Long-term neuropsychological follow-up of a child with Klüver–Bucy syndrome

We describe the case of a 10-year-old girl who developed behavioral changes consistent with Klüver–Bucy Syndrome following Listeria meningoencephalitis at 2½ years of age. MRI at age 4 revealed evidence of diffuse brain atrophy with predominant temporal lobe involvement. Electroencephalograpy at 9½ years of age showed abnormal electrical discharges from the left temporal area. Follow-up MRI with volumetric analysis of the mesial temporal structures at 9 years of age demonstrated decreased hippocampal volume bilaterally. Consistent with the morphological abnormalities, serial neuropsychological evaluations demonstrated expressive and receptive language impairment and an amnestic syndrome that significantly decreased her ability to make new declarative memories and maintain adequate academic progress.     

Partial Klüver–Bucy syndrome in a patient with acute disseminated encephalomyelitis

The symptoms of Klüver–Bucy syndrome (KBS) include hyperorality, hypersexuality, visual agnosia, hypermetamorphosis and decreased motor or vocal reaction to fear- or anger-provoking stimuli. This syndrome has been associated with a wide variety of neurodegenerative disorders, as well as traumatic, non-traumatic and infectious brain injuries. We report an 11-year-old boy who developed a fairly classical presentation of KBS, presumably in the setting of post-infectious acute disseminated encephalomyelitis (ADEM). This patient’s presentation is a reminder of this rare syndrome and extends the clinical manifestations of ADEM, which is a relatively more common condition.     

Long-term neuropsychological follow-up of a child with Klüver-Bucy syndrome

We describe the case of a 10-year-old girl who developed behavioral changes consistent with Klüver-Bucy Syndrome following Listeria meningoencephalitis at 2? years of age. MRI at age 4 revealed evidence of diffuse brain atrophy with predominant temporal lobe involvement. Electroencephalograpy at 9? years of age showed abnormal electrical discharges from the left temporal area. Follow-up MRI with volumetric analysis of the mesial temporal structures at 9 years of age demonstrated decreased hippocampal volume bilaterally. Consistent with the morphological abnormalities, serial neuropsychological evaluations demonstrated expressive and receptive language impairment and an amnestic syndrome that significantly decreased her ability to make new declarative memories and maintain adequate academic progress.     

Klüver & Bucy syndrome: an investigation of social and affective cognition

ABSTRACT

Klüver-Bucy syndrome (KBS) leads to important behavioral symptoms and social maladaptation. Rarely described, no previous study has investigated its social and affective cognitive profile. We report the case of ASP, a patient who developed a complete KBS at 9 years that evolved into an incomplete KBS. Orbitofrontal and temporal damages were evidenced. While a classic neuropsychological assessment showed a preserved global functioning, an extensive evaluation of her social and affective cognition (reversal learning, decision-making, emotion recognition, theory of mind, creative thinking) showed remarkable deficits. The relevancy of such findings for the characterization KBS and the field of neuropsychology are discussed.     

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.     

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.     

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.     

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     

Prolonged left homonymous hemianopsia associated with migraine-like attacks in a child with Sturge–Weber syndrome

We report a patient with Sturge–Weber Syndrome (SWS) who developed migraine-like headaches followed by cerebral infarction. SWS without facial nevus was diagnosed based on calcification detected by CT and pial angioma detected by enhanced MRI. His migraine-like headaches were preceded by left homonymous hemianopsia, which persisted for more than 60 min. Although homonymous hemianopsia disappeared with cessation of the headache until 13 years of age, from age 14 years onward, this homonymous hemianopsia persisted after the headaches ended. Moreover, reduced cerebral blood flow was seen in the right occipital area on SPECT. At first, his left homonymous hemianopsia persisted for several months after the headache disappeared, but it had recovered completely. However, the durations of episodes of left homonymous hemianopsia, which persisted after headache disappearance, gradually became longer. At last one year after his first admission, the visual defect had become permanent. SWS is well known to be associated with migraine attacks and hemianopsia. However, the course of our present patient, i.e. recurrent homonymous hemianopsia, associated with migraine-like headaches becoming permanent, is rare. The pathophysiological mechanism underlying this clinical course is uncertain. The efficacy of valproate and propranolol as preventive therapy has been inadequate, to date.     

Henoch-Schönlein purpura in a child with Guillain-Barré syndrome

A case of Henoch-Sch?nlein purpura with Guillain-Barré syndrome in a 3-year-old-girl is presented. This association is extremely rare. During the course of Guillain-Barré syndrome, a decrease in plasma factor XIII activity was noted. When the Guillain-Barré symptoms improved, the factor XIII activity returned to normal. In the present case of Henoch-Sch?nlein purpura complicated by Guillain-Barré syndrome, the factor XIII level was measured, a novel feature of this study. The findings suggest the involvement of factor XIII, which is characteristically involved in Henoch-Sch?nlein purpura, also is involved in the associated Guillain-Barré syndrome.     

Glioblastoma arising in a patient with Mayer–Rokitansky–Küster–Hauser syndrome

Mayer–Rokitansky–Küster–Hauser syndrome is a rare developmental disorder marked by the congenital absence of the uterus and vagina. The syndrome has been associated with tumors of the female reproductive system, but rarely in other organ systems and to our knowledge, never in the brain. We report a glioblastoma in a 34-year-old patient with Mayer–Rokitansky–Küster–Hauser syndrome.     

Anti-GT1a IgG antibodies in a child with severe Guillain-Barré syndrome

This report describes a male, age 8 years 10 months, with severe Guillain-Barré syndrome after Campylobacter jejuni infection. The patient developed fulminant muscle weakness, external ophthalmoplegia, bulbar palsy, and respiratory distress. A high level of serum monospecific anti-GT1a immunoglobulin G antibody was detected. He was treated with intravenous immunoglobulins and artificial ventilation. Two years after the onset, the patient still suffered from residual leg weakness and foot drop. After 3 years and clinical recovery, the antibody was no longer detectable. This report presents the first case in childhood suggesting an association between a severe Guillain-Barré syndrome after C. jejuni enteritis with monospecific anti-GT1a immunoglobulin G antibody.     

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.     

Post operative delirium with hyponatriemia after transurethral resection of the prostate: a case of transurethral resection syndrome?

Postoperative delirium is one of the most spectacular, frightening and misdiagnosed postoperative complications of surgery. We describe the case of a caucasian 77-year-old male patient, who developed a severe postoperative delirium after combined transurethral resection of the prostate and cystolithotripsy. This systemic and unpredictable complication of endoscopic surgery is caused by excessive absorption of electrolyte-free irrigation fluids, leading to brain edema and metabolic encephalopathy. The clinical spectrum ranges from asymptomatic hyponatraemia, to electrocardiographic (ECG) changes, nausea, vomiting, convulsions, coma, pulmonary edema, cardiovascular compromise and death. Because of the heterogeneous clinical presentation diagnosis can be difficult. In a patient who develops alterations of consciousness with evidence of hypervolemia and hyponatremia after endoscopic surgery, transurethral resection syndrome must be considered.     

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.