IJCRI International Journal of Case Reports and Images, Vol. 4 No. 3, March 201 3. ISSN [0976-31 98]

IJCRI 201 3;4(3):1 751 79.www.ijcasereportsandimages.com

A case of frontotemporal dementiaHeng Siang Ting, B M Yashodhara, Bashir Ahmad Dar, Uduman AliMohamed Yousuf, Adinegara Lutfi Abas

ABSTRACTIntroduction: Frontotemporal dementia is acommon cause of dementia. It is easilymisdiagnosed as a psychiatric illness due to itspresentation with behavioral problems, mutism,language problems and in some cases withaggression and antisocial behavior. CaseReport: We report a case of a 72yearold manwho presented with behavioral abnormalities,lack of personal hygiene, personality changes ofgradual onset for 23 years, suggested byinability to take care of self, mutism, socialdisinhibition in the form of micturition in thepresence of family members. Computedtomography (CT) scan of the brain showedatrophy of frontal and temporal lobes. Thefamily members were counseled for theoutcome and prognosis of the case. Conclusion:The diagnosis of frontotemporal dementia ismade based on presentation, diagnostic criteriaand brain imaging. Definite treatment is not

available, but treating according to presentingcomplaints of the patient is helpful e.g. if thepatient has depression, antidepressants alongwith family counselling about the progression ofthe disease and awareness about requirementfor the family support are important.Keywords: Dementia, Frontotemporal,Abnormal behavior, Poor personal hygiene

*********Ting HS, Yashodhara BM, Dar BA, Yousuf UAM, AbasAL. A case of frontotemporal dementia. InternationalJournal of Case Reports and Images 20134(3):175179.

*********doi:10.5348/ijcri201303288CR9

INTRODUCTIONDementia is a common disease in middle age andelderly. It may be due to Alzheimer's disease, vasculardementia, frontotemporal dementia (FTD) or Lewybody dementia. It may also be due to secondary causeslike HIV infection, Huntingtons disease, head injury,Parkinsons disease, metabolic and endocrine diseases,poisoning, infections like neurosyphilis, vitamindeficiencies, normal pressure hydrocephalus andpseudodementia due to depression.Alzheimer disease patients typically present withproblems in cognitive functions memory, executivefunctions, language and constructional praxis. On brainscanning there is atrophy of the brain from occipitallobe to parietal lobe and later in the disease of thefrontotemporal areas. Patients tend to get lost and findit difficult to get back to their place due to problems invisualspatial skills. They also lose things easily.Patients of frontotemporal dementia typically haveproblems in behavior, personality and language when

CASE REPORT OPEN ACCESS

Heng Siang Ting

1

, B M Yashodhara

2

, Bashir Ahmad Dar

2

,

Uduman Ali Mohamed Yousuf

3

, Adinegara Lutfi Abas

4

Affi l iations:

1

Final year Medical Student, Melaka Manipal

Medical College, Melaka, Malaysia;

2

Associate Professor,

Department of Medicine, Melaka Manipal Medical College,

Melaka, Malaysia;

3

HOD, Department of Medicine, Melaka

Manipal Medical College, Melaka, Malaysia;

4

Deputy

Dean, HOD, Department of Community Medicine, Melaka

Manipal Medical College, Melaka, Malaysia.

Corresponding Author: Dr. Yashodhara BM, Associate

Professor, Department of Medicine, Melaka Manipal

Medical College 751 50, Melaka, Malaysia; Ph: 006-

01 76948029; Fax: 006281 7977; Email :

bmyashodhara@gmail .com

Received: 23 August 201 2

Accepted: 1 5 October 201 2

Published: 01 March 201 3

Ting et al. 1 75

IJCRI International Journal of Case Reports and Images, Vol. 4 No. 3, March 201 3. ISSN [0976-31 98]

IJCRI 201 3;4(3):1 751 79.www.ijcasereportsandimages.com Ting et al. 1 76

assessed by neuropsychiatric battery of tests but haveintact visualspatial skills. The presenting features arein the form of behavioral abnormalities like antisocialconduct, poor personal grooming, thefts and vehicleaccidents. Generally the diagnosis is not made early bynonspecialists, even though it is one of the commoncauses of dementia.

CASE REPORTA 72yearold man presented with history of poororal intake for two days, chills and rigors, without thepresence of fever on daily temperature recording. As hewas not able to give accurate history, his daughter wasinterviewed and it was found that the patient has hadpersonality change for 23 years now. It was insidiousin onset and progressively worsening. Daughter noticedthat patient had problems with memory, particularlyshort term memory loss, with patient not being able toremember the task he was doing at a time. Patient wasalso found to have multiple, minor, motor vehicleaccidents, but he was not able to recall what happened.Patient also frequently misplaced things. The patientcould still recognize family members, and was able todrive out alone without getting lost. For the past oneyear, patient had a gradual onset of disinhibition. Hedid not have urinary incontinence, but he would urinateanywhere and anytime at home even in the presence ofother family members. He also became more reserved,communicated poorly with family members and attimes was found to be nonresponsive to questions.Patients daughter also noticed that patient has peculiarbehaviors on and off such as sudden thought block andsmiling to himself. He did not have visualhallucinations. The patients daughter observed that hehad vesicular rash on his left thigh which was painfuland not itchy. There was no history of previous headtrauma, no fever, no movement disorders and nohistory of frank delusion or hallucination. There was noprevious history of psychiatric disorder in the patient orfamily members. On examination, the patient was alertand conscious with GCS 15/15. However, he was notpaying attention to his surroundings. He was afebrile,pulse rate was 83 beats per min, and blood pressure was128/62 mmHg. A tender, erythematous vesicular lesionwas found on left thigh. All cranial nerves were found tobe intact. Speech was normal, but at times patientwould become mute. His mini mental state examination(MMSE) score was 21/30, as assessed recently on followup by one of the attending neurologist. He was found tohave positive sucking, palmomental reflexes. Rest ofcentral nervous system examination and otherexaminations for respiratory, cardiovascular,gastrointestinal and musculoskeletal systems wereessentially normal.Patients full blood count, renal functions, thyroidfunctions, liver functions and routine urineexaminations were normal. He tested negative for HIV,Hepatitis B, Hepatitis C, venereal disease researchlaboratory test (VDRL), and treponema palladium

hemagglutination test (TPHA). His serum electrolyteswere normal. Based on the history and clinicalpresentation, a diagnosis of dementia with Herpessimplex of left thigh was considered. Computedtomography scan of the brain showed atrophy of frontaland temporal areas of the brain (Figures 14).

Figure 1: Bifrontotemporal atrophy and prominent sylvianfissures. Frontal horns of ventricles are just visible.

Figure 2: Bifrontotemporal atrophy and prominent sylvianfissures. Frontal horns of ventricles are clearly visible.

IJCRI International Journal of Case Reports and Images, Vol. 4 No. 3, March 201 3. ISSN [0976-31 98]

IJCRI 201 3;4(3):1 751 79.www.ijcasereportsandimages.com Ting et al. 1 77

A find diagnosis of frontotemporal dementia withrash due to herpes simplex infection on left thigh wasmade. He received acyclovir 200 mg 5 times a day for 5days at discharge. The daughter was counseled aboutthe disease and its progression and follow up. Thefamily members were very supportive to the patient.They were advised to not to let patient drive on his own.

DISCUSSIONFrontotemporal dementia is one of the causes ofearlyonset dementia (EOD) and has a 2.6% prevalence.Vascular dementia (42.5%), and Alzheimert disease(25.6%) are the most common causes, as noted in apopulation based study from Japan [1]. Frontotemporaldementia is a focal form of dementia in the presenium,yet remains poorly recognized [2], It is frequentlymistaken for Alzheimers disease or psychiatric diseases[3]. The age of onset of FTD is somewhat younger thanother degenerative dementias, with a mean age of onsetof about 58 years [4, 5]. It is now recognized as one ofthe three types of frontotemporal lobar degenerations(FTLD) frontotemporal dementia, semantic dementiaand progressive nonfluent aphasia [PNFA] [5]. Theprevalence of FTD is variable in different age ranges:3.6/100000 at age 5059 years, 9.4/100000 at age6069 years and 3.8/100000 at age 7079 years [4].The reported age of onset ranges from 33 to 80 years[4], with a male predominance in FTD and semanticdementia [5]: Frontotemporal dementia is the secondmost common cause of dementia in patients less than 65years of age [5]. Our patient developed frontotemporaldementia of 72 years of age.Macroscopic examination of the brain of a patientwith FTLD typically shows symmetrical focal atrophy ofthe frontal lobes, temporal lobes. Microscopicexamination of the cerebral cortex in most forms ofFTLD, shows microvacuolation and neuronal loss. Onstaining for deposits in neurons, abnormal tau proteinaggregates (46%) and ubiquitinpositive inclusions(29%) were found [6]. Our patient had frontal andtemporal lobe atrophy on CT scan. The exact etiologyand pathophysiology of frontotemporal dementia isincompletely understood but recent studies suggest thatgenetics plays an important role in disease causation [7].This is supported by the fact that about 50% of patientswith frontotemporal dementia (FTD) report a positivefamily history [7].Patients with FTD display a heterogeneous clinicalpicture, which may include behavioral, cognitive, andmotor manifestations [8]. They can be divided into twovariants: the behavioral variant and the language variant(also referred as primary progressive aphasia) [8]. Thebehavioral variant, which was a likely variant in ourpatient is characterized by progressive personalitychanges, lack of insight, loss of social awareness,emotional blunting and loss of empathy [8]. Thelanguage variant on the other hand can be furtherdivided into a welldefined clinicpathological entity,semantic dementia (SD), and progressive nonfluentaphasia (PNFA) [8]. Progressive nonfluent aphasia ischaracterized by a progressive deficit in language,starting from anomia and agramatism progressing todysfluent spontaneous speech and eventually mutism[8]. Our patient had shown features of mutism, duringhis admission in the inpatient department and was alsonoted by the daughter of the patient. The vesicular rashon the thigh in our patient, was diagnosed to be due toHerpes simplex and was treated appropriately.

Figure 3: Bifrontotemporal atrophy and prominent sylvianfissures. Frontal horns of the ventricles are dilated.

Figure 4: Prominent dilated ventricles. Left ventricle wasdilated more than the right ventricle.

IJCRI International Journal of Case Reports and Images, Vol. 4 No. 3, March 201 3. ISSN [0976-31 98]

IJCRI 201 3;4(3):1 751 79.www.ijcasereportsandimages.comFrontotemporal dementia is largely made by clinicalassessment, as in our case. Neuroimaging studies areequally important to provide supportive evidence fordiagnosis as well as to exclude other structural diseases[8]. Multiple diagnostic criteria to aid in diagnosis(Table 1) have been devised and published [9].Treatment mode available for frontotemporal dementiais very limited as there are no currently FDAapproveddiseases modifying treatments [10]. Hence, offlabelmedication usage is frequent in the hope to cope with its

Table 1: Clinical and investigational diagnostic criteria for frontotemporal dementia

devastating functional disability. Antidepressant andantipsychotic medications, (US FDAapprovedtreatments for Alzheimers disease) includingcholinesterase inhibitors and memantine have been used[10]. Antidepressant and antipsychotic medications [11],have proven to give some benefits while cholinesteraseinhibitors have limited efficacy [12]. The role ofmemantine in frontotemporal dementia is under furtherevaluation [10].

CONCLUSIONFrontotemporal dementia is easy to diagnose andshould be suspected in middle age and elderly personspresenting with gradual onset of personality changes,aggressive behavior, apathy and mutism. It is easilydiagnosed by standard criteria and brain scan findings.Family history may be positive in some cases.

*********Author ContributionsHeng Siang Ting Substantial contributions toconception and design, Acquisition of data, Drafting thearticle, Revising it critically for important intellectualcontent, Final approval of the version to be publishedYashodhara BM Substantial contributions toconception and design, Acquisition of data, Drafting thearticle, Revising it critically for important intellectualcontent, Final approval of the version to be publishedBashir Ahmad Dar Substantial contributions toconception and design, Analysis and interpretation ofdata, Final approval of the version to be publishedUduman Ali Mohamed Yousuf Substantialcontributions to conception and design, Analysis andinterpretation of data, Final approval of the version tobe publishedAdinegara Lutfi Abas Substantial contributions toconception and design, Analysis and interpretation ofdata, Final approval of the version to be published

GuarantorThe corresponding author is the guarantor ofsubmission.Conflict of InterestAuthors declare no conflict of interest.CopyrightHeng Siang Ting et al. 2013 This article is distributedunder the terms of Creative Commons Attribution 3.0License which permits unrestricted use, distributionand reproduction in any means provided the originalauthors and original publisher are properly credited.(Please see www.ijcasereportsandimages.com/copyrightpolicy.php for more information.)

REFERENCES1. Chiaki Ikejima, Fumihiko Yasuno, KatsuyoshiMizukami, Megumi Sasaki, Satoshi Tanimukai,Takashi Asada. Prevalence and Causes of EarlyOnset Dementia in Japan: A PopulationBasedStudy. Stroke 200940(8):270914.2. Snowden JS, Neary D, Mann DM. Frontotemporaldementia. The British Journal of Psychiatry2002180:1403.3. McKhann GM, Albert MS, Grossman M, Miller B,Dickson D, Trojanowski JQ. Clinical andPathological Diagnosis of Frontotemporal Dementia,Report of the Work Group on Frontotemporal

Ting et al. 1 78

IJCRI International Journal of Case Reports and Images, Vol. 4 No. 3, March 201 3. ISSN [0976-31 98]

IJCRI 201 3;4(3):1 751 79.www.ijcasereportsandimages.comDementia and Pick's Disease. Arch Neurol200158(11):18039.4. Rosso SM, Donker Kaat L, Baks T, et al.Frontotemporal dementia in The Netherlands:patient characteristics and prevalence estimatesfrom a populationbased study. Brain 2003Sep126(Pt 9):201622.5. Johnson JK, Diehl J, Mendez MF, et al.Frontotemporal Lobar Degeneration: DemographicCharacteristics of 353 Patients. Arch Neurol200562(6):92530.6. Forman MS, Farmer J, Johnson JK, et al.Frontotemporal dementia: Clinicopathologicalcorrelations. Ann Neurol 200659(6):95262.7. Christian Wider, Zbigniew K Wszolek. Etiology andPathophysiology of Frontotemporal Dementia,Parkinson Disease and Alzheimer Disease: Lessonsfrom Genetic Studies. Neurodegener Dis 20085(34):1225.8. Leyton CE, Hodges JR. Frontotemporal dementias:Recent advances and current controversies. AnnIndian Acad Neurol 201013(Suppl 2):S7480.

Ting et al. 1 79

9. Neary D, Snowden JS, Gustafson L, et al.Frontotemporal lobar degeneration: a consensus onclinical diagnostic criteria. Neurology 1998Dec51(6):154654.10. Geoffrey A Kerchner, Maria Carmela Tartaglia,Adam L Boxer. Abhorring the vacuum: use ofAlzheimers disease medications in frontotemporaldementia. Expert Rev Neurother 2011May11(5):70917.11. Florence Lebert, Willy Stekke, ChristineHasenbroekx, Florence Pasquier. FrontotemporalDementia: A Randomised, Controlled Trial withTrazodone. Dement Geriatr Cogn Disord200417(4):3559.12. Mendez MF, Shapira JS, McMurtray A, Licht E.Preliminary Findings: Behavioural Worsening onDonepezil in Patients With FrontotemporalDementia. American Journal of Geriatric Psychiatry2007 January15(1):847.

Access full text article onother devices Access PDF of article onother devices