de clerambault's syndrome (erotomania) as a presenting feature of

Behavioural Neurology 18 (2007) 193–195 193IOS Press

Clinical Note

De Clerambault’s syndrome (erotomania) as apresenting feature of fronto-temporaldementia and motor neurone disease(FTD-MND)

Charles Olojugbaa,c,∗, Rajith de Silvab, Luke D Kartsounisb, Lindsay Royana and Janet Cartera,d

aThe Petersfield Centre, Essex RM3 9PB, UKbDepartment of Neurology, Essex Centre for Neurological Sciences, Queen’s Hospital, Romford RM7 0AG, UKcSt. Pancras Hospital, London NW1 OPE, UKdDepartment of Mental Health Sciences, University College London, London W1N 8EY, UK

Keywords: De Clerambault’s syndrome, erotomania, fronto-temporal dementia, motor neurone disease

1. Introduction

Motor neurone disease (MND) is characterized bythe progressive degeneration of lower and upper mo-tor neurones in the spinal cord, brainstem and motorcortex of the brain, leading to a variable pattern ofmotor dysfunction. Typical symptoms include muscleatrophy, limb weakness, bulbar palsy and respiratoryfailure. Fronto-Temporal Dementia (FTD) is a com-mon neurodegenerative disorder associated with pro-gressive atrophy of the frontal and temporal lobes ofthe brain, resulting in changes in personality and be-haviour and/or speech and language dysfunction. Fiftyper cent of patients with MND exhibit frontal executivedeficits during the course of their illness, representingin most instances the co-occurrence of FTD. Previous

∗Address for correspondence: Dr. Charles Olukunle Olojugba,MBBS BSc MRCpsych DGM-RCP, Specialist registrar in old agepsychiatry, St. Pancras Hospital, St. Pancras Way, London NW1OPE, UK. Tel.: +44 207 530 4400; Fax: +44 207 540 4211; E-mail:[email protected].

descriptions of FTD indicate that psychotic symptoms(including hallucinations and delusions) are rare andspecifically cases of erotomania in FTD have not to ourknowledge been previously described [1].

Erotomania or De Clerambault’s syndrome is a delu-sional disorder typically presenting in middle-aged fe-males in association with psychiatric illness, includingSchizophrenia and Bipolar Affective Disorder [1]. It ischaracterised by the delusional belief that another per-son (the object), who is socially unobtainable becauseof unbridgeable gaps in social and financial statuses,passionately loves the patient. In this paper, a case ofFTD-MND in an elderly woman presenting with ero-tomania is reported.

2. Case report

The patient was a 65-year-old divorcee living alonein her own flat. Over a six-month period she had be-come increasingly preoccupied with the late celebrityDick Haymes (DH), who was one of the most popular

ISSN 0953-4180/07/$17.00 2007 – IOS Press and the authors. All rights reserved

194 C. Olojugba et al. / De Clerambault’s syndrome (erotomania) as a presenting feature of fronto-temporal dementia

American male vocalists of the 1930s–40s known forhis love ballads and marriage to the Hollywood actressRita Hayworth. She believed that DH was in love withher and was living next door to her. She had purchasedmultiple copies of CDs and DVDs featuring DH, andhad even waited for hours outside her flat anticipatingDH’s arrival. She had shown signs of aggression byslapping her daughter when challenged about her be-haviour. In addition she was not eating and sleepingproperly, and had been wandering the streets at oddtimes.

She had no previous psychiatric history. Her familyhistory revealed panic attacks, agoraphobia and audito-ry hallucinations in her mother and alcoholism in her fa-ther. Her medical history included a previous transientischaemic attack and non-arteritic anterior ischaemicleft optic neuropathy. She smoked 40 cigarettes a dayuntil 5 years previously and had not abused alcohol.She had been married for 18 years before becoming di-vorced, and had two children. On initial examination,she was mildly dysarthric but no other neurological ab-normalities were noted. Her mood was elated and attimes she was disinhibited, for example laughing inap-propriately. She had a fixed belief that DH was in lovewith her and she with him, stating “I love him to bits”.She was preoccupied with this theme and it was difficultto distract her. Despite being told that DH was dead sheinsisted that he was alive. There was no evidence ofhallucinosis in any modality and her mini mental stateexamination score was 29/30. More detailed cogni-tive testing indicated impaired abstracting ability, andplanning and organisational difficulties. MRI of thebrain showed chronic small vessel ischaemia and atro-phy affecting the anterior temporal lobes. An HMPAO-SPECT scan demonstrated hypoperfusionespecially ofthe left fronto-temporal region (Fig. 1). She was start-ed on olanzapine 2.5 mg to which she responded welland her delusions abated. Subsequently her speech de-teriorated rapidly, becoming more dysarthric and dys-phonic. She had difficulty swallowing food, and shedeveloped wasting of the small muscles in her hands,resulting in loss of function. She developed progressiveweakness of her legs, and had weight loss. The patientwas referred urgently for a neurological opinion, eigh-teen months after her initial presentation. At that point,she was anarthric, with restricted soft palate movementand fasciculations in her tongue. A left-sided palmo-mental and pouting responses were present. In her up-per limbs she had profound small hand muscle wast-ing bilaterally with clawing. Profuse fasciculationswere present in the upper and lower limbs. Limb re-

Fig. 1. HMPAO-SPECT scan demonstrating hypoperfusion of thefronto-temporal region.

flexes were present, and plantar responses were flexor.A speech and language therapy assessment includingvideo fluoroscopy demonstrated oral stage dysphagiacharacterised by weak tongue and reduced soft palatemovements. Electrophysiology confirmed widespreadacute and chronic partial denervation, suggestive ofMND.

Neuropsychological examination revealed that thepatient had marked difficulty in sustaining concentra-tion over time precluding comprehensive assessment.She became “stimulus bound” with any change in theenvironment,and showed motor perseveration and poorself-monitoring. She was impulsive in her responsesand exhibited utilisation behaviour, attempting to useany object placed in her vicinity. As a result, testingcould proceed only by the examiner physically restrain-ing her from making impulsive responses. On MatrixReasoning, a set of nonverbal reasoning problems ofthe Wechsler Adult Intelligence Scale [2], she scored ata borderline level. On the Test for Reception of Gram-mar [4], she performed creditably at a single word lev-el. She appeared able to comprehend only very sim-ple sentences. She was able to match a few printedsingle words to corresponding pictures. She showedgood recognition of common objects. On the ObjectDecision Test of visual perception [5] she performedwithin the normal range for her age group (15/20). Shewas unable to complete the Weigl Colour From Sort-ing Test, a relatively undemanding test of nonverbalabstract reasoning. These observations suggested thather posterior cortical cognitive functions were proba-bly well preserved. By contrast, she presented withprofound executive deficits, with environmental depen-dency, impulsivity, poor self-monitoring and elements

C. Olojugba et al. / De Clerambault’s syndrome (erotomania) as a presenting feature of fronto-temporal dementia 195

of utilisation behaviour, indicative of marked frontallobe dysfunction. A diagnosis of MND with FTD wasmade, and she was started on Riluzole.

3. Discussion

Erotomania in the elderly is a rare presenting symp-tom. Initial examination of our patient revealed execu-tive deficits and mild dysarthria in addition to the delu-sional disorder. Thus, she showed cognitive and physi-cal evidence of a neurological disorder at presentation,as well as a psychiatric syndrome. The neuroimag-ing data, the presence of frontal executive dysfunc-tion, despite an initial normal MMSE score, and mooddisturbance supported a diagnosis of fronto-temporaldementia, according to the Lund-Manchester diagnos-tic criteria [7]. The development of progressive bul-bar signs prompted a second neurological examination,which identified wasting of the small hand musclesand gait impairment – leading to a diagnosis of MND.This highlights the importance of repeating the neuro-logical evaluation of elderly patients who present withpsychiatric syndromes that worsen despite treatment,especially when new neurological complaints develop.

In FTD-MND psychotic symptoms are relativelyrare [6]. In a previous study of three cases of psy-chosis in FTD-MND, psychotic symptoms did not pre-date the onset of motor signs of MND. However, delu-sions when present were always of a persecutory con-tent. Additionally in the cases studied all the patientsexperienced hallucinations as part of their psychosis.The early involvement of the temporal lobes in the de-generative process was suggested to have been a possi-ble cause of hallucinations. No explanation for the oc-currence of delusions was offered [9]. In contrast, thedelusions in the patient reported here were erotomanicin nature and unassociated with hallucinations. Theexplanations for the observed differences in these casesare unknown, but premorbid factors such as personalitymay play a role.

In Alzheimer’s disease where delusions are re-ported in 30%–40% of cases, PET neuroimaginghas demonstrated a significant correlation between

hypometabolism in the right prefrontal cortex and delu-sional thought [3]. However, a SPECT analysis in pa-tients with FTD failed to show any association betweenfrontotemporal hypoperfusion and psychotic symp-toms [8], further highlighting the uncertainty surround-ing the origin of delusions in FTD-MND.

4. Conclusion

FTD-MND can present with delusional disorder.The neuronal mechanisms underlying this type of pre-sentation are presently unclear. More research is need-ed to evaluate the clinical course of such patients.

Declaration of interest: none.The authors acknowledge the contributions of Dr.

Sanjiv Chawda (Neuroradiology), Dr. Ranjan Gu-nasekera (HMPAO-SPECT data),Dr. Santiago Catania(Clinical Neurophysiology), and Ms. Melanie White-head (swallowing assessments).

References

[1] B. Martin and S. Stefan, Erotomania variants in dementia,JGeriatr Psychiatry and Neurol 16 ( 2003), 232–234.

[2] D. Wechsler,Wechsler Adult Intelligence Scale (WAIS-III), Lon-don, The Psychological Corporation, 1997.

[3] D.L. Sultzer and C.V. Brown, Delusional thoughts and regionalfrontal/temporal cortex metabolism in Alzheimer’s disease,AmJ Psychiatry 161 (2004), 764.

[4] D.V.M. Bishop, The Test for Reception of Grammar (TROG),Cambridge, Medical Research Council, 1992.

[5] E.K. Warrington and M. James,The Visual Object and SpacePerception Battery, Bury St Edmunds, Thames Valley Test Co,1991.

[6] J. Hodges, R. Davies and J. Xuereb, Clinicopathological cor-relates in frontotemporal dementia,Ann Neurol 108 (2004),515–523.

[7] Lund and Manchester Groups Clinical and neuropathologicalcriteria for frontotemporal dementia,J Neurol Neurosurg Psy-chiatry 57 (1994), 416–418.

[8] M.F. Mendez and A. McMurtray, Functional neuroimaging andpresenting psychiatric features in frontotemporal dementia,JNeurol Neurosurg Psychiatry 77 (2006), 4–7.

[9] R. Nitrini and S. Rosemberg, Psychotic symptoms in dementiaassociated with motor neurone disease: a pathological hypoth-esis,J Neuropsych Clin Neurosci 10 (1998), 456–458.

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