Response to Letter to the Editor

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

Dear Editor,

We thank Drs. Kluger and Berweck for this very interesting addition to our work. Their comments based in their large (unpublished) cohort are very relevant since the notion of a Klüver-Bucy syndrome (KBS) phase as a frequent stage occurring during the recovery of a persistent vegetative state (PVS) is not widely recognized among child neurologists. As they point out in their letter, behavioral disturbances within this group are more common in those following traumatic brain injuries (TBI). KBS has been noted to occur even in the instances of apparently minor closed head injury.[1] [2]

The transient nature of the KBS in this population group is intriguing. While some degree of axonal diffuse damage affecting the mesiotemporal system could be entertained as the underlying cause of post-traumatic KBS, radiological evidence of chronic temporal lobe involvement has been provided in some instances but, to our knowledge, no detailed analysis has addressed temporal involvement in a large series of patients with PVS like the one referred to by Kluger and Berweck. In this regard, timely imaging to demonstrate anatomical or functional (hypometabolism) impairment of the mesiotemporal system in patients entering the KBS phase after PVS is probably warranted.

KBS may be underreported in the context of the patient with severe brain injuries. Conversely, the use of the Innsbruck remission scale, including a specific KBS phase and post-KBS phase, may have led to some degree of generalization of a usually very distinct diagnostic category. Whatever the case, it is conceivable that persistent dysfunction of the hippocampal–amygdaloid complex or its projections following recovery of aim-directed primitive motor patterns and perceptive systems may account for the delayed emergence of KBS in post-TBI PVS, with symptoms presumably lasting until mesiotemporal reconnection is completed.

• References

• 1 Bhat PS, Pardal PK, Das RC. Partial Kluver-Bucy syndrome as a delayed manifestation of head injury. Ind Psychiatry J 2009; 18 (02) 117-118
• 2 Morcos N, Guirgis H. A case of acute-onset partial Kluver-Bucy syndrome in a patient with a history of traumatic brain injury. J Neuropsychiatry Clin Neurosci 2014; 26 (03) E10-E11