Klüver-Bucy syndrome in humans

The Klüver-Bucy syndrome (KBS) was first described in 1937 as an experimental neurobehavorial syndrome in monkeys with bitemporal brain lesions. The syndrome in man was subsequently observed to be transient or permanent in a variety of neurodegenerative disorders and after traumatic, nontraumatic, and infectious brain injury. Its most common manifestations are hyperorality with changes in dietary habits, hypersexuality, and visual agnosia. Seizures are another frequent symptom. Here we describe KBS in a female inpatient aged 30 in whom KBS and psychotic symptoms occurred together.     

The Klüver-Bucy syndrome in man

It is apparent that the importance of the limbic system is essentially for survival. The inability to discriminate enemy from friend as well as loss of the proper affective response to what is dangerous or safe to the organism may be thought of as more central to survival than what is described as "other higher intellectual functions." The case described is a dramatic expression of such a transient limbic dementia originally described by Klüver and Bucy in their syndrome. Since Klüver and Bucy originally described their syndrome, the structural and functional understanding of the limbic system has been greatly increased. Although a rare occurrence in man, the Klüver-Bucy syndrome serves as a demonstration of an unusual brain syndrome whose theoretical and experimental basis has been greatly enlarged since first described.     

Complete Klüver-Bucy syndrome in man.

A 22 year old right-handed man suffered a viral meningoencephalitis, possibly herpetic, resulting in bilateral damage to the temporal lobes as confirmed by appropriate clinical, electrophysiological and neuroradiological studies. Extended clinical neuropsychological evaluation documented all the characteristic features of the syndrome described by Klüver and Bucy following bilateral ablation of the temporal lobes in adult Rhesus monkeys, including "psychic blindness," oral exploration, hypermetamorphic impulse to action," lack of emotional responsiveness, aberrant sexual behavior, and an insatiable appetite. Additionally, a severe Wernicke's aphasia and a profound memory disorder were evident. The significance of these features as regards limbic function in the human is discussed.     

Klüver-Bucy syndrome following status epilepticus associated with hepatic encephalopathy

Described here is the case of a patient with liver cirrhosis who developed bilateral temporo-occipital lobe lesions on MRI and Klüver-Bucy syndrome following status epilepticus. Herpes encephalitis, paraneoplastic syndrome, Hashimoto's encephalopathy, reversible posterior leukoencephalopathy syndrome, mitochondrial encephalomyopathy, lactic acidosis, and strokelike episode syndrome were judged not to be involved on the basis of laboratory results. The possible cause of the temporo-occipital lesions on MRI in this patient was cortical damage related mainly to status epilepticus and partially to coexisting hepatic encephalopathy.     

Persistent Klüver-Bucy syndrome after bilateral temporal lobe infarction

Klüver-Bucy (KBS) syndrome is a rare and complicated neurobehavioral syndrome in humans resulting from damage of bilateral anterior temporal portion, especially the amygdala. It can be seen in association with a variety of etiologies. Stroke is a rarely reported. Here we present a 50-year-old right handed man who developed persistent KBS after cardioembolic stroke involving bilateral lateral temporal lobes. He exhibited all clinical features of KBS including visual agnosia, hypersexuality, placidity, hyperorality and hypermetamorphosis. The anatomical basis of pathophysiolgy, clinical course and possible treatment are discussed.     

Nerve ultrasound follow-up in a child with Guillain-Barré syndrome

Introduction: Nerve ultrasound has been used increasingly in neurophysiology laboratories, but data on Guillain‐Barré syndrome (GBS) are still limited, and no follow‐up studies are available. Case report: An 8‐year‐old boy was admitted with severe demyelinating GBS. Serial neurophysiological evaluations were performed initially and in follow‐up. Ultrasound studies showed diffuse and heterogeneous nerve swelling and focal enlargement of single fascicles inside the nerve. Together with clinical and electrophysiological improvement, progressive normalization of ultrasound changes was seen. Conclusions: Ultrasound demonstrated structural nerve abnormalities in GBS. These changes normalized as the patient improved clinically and electrophysiologically. Further studies are needed to elucidate the diagnostic and prognostic value of ultrasound in GBS. Muscle Nerve 46: 270–275, 2012     

Methotrexate leukoencephalopathy presenting as Klüver-Bucy syndrome and uncinate seizures

Methotrexate causes several biochemical changes that impact the nervous system. The neurotoxicity usually affects the cerebral white matter, causing a leukoencephalopathy that can be chronic and progressive with cognitive decline. A 15-year-old male developed olfactory seizures and behavioral abnormalities (hypersexuality, placidity, and memory disturbances) compatible with partial Klüver-Bucy syndrome after treatment for central nervous system leukemia with intraventricular methotrexate. A magnetic resonance imaging study revealed evidence of white matter disease affecting both temporal lobes. A brain biopsy revealed a necrotizing encephalopathy compatible with methotrexate-related white matter injury. It may be prudent to verify normal cerebrospinal fluid dynamics before the administration of intraventricular methotrexate in children with a history of central nervous system leukemia.     

Hyperorality in epileptic seizures: periictal incomplete Klüver-Bucy syndrome

PURPOSE: To analyze systematically hyperorality associated with epileptic seizures and its relation to the localization of epileptic activity. METHODS: To identify patients with periictal hyperorality, we reviewed video-recordings of 269 patients (aged 6-59 years) who had consecutively undergone presurgical evaluations including ictal video-EEG recordings and high-resolution magnetic resonance imaging (MRI) and had had epilepsy surgery because of intractable frontal (FLE) or temporal lobe epilepsy (TLE). Periictal hyperorality was defined if patients put or unambiguously intended to put nonfood items into their mouths during or after at least one of the reviewed seizures. For the further analysis, we included only patients with periictal hyperorality. We reviewed their medical records and reexamined their ictal video-EEG recordings. RESULTS: We identified eight patients (six women) aged 8-59 years who had hyperorality during or after seizures. Seven patients had TLE, and one patient had frontal lobe epilepsy (FLE). Three of these patients underwent right-sided surgery, whereas five patients had surgery on the left. Three patients exhibited ictal and five showed postictal hyperorality. Interictal EEG suggested bilateral interictal epileptiform discharges (IEDs) in three patients; in two other patients, no IEDs were detected. Ictal EEG suggested bilateral involvement in six cases. Patients with unilateral epileptiform activity had left TLE. CONCLUSIONS: Periictal hyperorality is a rare phenomenon occurring in 3% of the investigated epilepsy population. We suggest that periictal hyperorality is an ictal-postictal mental disturbance, an incomplete Klüver-Bucy syndrome. In most patients, bilateral seizure activity plays an important role in the pathomechanism, but it would appear that left-sided epileptic activity without contralateral involvement also can cause periictal hyperorality.     

Should every child with epilepsy undergo a neuropsychological evaluation?

Neuropsychological (NP) testing has always been an important part of the presurgical workup of patients with epilepsy. Yet, there is no agreement on the timing or circumstances in which NP testing should be ordered in the evaluation of children with epilepsy. This article addresses this controversial question. The early identification of learning or of mental health problems constitutes one of the arguments in support of NP testing. The relative unavailability and high cost of NP testing, the potential inappropriate labeling of children as "learning disabled," and the risk of attached stigma are some of the arguments in support of limiting NP testing to children suspected of having cognitive or behavioral problems. Nevertheless, we concur that at the least, the need for NP testing should be considered by clinicians in the course of their evaluation of every child with epilepsy.     

病毒性脑炎引起Klüver-Bucy综合征二例报告

kluver-Bucy综合征是双颞叶前部损害而引起的严重记忆障碍、情绪及行为的改变,如不认识亲人、爆发情感、不知恐惧、性欲增强、食欲增加等症状。病毒性脑炎并不少见,而病毒性脑炎引起Kluver-Bucy综合征较罕见。     

Successful treatment of severe disruptive disorder featuring symptoms of the Klüver-Bucy Syndrome following a massive right temporal-parietal hemorrhage

We know little about effective treatment for patients suffering from partial or complete Klüver-Bucy Syndrome (KBS) and other disruptive behaviors following a stroke. Reported cases have shown that certain medication, given alone or combined, can be partially effective. In this specific case study, we will try to demonstrate the effectiveness of a combination of carbamazepine, clonidine, quetiapine and methylphenidate in the alleviating of these symptoms. The wide range of symptoms found in KBS led us to use several kinds of psychotropic medication in spite of the inherent risks associated to polypharmacy.     

伴Klüver-Bucy综合征的单纯疱疹病毒性脑炎一例

<正>患者男性,68岁,因"发热头痛4d,加重伴人事不知1d"入院。4d前出现发热头痛,发热无明显规律,最高达39℃,头痛位于整个头部,呈昏胀痛,期间伴有恶心呕吐1次,为胃内容物,非喷射性。当地医院曾给予输液治疗症状未见好转,并于入院前1d出现胡言乱语,烦躁,摸索样动作,小便失禁。查体:T 38.0℃,BP 130/85mmHg,意识模糊,烦躁,语言含糊不清,双上肢无目的摸索样动作,四肢肌张力增     

Early onset facioscapulohumeral muscular dystrophy – Long-term follow-up of a patient with total facial diplegia

We report a patient with early onset facioscapulohumeral muscular dystrophy type 1 (FSHD1) who was not diagnosed until 48 years of age. She developed progressive facial diplegia from the age of 4–5 years followed by limb muscle weakness. Motor nerve conduction was normal, myopathic changes were seen electromyographically. Creatine kinase activity was mildly increased at the beginning. Muscle biopsy at 8 years suggested a neurogenic pattern, a second biopsy at age 30 was chronic myopathic with fibre calibre variation. The patient lost the ability to walk at age 44. When last seen she had total facial diplegia, no active movements in her limbs, mild kyphoscoliosis and a rigid thoracic spine. Molecular studies revealed a shortened D4Z4 fragment confirming the diagnosis FSHD1. Her family history was unremarkable, suggesting a de novo mutation. This report is to illustrate the evolving phenotype of early onset FSHD1 with predominating facial palsy.     

Guillain-barré-like syndrome in a child with lead poisoning

Lead poisoning is a medical condition caused by increased levels of the heavy metal lead in the body, generally resulting from environmental exposure. Lead poisoning can cause a variety of symptoms which vary depending on the individual and the duration of lead exposure; lead poisoning is more noxious to children than adults, because it can damage the brain and nerve development of children.We report a case of lead poisoning in a 5-year-old girl caused by accidental ingestion of a metal ring. Simultaneously, the child showed anemia, encephalopathy, and peripheral neuropathy with albuminocytological dissociation mimicking Guillain-Barré syndrome.This case suggests that anemia with signs of peripheral and central nervous system damage could be considered as a possible manifestation of lead poisoning in children.     

Electrophysiological signs of permanent axonal loss in a follow-up study of patients with Guillain-Barré syndrome

The neurophysiological mechanisms for persisting impairment of motor function after Guillain-Barre syndrome (GBS) were assessed in 37 unselected patients 1-13 years after diagnosis. For evaluation of reinnervation and axonal loss, macroelectromyography (macro-EMG) including measurement of fiber density (FD) was performed. Data from neuropathy symptom score, neuropathy disability score, nerve conduction studies, and quantitative sensory examination were ranked and summed to a neuropathy rank sum score (NRSS). The isokinetic muscle strength at the ankle was measured. Signs of axonal loss with increase of either macro motor unit potential (macro-MUP) amplitude or FD occurred in 76% of patients. The macro-MUP amplitude correlated with muscle strength and with NRSS. Patients with evidence of residual neuropathy had increased macro-MUP amplitude and FD as well as decreased muscle strength compared to patients without evidence of residual neuropathy. We conclude that axonal loss takes place in a substantial number of GBS patients and is associated with permanent muscle weakness caused by insufficient reinnervation. Possible patterns of pathology are discussed in relation to the macro-EMG findings.