Gabison-Hermann D, Pelletier A, Taleb M, Bouleau J-H
Centre Jean-Delay, Centre Hospitalier René-Dubos, secteur G12, 6, avenue de l'Ile-de-France, 95301 Pontoise, France.
Encephale. 2009 Oct;35(5):491-5. doi: 10.1016/j.encep.2008.06.013. Epub 2008 Dec 19.
CASE-REPORT: A thirty-seven-year-old man, with temporal epilepsy, had transient, atypical psychiatric states with periods of time without any symptom. These episodes included hypersexuality with qualitative changes of sex drive, obscene behavior, exhibitionism, masturbation and modified sexual orientation. Blunted affect, inability to recognize significant persons (visual agnosia) were also detected. Magnetic resonance imaging was normal and interictal single-photon emission computed tomography (SPECT) showed decreased cerebral perfusion in both temporal lobes.
The principal hypothesis is a Klüver-Bucy syndrome (KBS). In animals and human beings, this syndrome can be produced by bilateral temporal lobectomy. It is characterised by hypersexuality, visual agnosia, strong oral tendency, dietary changes, hypermetamorphosis and blunted affect. A minimum of three KBS elements suggests bilateral temporal dysfunction and supports the diagnosis. The syndrome may occur in herpes encephalitis, head trauma, Pick disease and temporal epilepsy. A single case of a patient, without any evidence for structural lesion in temporal lobes, is presented with many KBS symptoms, behavioral changes being due to complex partial seizure. Bitemporal dysfunction for this patient was confirmed by SPECT scan. On the other hand, the detected behavioral changes cannot be explained by temporal epilepsy alone. Postictal hypersexuality in temporal epilepsy consists in sexual arousal but not sexual aberrations as found in KBS.
KBS following complex partial status epilepticus is a rare phenomenum. The case described here shows how atypical psychiatric episodes can cover complex neurologic disorders.
一名37岁患有颞叶癫痫的男性,出现短暂的、非典型精神状态,且有一段时间无症状。这些发作包括性欲亢进及性欲性质改变、秽亵行为、露阴癖、手淫和性取向改变。还检测到情感迟钝、无法识别重要人物(视觉失认症)。磁共振成像正常,发作间期单光子发射计算机断层扫描(SPECT)显示双侧颞叶脑灌注减少。
主要假说是克吕弗-布西综合征(KBS)。在动物和人类中,该综合征可由双侧颞叶切除术引起。其特征为性欲亢进、视觉失认症、强烈的口欲倾向、饮食改变、过度变态反应和情感迟钝。至少具备三项KBS要素提示双侧颞叶功能障碍并支持诊断。该综合征可发生于疱疹性脑炎、头部外伤、匹克病和颞叶癫痫。本文报告一例患者,颞叶无任何结构病变证据,但出现许多KBS症状,行为改变归因于复杂部分性发作。SPECT扫描证实该患者存在双侧颞叶功能障碍。另一方面,所检测到的行为改变不能仅用颞叶癫痫来解释。颞叶癫痫发作后的性欲亢进表现为性唤起,而非如KBS中所见的性变态。
复杂部分性癫痫持续状态后的KBS是一种罕见现象。本文所述病例显示非典型精神发作如何掩盖复杂的神经系统疾病。