Sertraline for Klüver—Bucy syndrome in an adolescent

Dear Editor,

Klüver–Bucy syndrome (KBS), a neurobehavioral syndrome first described in rhesus monkeys, is characterized by visual agnosia, placidity, hypermetamorphosis (strong tendency to react to visual stimulus), increased oral exploration, altered dietary habits, and hypersexuality [3]. In addition, human cases have features such as fluent aphasia, amnesia, dementia and seizures [3]. Although this disorder is generally considered to have an unfavorable outcome, we wish to report a case of KBS who didn't respond to a combination of an antipsychotic and carbamazepine but responded when sertraline was added.

Ms. A, a 14-year-old adolescent presented with history of hypersexuality, hyperphagia, oral tendency, hyperactivity, misidentification and poor personal care of 4 years duration. Pre-morbidly she was an intelligent and social girl with a good academic record, but 4 years ago she developed an illness characterized by severe headache, high-grade fever, and was in a comatose state for 15 days. Although her parents were not sure about the exact nature of this illness, the picture was consistent with encephalitis. While recovering from her illness, the patient started exhibiting disinhibited behaviors such as disrobing in front of others, manipulating her genitals, and making sexual advances toward her father. She would lick any object lying on the ground and whenever she got an opportunity, she would rush to the toilet and try to put urine and feces in to her mouth (urophagia and coprophagia, respectively). She also exhibited hyperphagia and would eat three times the usual amount of meal at a time. She was impulsive and would try to snatch any object and slap others without reasons. She would misidentify parents and relatives and would call them by different names. A meaningful mental state examination was not possible in view of her irrelevant speech and impulsive and disorganized behavior. Her neurologic examination did not show any long tract signs. Her blood work up was unremarkable and so was an EEG. However, a head CT scan showed diffuse mild cerebral atrophy with no focal abnormalities.

The patient was started on carbamazepine titrated up to 600 mg per day without any improvement in symptoms. Three weeks later, risperidone 4 mg per day was added, but only mild improvement was noticed. Six weeks after this combination, sertraline 25 mg was added which was increased to 100 mg over a period of 2 weeks. Surprisingly, 6 weeks after commencing sertraline, all her symptoms improved remarkably, including hypersexuality, disinhibited behavior, hyperorality, urophagia and coprophagia. On follow-up 2 months later, she had stopped taking sertraline for 2 weeks (while continuing other drugs) and had worsening of the above-mentioned symptoms; reinstituting sertraline again ameliorated the symptoms. The patient was doing well for next 6 months till her last follow-up.

Our case exhibited most of the symptoms of KBS. Although KBS has been shown to be associated with demonstrable bilateral lesions in the anterior temporal horn or amygdale [4], other authors have reported that lesions in the amygdala are not necessary for KBS in animals or humans [1]. This is consistent with our patient who showed diffuse brain atrophy but no localized lesions. Carbamazepine and antipsychotics have been previously reported to be effective in managing some of the behavioral symptoms of KBS [2]. There is only one report describing the successful use of selective serotonin reuptake inhibitors (SSRIs) in two patients with KBS secondary to head trauma [5]. In one of these cases, the patient didn't respond to carbamazepine alone but responded only after being started on fluoxetine. In the second case, the patient responded to a combination of antipsychotics and sertraline. That sertraline may have an independent effect on symptoms of KBS is further bolstered by relapse of symptoms on withdrawing this drug and their resolution again on rechallenge in our patient. Some of the symptoms of KBS are compulsive in quality and may reflect a disturbance of aggression regulation and impulse control. It may be worth exploring if the known efficacy of SSRIs against these symptoms cluster may play a role in treating KBS.