Affiliations: Department of Neurology, Sanjay Gandhi PGIMS, Lucknow,
India | Department of Genetics, Sanjay Gandhi PGIMS, Lucknow,
India | Department of Neurosurgery, Sanjay Gandhi PGIMS,
Lucknow, India
Note: [] Correspondence: Dr. Sanjeev Jha, Department of Neurology, Sanjay
Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road, Lucknow-
226014, India. Fax: +91 0522 2668017; E-mail: [email protected]
Abstract: Kluver-Bucy syndrome (KBS) includes increased oral exploration,
placidity, indiscriminate hypersexuality and altered dietary habits, reported
to develop following insult to temporal lobes. Psychic blindness (inability to
recognize familiar objects) and hypermetamorphosis (strong tendency to react to
visual stimulus) are other constituents of this neurobehavioral syndrome. We
encountered 12 children with features of KBS among cases of delayed mental and
motor milestones following cerebral birth anoxia consequent to hypoxic-ischemic
encephalopathy. Period of the study was 11 years (January 1994–June 2004).
Diagnosis of hypoxic-ischemic encephalopathy was based on birth records,
pediatricians' report, history provided by mother or relatives, clinical and
radiological features. Their age ranged between 6 and 14 years. Development of
hyperorality, sexual hyperactivity and abnormal behavior suggested the presence
of KBS in them. Hyperorality and abnormal behavior were common manifestations
in all patients. Hyperorality was in form of an attempt to put non-edible
substances in mouth, while abnormal behavior manifested as hostility,
irritability or placidity. Hypersexuality was evident in seven children and
presented as fondling, holding or rubbing the genitals along with paroxysmal
pelvic thrusting. Seizures were present in eight patients. In five of these,
they were well-controlled (more than 60% decline in frequency), with
improvement in behavior in three of them, following control of seizures. In
other patients, behavior was unaltered. It appears that KBS may be more common
than was suspected previously in children of cerebral birth anoxia consequent
to hypoxic ischemic encephalopathy. Though it may manifest with a wide clinical
spectrum, hyperorality is its commonest constituent. Seizures are commonly
associated and their control may help in improving the behavioral disorder.
