Behavioural Neurology 18 (2007) 193–195 193 IOS Press Clinical Note De Clerambault’s syndrome (erotomania) as a presenting feature of fronto-temporal dementia 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, UK bDepartment of Neurology, Essex Centre for Neurological Sciences, Queen’s Hospital, Romford RM7 0AG, UK cSt. Pancras Hospital, London NW1 OPE, UK dDepartment 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 descriptions of FTD indicate that psychotic symptoms (including hallucinations and delusions) are rare and Motor neurone disease (MND) is characterized by specifically cases of erotomania in FTD have not to our the progressive degeneration of lower and upper mo- knowledge been previously described [1]. tor neurones in the spinal cord, brainstem and motor Erotomania or De Clerambault’s syndrome is a delu- cortex of the brain, leading to a variable pattern of sional disorder typically presenting in middle-aged fe- motor dysfunction. Typical symptoms include muscle males in association with psychiatric illness, including atrophy, limb weakness, bulbar palsy and respiratory Schizophrenia and Bipolar Affective Disorder [1]. It is failure. Fronto-Temporal Dementia (FTD) is a com- characterised by the delusional belief that another per- mon neurodegenerative disorder associated with pro- son (the object), who is socially unobtainable because gressive atrophy of the frontal and temporal lobes of of unbridgeable gaps in social and financial statuses, the brain, resulting in changes in personality and be- passionately loves the patient. In this paper, a case of haviour and/or speech and language dysfunction. Fifty FTD-MND in an elderly woman presenting with ero- per cent of patients with MND exhibit frontal executive tomania is reported. deficits during the course of their illness, representing in most instances the co-occurrence of FTD. Previous 2. Case report

∗Address for correspondence: Dr. Charles Olukunle Olojugba, The patient was a 65-year-old divorcee living alone MBBS BSc MRCpsych DGM-RCP, Specialist registrar in old age in her own flat. Over a six-month period she had be- psychiatry, St. Pancras Hospital, St. Pancras Way, London NW1 OPE, UK. Tel.: +44 207 530 4400; Fax: +44 207 540 4211; E-mail: come increasingly preoccupied with the late celebrity [email protected]. Dick 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 for his love ballads and marriage to the Hollywood actress Rita Hayworth. She believed that DH was in love with her and was living next door to her. She had purchased multiple copies of CDs and DVDs featuring DH, and had even waited for hours outside her flat anticipating DH’s arrival. She had shown signs of aggression by slapping her daughter when challenged about her be- haviour. In addition she was not eating and sleeping properly, and had been wandering the streets at odd times. She had no previous psychiatric history. Her family history revealed panic attacks, agoraphobia and audito- ry hallucinations in her mother and alcoholism in her fa- ther. Her medical history included a previous transient Fig. 1. HMPAO-SPECT scan demonstrating hypoperfusion of the ischaemic attack and non-arteritic anterior ischaemic fronto-temporal region. left optic neuropathy. She smoked 40 cigarettes a day until 5 years previously and had not abused alcohol. flexes were present, and plantar responses were flexor. She had been married for 18 years before becoming di- A speech and language therapy assessment including vorced, and had two children. On initial examination, video fluoroscopy demonstrated oral stage dysphagia she was mildly dysarthric but no other neurological ab- characterised by weak tongue and reduced soft palate normalities were noted. Her mood was elated and at movements. Electrophysiology confirmed widespread times she was disinhibited, for example laughing inap- acute and chronic partial denervation, suggestive of propriately. She had a fixed belief that DH was in love MND. with her and she with him, stating “I love him to bits”. Neuropsychological examination revealed that the She was preoccupied with this theme and it was difficult patient had marked difficulty in sustaining concentra- to distract her. Despite being told that DH was dead she tion over time precluding comprehensive assessment. insisted that he was alive. There was no evidence of She became “stimulus bound” with any change in the hallucinosis in any modality and her mini mental state environment, and showed motor perseveration and poor examination score was 29/30. More detailed cogni- self-monitoring. She was impulsive in her responses tive testing indicated impaired abstracting ability, and and exhibited utilisation behaviour, attempting to use planning and organisational difficulties. MRI of the any object placed in her vicinity. As a result, testing brain showed chronic small vessel ischaemia and atro- could proceed only by the examiner physically restrain- phy affecting the anterior temporal lobes. An HMPAO- ing her from making impulsive responses. On Matrix SPECT scan demonstrated hypoperfusion especially of Reasoning, a set of nonverbal reasoning problems of the left fronto-temporal region (Fig. 1). She was start- the Wechsler Adult Intelligence Scale [2], she scored at ed on olanzapine 2.5 mg to which she responded well a borderline level. On the Test for Reception of Gram- and her delusions abated. Subsequently her speech de- mar [4], she performed creditably at a single word lev- teriorated rapidly, becoming more dysarthric and dys- el. She appeared able to comprehend only very sim- phonic. She had difficulty swallowing food, and she ple sentences. She was able to match a few printed developed wasting of the small muscles in her hands, single words to corresponding pictures. She showed resulting in loss of function. She developed progressive good recognition of common objects. On the Object weakness of her legs, and had weight loss. The patient Decision Test of visual perception [5] she performed was referred urgently for a neurological opinion, eigh- within the normal range for her age group (15/20). She teen months after her initial presentation. At that point, was unable to complete the Weigl Colour From Sort- she was anarthric, with restricted soft palate movement ing Test, a relatively undemanding test of nonverbal and fasciculations in her tongue. A left-sided palmo- abstract reasoning. These observations suggested that mental and pouting responses were present. In her up- her posterior cortical cognitive functions were proba- per limbs she had profound small hand muscle wast- bly well preserved. By contrast, she presented with ing bilaterally with clawing. Profuse fasciculations profound executive deficits, with environmental depen- were present in the upper and lower limbs. Limb re- 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 frontal hypometabolism in the right prefrontal cortex and delu- lobe dysfunction. A diagnosis of MND with FTD was sional thought [3]. However, a SPECT analysis in pa- made, and she was started on Riluzole. tients with FTD failed to show any association between frontotemporal hypoperfusion and psychotic symp- toms [8], further highlighting the uncertainty surround- 3. Discussion ing the origin of delusions in FTD-MND.

Erotomania in the elderly is a rare presenting symp- tom. Initial examination of our patient revealed execu- 4. Conclusion tive deficits and mild dysarthria in addition to the delu- sional disorder. Thus, she showed cognitive and physi- FTD-MND can present with delusional disorder. cal evidence of a neurological disorder at presentation, The neuronal mechanisms underlying this type of pre- as well as a psychiatric syndrome. The neuroimag- sentation are presently unclear. More research is need- ing data, the presence of frontal executive dysfunc- ed to evaluate the clinical course of such patients. tion, despite an initial normal MMSE score, and mood Declaration of interest: none. disturbance supported a diagnosis of fronto-temporal The authors acknowledge the contributions of Dr. dementia, according to the Lund-Manchester diagnos- Sanjiv Chawda (Neuroradiology), Dr. Ranjan Gu- tic criteria [7]. The development of progressive bul- nasekera (HMPAO-SPECT data),Dr.Santiago Catania bar signs prompted a second neurological examination, (Clinical Neurophysiology), and Ms. Melanie White- which identified wasting of the small hand muscles head (swallowing assessments). and gait impairment – leading to a diagnosis of MND. This highlights the importance of repeating the neuro- logical evaluation of elderly patients who present with References psychiatric syndromes that worsen despite treatment, especially when new neurological complaints develop. [1] B. Martin and S. Stefan, Erotomania variants in dementia, J In FTD-MND psychotic symptoms are relatively Geriatr Psychiatry and Neurol 16 ( 2003), 232–234. [2] D. Wechsler, Wechsler Adult Intelligence Scale (WAIS-III), Lon- rare [6]. In a previous study of three cases of psy- don, The Psychological Corporation, 1997. chosis in FTD-MND, psychotic symptoms did not pre- [3] D.L. Sultzer and C.V. Brown, Delusional thoughts and regional date the onset of motor signs of MND. However, delu- frontal/temporal cortex metabolism in Alzheimer’s disease, Am J Psychiatry 161 (2004), 764. sions when present were always of a persecutory con- [4] D.V.M. Bishop, The Test for Reception of Grammar (TROG), tent. Additionally in the cases studied all the patients Cambridge, Medical Research Council, 1992. experienced hallucinations as part of their psychosis. [5] E.K. Warrington and M. James, The Visual Object and Space The early involvement of the temporal lobes in the de- Perception Battery, Bury St Edmunds, Thames Valley Test Co, 1991. generative process was suggested to have been a possi- [6] J. Hodges, R. Davies and J. Xuereb, Clinicopathological cor- ble cause of hallucinations. No explanation for the oc- relates in frontotemporal dementia, Ann Neurol 108 (2004), currence of delusions was offered [9]. In contrast, the 515–523. delusions in the patient reported here were erotomanic [7] Lund and Manchester Groups Clinical and neuropathological criteria for frontotemporal dementia, J Neurol Neurosurg Psy- in nature and unassociated with hallucinations. The chiatry 57 (1994), 416–418. explanations for the observed differences in these cases [8] M.F. Mendez and A. McMurtray, Functional neuroimaging and are unknown, but premorbid factors such as personality presenting psychiatric features in frontotemporal dementia, J may play a role. Neurol Neurosurg Psychiatry 77 (2006), 4–7. [9] R. Nitrini and S. Rosemberg, Psychotic symptoms in dementia In Alzheimer’s disease where delusions are re- associated with motor neurone disease: a pathological hypoth- ported in 30%–40% of cases, PET neuroimaging esis, J Neuropsych Clin Neurosci 10 (1998), 456–458. has demonstrated a significant correlation between M EDIATORSof INFLAMMATION

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