1_18

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  Abstract 


Bipolar illness and Frontotemporal dementia (FTD) share many common features. The clinical features of mania such as excessive cheerfulness, hyper-sexuality and overspending can mimic impaired judgement and loss of inhibition seen in FTD. The depressive features such as anhedonia, decreased social interaction can mimic apathy associated with FTD. Among the FTD subtypes the behavioural variant of FTD (bv-FTD) can mimic a bipolar disorder, especially when it occurs in late life. Initially, patients with behavioural variant of FTD generally have behavioural changes with relatively preserved memory thus presenting a diagnostic challenge. The literature on bipolar disorder converting or progressing to FTD is limited to case reports and case series. In this case report we describe about an elderly gentleman with prior bipolar illness who later evolved into FTD. The diagnostic as well as management challenges have been discussed.

Keywords: Bipolar disorder, catatonia, frontotemporal dementia


How to cite this article:
Reddy Mukku SS, Simiyon M, Vanamoorthy U, Loganathan S, Varghese M. Red herrings in the diagnosis of frontotemporal dementia: A case of probable bipolar disorder evolving into frontotemporal dementia. J Geriatr Ment Health 2018;5:71-4

How to cite this URL:
Reddy Mukku SS, Simiyon M, Vanamoorthy U, Loganathan S, Varghese M. Red herrings in the diagnosis of frontotemporal dementia: A case of probable bipolar disorder evolving into frontotemporal dementia. J Geriatr Ment Health [serial online] 2018 [cited 2018 Dec 18];5:71-4. Available from: http://www.jgmh.org/text.asp?2018/5/1/71/235358




  Introduction Top


Bipolar disorder is a severe psychiatric illness with episodes of depression and mania or hypomania.[1] Mood disorders are an established risk factor for developing cognitive deficits.[2] Geriatric depression has been proposed as a risk factor and a prodrome of dementia.[3],[4] A registry-based study from Denmark reported that with each episode of depression, there is a 13% increase in risk for dementia and 6% in case of bipolar disorder.[5]

Frontotemporal dementia (FTD) is a degenerative condition with progressive atrophy of frontal and anterior temporal lobes.[6] FTD presents as behavioral disinhibition, impaired social cognition, and language dysfunction. The link between bipolar disorder and FTD is multifaceted. Patients with FTD often receive a primary psychiatric diagnosis before reaching the final confirmatory diagnosis, suggesting the various ways in which they may present to the clinician. It may be challenging to cross-sectionally differentiate apathy, behavioral disinhibition, and compulsive behaviors of FTD from a late-onset depression or hypomania of bipolar disorder. These psychiatric symptoms could either be a prodrome of dementia or can masquerade as a neuropsychiatric manifestation of an underlying degenerative condition.[7] Bipolar disorder patients who later developed behavioral variant (bv) of FTD have been reported earlier.[8],[9],[10] These two disorders are also genetically related with the presence of progranulin gene (GRN) polymorphism among a subset of patients in both these disorders.[11],[12],[13] The diagnosis of FTD can present as a challenge to the clinician as these patients predominantly have behavioral changes with little memory involvement. This may delay the diagnosis of FTD and such a diagnosis may be possible only with longitudinal observation. In this report, we describe about the diagnostic dilemma and management issues in an elderly gentleman with bipolar disorder who evolved later into FTD.


  Case Report Top


Mr. T, a 65-year-old gentleman, was brought to our psychiatry outpatient department in 2014 (a tertiary care facility at Bengaluru) with complaints of sadness, anhedonia, decreased sleep, and suicidal ideas for 8 months. On clarification, it was found that he was premorbidly hyperthymic and had a family history of a depressive illness in his father and son. He was also being treated for hypertension and dyslipidemia. Psychiatric history revealed that he had his first depressive episode when he was 55 years old (in 2004), which was severe in nature with psychotic symptoms. So far, he had three episodes of depression (in 2004, 2005, and 2008) and a brief hypomanic episode in 2008. He maintained well till 2012 while he was on sodium valproate tablets at 1 g/day. In February 2012, he had a brief lasting hypomanic episode, and the dose of valproate was reduced to 750 mg/day in view of pedal edema. Later, he developed marked anxiety, easy fatigability, agitation, fidgetiness, pervasive low mood, and delusion of persecution. For these symptoms, the patient was treated elsewhere with the multiple antidepressants (escitalopram, desvenlafaxine, dothiepin, sertraline, pramipexole, and agomelatine at therapeutic doses) and also received 16 sessions of electroconvulsive therapy (ECT). With no response to the above treatments, he was brought to our hospital in July 2013. A general physical examination including a detailed neurological evaluation was normal. On mental status examination, Mr. T was agitated, with depressive cognitions, delusion of persecution, anxious mood, and intact memory. At this stage, a diagnosis of bipolar affective disorder current episode severe depression with psychotic symptoms was considered. During inpatient care, he would not comply with any activity, including activities of daily living (ADL), and on few occasions, he had disrobed himself. He was started on quetiapine tablets (dose 200 mg/day), and later magnesium valproate, 1 g tablet was added. Psychotherapeutic and behavioral interventions could not be initiated as Mr. T was not cooperative. Magnesium valproate was stopped due to hyponatremia, and only quetiapine was continued. With the above medications and seven ECTs, he had improved by about 50% in terms of agitation, suicidal ideas, and ADL and was discharged in October 2013. During the ECT sessions, there were no episodes of confusion or disorientation noticed. Formal cognitive assessment is routinely done at our hospital but was not carried out as the patient was in catatonia and uncooperative.

He returned back to us 2 months later with symptoms of severe depression and stubborn behavior, such as actively resisting any activity, and he was also found to have positive palmomental reflex and glabellar reflex. A neuropsychological assessment was done at this stage that suggested impaired focal and sustained attention, phonemic fluency, set shifting, response inhibition, planning, and impaired lexical fluency, with intact memory. He scored 29 in frontal behavioral inventory [14] and 28/31 in Hindi mental status examination.[15] Magnetic resonance imaging (MRI) brain (plain) showed mild atrophic changes in the frontotemporal areas and dilated ventricles [Figure 1]. Laboratory investigations for a dementia workup were as shown in [Table 1]. The treating team now considered a diagnosis of bv-FTD.
Figure 1: Magnetic resonance imaging brain-T2 and flair sequence showing atrophy of frontal lobe and mild atrophy of temporal lobes

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Table 1: Laboratory results

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Mr. T was readmitted again in October 2014 as he refused to take food. During serial mental status examination, Mr. T had the following catatonic symptoms: negativism, gegenhalten, rigidity, mutism, and decreased food intake. He was given 10 ECT sessions which led to significant improvement in catatonic symptoms. During the last follow-up in 2015, patient's mood symptoms were better, but he continued to have apathy, difficulties in ADL, few episodes of unconcerned urinary continence, and cognitive decline. During the last follow-up, he was maintained on tianeptine 12.5 mg tablets given thrice daily and donepezil 10 mg tablet once daily. The family now moved back to Pune where they originally came from for treatment and have not been seen at follow-up since then. We are hoping to see him in future follow-ups and assess his progress.


  Discussion Top


The patient predominantly had depressive episodes with few hypomanic episodes in the past suggestive of an initial presentation of bipolar disorder. The subsequent presentations which lead to suspicion of organic brain disorder were apathy, catatonic symptoms, presence of primitive reflexes, and treatment nonresponse to depression. All these lead to further evaluation such as MRI-brain, fluorodeoxyglucose positron emission tomography, and neuropsychological assessment. The diagnosis of FTD was made based on continuous progressive illness since 2012, apathetic behavior, difficulty in ADL, MRI brain suggestive of bilateral atrophy of frontotemporal lobes, and findings on neuropsychology testing. A recently conducted meta-analysis suggested a definite risk of dementia in patients with bipolar disorder.[16],[17] As far as FTD was concerned, there were no longitudinal studies looking at bipolar disorder evolving into FTD. In a case series of four patients with bipolar disorder, a progression to apathy, disinhibition, stereotypical behavior, and compulsive behavior were noted. Among these cases, despite a follow-up of 3–7 years, there was a minimal progression to dementia, and they were considered as FTD mimics.[18] This case may possibly be the first case yet that may suggest a progression to frank FTD (since memory impairment and other findings are substantial) though more thorough follow-up is required.

The catatonic symptoms such as limited speech output, stereotypes, preservation, and gegenhalten can also occur as presenting feature of FTD.[19],[20] These symptoms respond to interventions that depend on the etiology for catatonia. It could be possible that in our patient, the catatonic symptoms were a late feature that had an organic etiology, in this case FTD. In our patient, the catatonic symptoms responded to ECT, a well-established treatment for catatonia. Despite improvement in catatonic symptoms, apathy and cognitive deficits that indicated underlying FTD continued to persist.

In a retrospective study of 252 patients with dementia, it was found that patients with a diagnosis of bv-FTD significantly more often (50.7%) received a psychiatric diagnosis, when compared to patients with Alzheimer's dementia (23.1%), semantic dementia (24.4%), or progressive nonfluent aphasia (11.8%). In the same study, bipolar disorder or schizophrenia was the likely diagnosis made in FTD compared to other neurodegenerative disease.[7] There were two cases reported who initially presented with late-onset bipolar disorder but later diagnosed as bv-FTD based on persistent cognitive deficits and neuroimaging finding.[8],[9] There was another reported case with long-standing bipolar disorder who came with manic relapse at 68 years. During the episode, she was found to have cognitive impairment and decline in personal conduct and finally diagnosed as bv-FTD.[10] In a case series, Papazacharias et al. described two patients with long-standing bipolar disorder who later developed FTD. The first case was a 56-year-old woman with Bipolar Affective disorder (BPAD) Type I, who presented with affective symptoms, and language impairment, the diagnosis was revised to primary progressive aphasia variant of FTD (PPA-FTD) based on imaging and neuropsychological deficits. The second case was a 53-year-old man with BPAD Type II presented later with gait disturbances and cognitive deficits; he was finally given a diagnosis of FTD with  Parkinsonism More Details.[21]

From our case example and discussion, there is a substantial occurrence of psychiatric symptoms during the earlier phase of FTD. In this case, a retrospective symptom analyses suggest that the patient's stubborn behavior was probably a form of negativism (catatonia) early on, episodes of disrobing that could have been disinhibition (of FTD) and the apathy itself being misconstrued and treated as depression all along, which had not responded to several antidepressant medications. It could be possible that the earlier hypomanic symptoms could have indeed been a manifestation of disinhibition of FTD. However, it may still be possible that Mr. T could probably be suffering from both of these illnesses – bipolar disorder and FTD. Following up with the patient may well answer this question. The signs and symptoms that probably help in differentiating the psychiatric disorder from FTD are as follows: onset after 45 years, insidious onset, progressive course, cognitive deficits, catatonic symptoms, and poor response to psychotropic medication.


  Conclusion Top


There are shared clinical features between FTD and mood disorder. This case highlights challenges in diagnosing FTD in a patient with prior history of psychiatric disorder. One must also be cautious in dealing with unexplained behavioral change and differentiating catatonic symptoms in late life.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Galimberti D, Prunas C, Paoli RA, Dell'Osso B, Fenoglio C, Villa C, et al. Progranulin gene variability influences the risk for bipolar I disorder, but not bipolar II disorder. Bipolar Disord 2014;16:769-72.  Back to cited text no. 11
    
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Ganguli M, Ratcliff G, Chandra V, Sharma S, Gilby J, Pandav R, et al. A Hindi version of the MMSE: The development of a cognitive screening instrument for a largely illiterate rural elderly population in India. Int J Geriatr Psychiatry 1995;10:367-77.  Back to cited text no. 15
    
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Papazacharias A, Lozupone M, Barulli MR, Capozzo R, Imbimbo BP, Veneziani F, et al. Bipolar disorder and frontotemporal dementia: An intriguing association. J Alzheimers Dis 2017;55:973-9.  Back to cited text no. 21
    


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