|LETTER TO THE EDITOR
|Year : 2019 | Volume
| Issue : 2 | Page : 106-107
Heart and brain: A new association – Report of a family with “probable frontotemporal dementia and heart block”
Sadanandavalli Retnaswami Chandra, Pooja Mailankody, Manisha Gupta
Department of Neurology, NIMHANS, Bengaluru, Karnataka, India
|Date of Submission||12-Aug-2019|
|Date of Decision||29-Sep-2019|
|Date of Acceptance||13-Oct-2019|
|Date of Web Publication||20-Feb-2020|
Prof. Sadanandavalli Retnaswami Chandra
Faculty Block, Department of Neurology, Neurocentre, NIMHANS, Bengaluru - 560 029, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Chandra SR, Mailankody P, Gupta M. Heart and brain: A new association – Report of a family with “probable frontotemporal dementia and heart block”. J Geriatr Ment Health 2019;6:106-7
|How to cite this URL:|
Chandra SR, Mailankody P, Gupta M. Heart and brain: A new association – Report of a family with “probable frontotemporal dementia and heart block”. J Geriatr Ment Health [serial online] 2019 [cited 2020 Apr 8];6:106-7. Available from: http://www.jgmh.org/text.asp?2019/6/2/106/278743
Heart and brain are linked in several ways. There are enough data linking the heart and brain in vascular dementia., However, with reference to degenerative dementias, there is an involvement of cardiac amyloid in diffuse Lewy body disease, Alzheimer's disease which can cause cardiomyopathy. Ours is a report of familial frontotemporal dementia with heart block.
A 59-year-old patient was admitted with memory disturbances of a 7-year duration. She had reduced interactions, was unable read or write, had calculation problems, and lost the sense of time. she had problems in working memory and could not get involved in family functions.she had tendency for aimless wandering, was cooking rice without pouring water, was putting one dress over another and also wearing inside out, brushing and going to toilet repeatedly, had very poor personal hygiene and self care. Hindi Mental Status Examination score was 8/31. Positron emission tomography (PET) scan done outside showed bilateral frontotemporal hypometabolism, and her thyroid functions, liver functions, lipid profile, homocysteine, B12, human immuno deficiency virus (HIV), and veneral disease research laboratory (VDRL) test VDRL were normal. She was not amenable for neuropsychological testing in her mother tongue also in view of the very poor attention. Addenbrooke's cognitive evaluation revealed working memory decline-poor spatial span, digit span, and inability to do even simple calculations, and spontaneous speech was severely reduced. However, she had relatively preserved semantic memory and pragmatics, and comprehension for simple commands was adequate but for complex command was impaired. Repetition was impaired, confrontation naming was 5/10, category fluency was 4, and phonemic fluency was nil. Attention testing showed significant distractibility, but she could be interviewed. Recent memory was severely affected, and remote was moderately affected. In visuospatial testing, the patient could not draw even simple diagrams, but no definite agnosias were identified. Probable frontotemporal dementia was considered applying the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition Criteria. Cardiac evaluation showed a pacemaker rhythm. Echo showed a pacemaker seen through the right atrium and right ventricle. Echo cardiography revealed left ventricular diastolic dysfunction with Ejection fraction of 58%. PET scan showed frontotemporal hypometabolism, and magnetic resonance imaging showed frontotemporal atrophy [Figure 1]. However, we could not do voxel-based morphometry to demonstrate any specific insular region volume loss. Her father, aunt, sister, brother, and herself were on pacemaker at ages from 49 to 53 years for heart block [Figure 2]. They all in addition had memory and behavior problems as informed by her husband, but the affected members were not available for detailed evaluation. One sister had sepsis and died at 55 years. This suggests a strong association of frontotemporal dementia (FTD) and heart block in a dominantly inherited pattern in this family. This family might point to a new genotype of FTD.
|Figure 1: (a-c) Magnetic resonance imaging T1 images showing frontotemporal atrophy and positron emission tomography scan showing hypometabolism in the same areas|
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|Figure 2: Family tree depicting the members affected with frontotemporal dementia and heart block|
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There was no family member with heart block without dementia to suggest a dual pathology. The well-known dominant types of FTD with chromosomes 17, 3, and 9 are associated with Parkinson's disease and motor neuron disease.
| What Is New?|| |
A strong family history of FTD and heart block in a dominant pattern is not reported in literature to our knowledge.
- We could not examine the other family members as they were not alive; however, the history given by the caregiver of the index case was very much consistent with probable FTD
- Genetic assessment was not done in view of economic reasons and lack of benefit at this point to the patient or family
- Voxel-based morphometry could not be done to look for insular region disproportionate volume change as the patient needed general anesthesia (GA) for repeating the images with a small potential of harm.
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.
We would like to thank the National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]