Effects of selective serotonin re‐uptake inhibitors on behavior in Klüver–Bucy syndrome during childhood

Abstract

Bilateral lesions in the anterior temporal lobe result in a neurobehavioral condition termed Klüver-Bucy syndrome (KBS). 1,2 This syndrome is characterized by psychic blindness (visual agnosia with inability to recognize objects), excessive oral tendencies, hypermetamorphosis (impulsive reaction to visual stimuli), placidity, changes in feeding behavior, and hypersexuality. Young animals and children with bi-temporal lesions, however, have different symptoms from those listed above. Psychic blindness in human children often manifests as an inability to recognize significant persons (discriminate family members from strangers), while hypermetamorphosis and hypersexuality are not usually significant. 3,4 These individuals also exhibit limited social contacts and have prominent self-directed stereotypic behaviors. 4 Herpetic encephalitis is one of the most frequent causes of bi-temporal lesions during childhood, 3 possibly due to viral invasion of the amygdala via the olfactory tract. We report here a patient with sequelae of neonatal Herpes simplex encephalitis who had childhood KBS. Selective serotonin re-uptake inhibitors (SSRI) affected the social behavior of this patient. We discuss the significance of this effect with regard to the pathophysiology of autism. Case reportThe patient was a boy who suffered from febrile illness 8 days after birth. He was diagnosed with neonatal herpetic meningoencephalitis based on an elevated cell count, positive polymerase chain reaction (PCR) for Herpes simplex virus-1 (HSV-1) in the cerebrospinal fluid, and elevation of serum HSV-IgM. The baby was discharged after treatment with acyclovir for 3 weeks. Computed tomography (CT) on discharge showed high-density regions over the bilateral anterior temporal lobe and insular cortex (Fig. 1a,b). Infantile spasms appeared at 5 months of age, and were successfully treated with adrenocorticotropic hormone. Hypsarrhythmia disappeared, and electroencephalography showed rare and equivocal spikes in the central areas. Psychomotor developmental delay, however, became evident thereafter. Although the patient could roll over at 8 months of age, choreic movements of the extremities rendered him unable to hold objects in his hands. These involuntary movements disappeared by 11 months of age, and soon after he could maintain a sitting position without support. Brain CT at this stage showed marked atrophy with calcification of the bilateral amygdala and anterior hippocampus (Fig. 1c). Calcification was also evident over the bilateral insular cortex, but no other brain structures showed abnormalities (Fig. 1d). He showed the stereotypical habit of placing his fingers into his mouth, along with toys, clothing, and the hands of other individuals. He had been eating paste food due to mild dysphagia, and appetite loss appeared during this period. He showed no emotional empathy for other individuals, and even smiled when scolded. Eye contact was brief and inconsistent. He did not express sadness when separated from his mother. He enjoyed tactile and a...

Pediatric Klüver–Bucy Syndrome: Report of Two Cases and Review of the Literature

“…Occasionally, some medications help with KBS symptomatology, such as carbamazepine, 2,25 which seems especially helpful with the affective component, or the selective serotonin reuptake inhibitors (SSRIs). 11,26 Behavior alterations and decrease agitation have been treated with haloperidol or risperidone. Clonidine or methylphenidate can be useful in selected cases.…”

Critical role of the right uncinate fasciculus in emotional empathy

“…Several cases of herpes encephalitis, 32 limbic encephalitis due to potassium channel antibodyassociated encephalopathy, 33 and juvenile neuronal ceroid lipofuscinosis 34 have been reported to have impaired empathy, in some cases associated with Kl€ uver-Bucy syndrome. 32,34 Although the damage to bilateral mesial temporal lobe, especial temporal pole, is usually emphasized, these individuals likely have damage to the uncinate fasciculus as well. Children with neuropsychiatric disorders due to brain injury have significantly lower fractional anisotropy in bilateral uncinate fasciculus.…”

“…Our data are complementary, as they show that individuals who were neurologically normal just days before show acute disruption of empathy that correlates with the degree of damage to the uncinate fasciculus. Several cases of herpes encephalitis, limbic encephalitis due to potassium channel antibody‐associated encephalopathy, and juvenile neuronal ceroid lipofuscinosis have been reported to have impaired empathy, in some cases associated with Klüver–Bucy syndrome . Although the damage to bilateral mesial temporal lobe, especial temporal pole, is usually emphasized, these individuals likely have damage to the uncinate fasciculus as well.…”