Kaohsiung Journal of Medical Sciences (2011) 27, 159e162
available at www.sciencedirect.com
journal homepage: http://www.kjms-online.com
CASE REPORT
KluvereBucy syndrome in one case with systemic lupus erythematosus 紅斑性狼瘡患者表現克魯爾－布西(Kluver-Bucy)症候群個案報告 Hsiu-Fen Lin a,b, Yi-Chun Yeh c, Chien-Fu Chen d, Wei-Chen Chang a,e, Cheng-Sheng Chen c,f,g,* 林秀芬
a,b
, 葉怡君 c, 陳建甫 d, 張瑋真 a,e, 陳正生
c,f,g,
*
a
Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan Department of Neurology, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan c Department of Psychiatry, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan d Department of Neurology, Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan e Department of Nursing, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan f Department of Psychiatry, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan g Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan b
Received 4 March 2010; accepted 29 June 2010 Available online 17 February 2011
KEYWORDS KluvereBucy syndrome; Systemic lupus erythematosus
關鍵詞 Kluver-Bucy症候群; 紅斑性狼瘡
Abstract KluvereBucy syndrome (KBS) is a collection of neuropsychiatric symptoms, including visual agnosia (prosopagnosia), hypermetamorphosis, placidity, hypersexuality, and hyperorality. Although neuropsychiatric manifestation is prevalent in cases with systemic lupus erythematosus (SLE), only one literature reported a case with SLE that had KBS previously. In this article, a 37-year-old woman with SLE who developed KBS and other neuropsychiatric symptoms is presented. Brain imaging proved the relevant structural lesion. The possible explanation of pathogenesis of KBS in SLE is discussed. 摘要 Kluver-Bucy 症候群包括視覺不能、異常衝動性的反應、過於馴服、性慾增加、與口慾增 加等表現。雖然紅斑性狼瘡或者常有神經精神症狀，但文獻中僅有一例Kluver-Bucy症候群的報 告。本文報告一例37歲的紅斑性狼瘡女性患者，表現出典型的Kluver-Bucy症候群。腦影像支持 其有相對應的雙側前顳葉的結構變化，可能的致病機轉將在本文進一步討論。 Copyright ª 2011, Elsevier Taiwan LLC. All rights reserved.
* Corresponding author. Department of Psychiatry, Kaohsiung Medical University Hospital, 100 Tzyou First Road, Kaohsiung City 80705, Taiwan. E-mail address:
[email protected] (C.-S. Chen). 1607-551X/$36 Copyright ª 2011, Elsevier Taiwan LLC. All rights reserved. doi:10.1016/j.kjms.2010.12.011
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Introduction KluvereBucy syndrome (KBS) is characterized by a constellation of neuropsychiatric symptoms, such as visual agnosia (prosopagnosia), hypermetamorphosis, placidity, hypersexuality, and hyperorality, initially observed in a rhesus monkey after bilateral temporal lobotomy [1]. Since the first identification in human in 1955 [2], more than 100 cases with various etiologies, such as acute herpes simplex encephalitis, stroke, Alzheimer’s disease, and carbon monoxide poisoning [3], have been reported. Among them, only one was found to be associated with systemic lupus erythematosus (SLE) [4]. We present here another patient with SLE along with KBS, in whom the clinical features mimicked other psychiatric disorders.
Case presentation A 37-year-old educated woman developed some aberrant behaviors consecutively, such as grabbing objects within reach to feed into mouth, compulsively eating and drinking, actively asking an unacquainted man for sexual intercourse, and purposelessly exploring objects around her even being thwarted. She could not identify her familial members by seeing them but by hearing their voices. Her family did not realize her neologistic jargon. Instead of naming some objects directly, she described them with circumlocution. Pop songs were sung in an unintelligible, as she claimed, “foreign language,” though with correct tunes. She looked as compliant as a pet, which was quite distinct from the demanding characteristics that she had before. Because of the elevated drive, decreased need for sleep, and disinhibited behaviors and speech, the diagnosis of manic state was initially made. However, her clinical condition fluctuated, until repeated episodes of general convulsion developed 6 months later, which led to her admission to our hospital. She had been diagnosed of SLE 4 years ago with pleuritis and renal function impairment. Although oral steroid was once administered for a short period, it was not taken in the recent 3 years. Certain facetious behaviors were also observed during her stay in the psychiatric ward, such as nostril digging, boasting of plump breasts, and dancing with seductive gestures in public. She often complained of pain in genital organs and asked for examination of the region. She was unaware of the inappropriateness of these sexual behaviors and was unable to describe the reasons in detail. She would show indifference to other patients’ provoking aggression and apathy out of context at one time, but unpredictable rage response and shouting with sexual obscenity appeared at the other. Repetitive exploration of environmental objects was prominent. She had such irresistible compulsion to eat that she had gained nearly 6 kg in 3 months’ hospitalization. Physical examination showed no abnormality except an emaciated appearance. Language disturbance included sensory aphasia, anomia, and varying degrees of agraphia, both phonological and semantic, in mandarin character writing. On the Wechsler Intelligence Scale, she achieved a verbal IQ of 66, performance IQ of 59, and full IQ of 61. Compared with her premorbid level of 12-year education, the results indicated significant cognitive deterioration in
H.-F. Lin et al. language, working memory, arithmetic, abstract and conceptual thinking, and visuospatial function. Other neurological examination revealed no focal sensorimotor neurological dysfunction. In laboratory examinations, serum antinuclear antibody was 1:512 and anti-La antibody was positive, whereas complete blood count, urinalysis, blood complements, and cerebrospinal fluid were normal. Serum venereal disease research laboratory (VDRL) test, anti-DNA antibody, and anti-Ro antibody were negative. In brain magnetic resonance imaging study, symmetric destructive lesions at bilateral anteromedial temporal lobes and diffuse cortical atrophy were noted. Brain single photon emission computed tomography (SPECT) demonstrated decreased cerebral blood flow at left parietotemporal and bilateral anterior temporal regions (Fig. 1). Oral prednisolone was administered at a dose of 40 mg per day (1 mg/kg body weight). Several psychotropic agents, including carbamazepine, neuroleptics, lithium, valproic acid, and benzodiazepine, also had been used in various durations for management of aberrant behavior. Although the immunological index associated with SLE showed a reduction (i.e. antinuclear antibody: from 1:512 to 1:128), she did not show obvious improvement in psychiatric and cognitive functions and was transferred to the chronic ward for further care 3 months later. Until now, she stays in a chronic ward without any further improvement in mental state. It can be explained that her brain lesions were persistent.
Discussion This case developed full KBS features, which was consistent with the localization of brain lesion evidenced by brain image study. Probably, owing to different premorbid characteristics and lesion severity involved among patients, the manifestation of KBS is different among humans. Hypersexuality, for instance, has various manifestations, such as sexual overture, comments, or attempted physical contact. This case presented seductive dancing and required the staff to touch her genital organs, which were supposed to be the manifestation of hypersexuality. Human KBS rarely occurred alone but often occurred as a part of complicated neuropsychiatric syndrome with various etiologies. The other neuropsychiatric symptoms in this case could judgmentally be divided into cognitive and behavioreemotional aspects. Of the cognitive deficits, amnesia, dementia, and fluent aphasia were the most common features associated with KBS [5]. To our knowledge, this is the first case of agraphia being reported to be associated with KBS. Agraphia in this case was characterized as writing with ease but lack of substantive words and contamination by phonological, semantic, and neological paragraphia in a sentence, called fluent posterior form. The anatomical lesion of agraphia accompanied with fluent aphasia, which is also noted in this case, is most mentioned at left parietotemporal area adjacent to anterior horn of temporal lobe [6]. The lesion localization of language disturbance is consistent with this patient. In view of the aberrant behaviors other than KBS that this case displayed, the most obvious were the facetious gesture, inappropriate jokes, and hypomania-like behavior, which were similar to
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Figure 1. Coronal view of brain SPECT in a patient with KluvereBucy syndrome. White arrows indicate hypoperfusion at the bilateral anterior temporal lobes.
“Witzelsucht of the extremities,” characterized by being socially uninhibited and lacking awareness of their abnormal behavior. However, focal orbitofrontal parenchyma lesion and not bilateral temporal lobe is often the anatomical substrate for these behavioral attitudes. We conjecture that the pathways connecting the amygdala, hippocampus with the orbitofrontal cortices, which are systems essential for regulation of impulses and emotions, were disrupted. Then, the disruption caused loss of the surveillance, attention, and arousal from dorsal system [7] and resulted in the uninhibited behavior. The occasionally unexpected rage attack noted in this case would be proposed to be incomplete ablation or the irritative nature
in the lesion. The possible pathogenesis has been proposed to explain the diverse neuropsychiatric manifestations in SLE. They include vasculopathy mediated by immune complexes, throbmoembolic disease, and direct antibodyemediated neuronal dysfunction. The neuropsychiatric manifestations in SLE are generally classified into two groups [8]. Some patients have “diffuse” symptoms, such as depression, psychosis, generalized seizure, or organic brain disorders. In this condition, the attack of the neuron-directed antibodies on the nervous system is considered the possible pathogenic mechanism. Whereas, the patient presented focal neurological deficits and focal cerebral lesions, and another group of antibodies (mainly antiphospholipid
Table 1 Comparisons among KluvereBucy syndrome, schizophrenia, and bipolar disorder, in terms of clinical presentation, neuroimaging findings, neuropsychological impairment, and treatment Syndrome/disorder KluvereBucy syndrome
Schizophrenia
Bipolar disorder
Clinical presentations
Delusion, hallucination, disorganized behavior
Manic or depressive symptoms, pervasive and persistent drive elevation Ventricular enlargement, temporal and fontal lobe involvement [12]
Neuroimaging
Visual agnosia, hypermetamorphosis, placidity, hypersexuality, hyperorality, situational disinhibited behaviors Bilateral anterior temporal lobe lesions
Neuropsychological Agnosia impairment
Treatment
Symptomatic treatment
Ventricular enlargement, temporal and fontal lobe involvement, subcortical structure [11] Attention, executive function, verbal and visuospatial working memory, and learning and memory [13] Antipsychotics
Verbal learning, executive functioning, and motor coordination [14] Mood stabilizers
162 antibodies) may play the roles in the development of the cerebral vascular lesions. Although the available data are inadequate to reveal which mechanism underlies the symptoms, the fact that the focal cerebral lesions cause overt neuropsychiatric pictures in our patient denotes the discrimination between so-called diffuse and focal neuropsychiatric manifestations in SLE, which is obscure. Compared with the first case report of KBS with SLE [4], our case developed full KBS, and the lesion involved was nearly confined to the bilateral temporal lobe. The distinct pathophysiological process may result in the difference. Because the temporal lobes are the common anatomical substrate for KBS and psychotic or affective symptoms [9], in addition to these, the case was misdiagnosed as a manic disorder initially, and schizophrenia had also been diagnosed in the other woman with KBS [10]. We summarized the difference in terms of clinical presentation, neuroimaging findings, neuropsychological impairment and treatment options between KBS, schizophrenia, and bipolar disorder in Table 1 [11e14]. We should reemphasize the fact that some neuropsychiatric syndromes being misdiagnosed as schizophrenia or mood disorders was not uncommon. We here suggested that the typical KBS accompanied with cognitive and behavior symptoms warranted further investigation for the possibility of bilateral temporal lobe lesion, particularly in patients with systemic disease.
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