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

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

Response to Letter to the Editor

Dear Editor,

With great interest, we read the article Pediatric Klüver-Bucy Syndrome: Report of Two Cases and Review of the Literature by N. Juliá-Palacios et al.

As the authors included in their in-depth review ∼51 patients with pediatric Klüver–Bucy syndrome (KBS) 4 patients with KBS after traumatic brain injury (TBI) and discussed the limited knowledge of the long-term persistence of KBS in children, the following might be of interest to you: we diagnosed more than 40 young patients with KBS—or rather Klüver–Bucy Phase (KBP)—in our neuropediatric rehabilitation center in the 1990s. These were patients within a cohort of ∼500 patients aged 1 to 17 years sent for early rehabilitation after severe traumatic or hypoxic brain injury. By then, our rehabilitation team was trained to use the (modified) Innsbruck Remission Scale (IRS)[2] ([Table 1]) in patients with prolonged disorders of consciousness, with phase 4 and 5 as KBP. However, we stopped using the IRS and introduced instead RemiPro as a routine instrument in early rehabilitation (developed by Ellen Romein and Melanie Hessenauer since 1995 at our center)[3] [4] ([Table 2]).

Table 1 Modified Innsbruck Remission Scale—eight phases[1]

Phase 1	Deep somnolent, temporary open eyes, optical fixation, sleep–wake rhythm fatigue regulated, primitive emotional reactions, primitive motor patterns partly diminished, flexed-stretched extremity position, remaining mass movements, rigido-spasticity
Phase 2	Somnolent, optical tracking, sleep–wake rhythm, begin of daytime regulation, emotional reaction tendency to differentiation, primitive motor patterns tendency aim directed, diminished mass movements, tendency to finalizing, diminished flexed-stretched body position, diminished rigidospasticity
Phase 3	Beginning of responsible wakefulness, somnolence phases, following simple commands, emotional reaction differentiated (positive, negative), primitive motor patterns differentiated (higher organized grasping reflexes, oral reflexes) oral feeding accepted, first finalized movements initiated by commands
Phase 4	Klüver–Bucy Phase, wakeful, sleep–wake rhythm daytime regulated, object grasping with attempt to bite and chew, no recognition of the objects, increased interest for the genital region, reacting to simple orders, producing primitive sounds, beginning signs of local brain lesions, rest of flexed-stretched body position, rest of rigidospasticity
Phase 5	Post Klüver–Bucy Phase, wakeful, sleep–wake rhythm day–night adapted, slight rest of flexed-stretched body position, slight signs of spasticity, diminishing of the Klüver–Bucy patterns, increasing of finalized movements, production of simple words, upcoming of local brain lesions signs
Phase 6	Phase of Korsakow-Symptoms, fully awake, disorientation, fully guidable fatigue phases, rest of primitive motor patterns, rest of spasticity, directed finalized movements, guidable, beginning of walking
Phase 7	Phase of the amnestic syndrome, fully awake, day–night regulated sleep–wake rhythm, rest of disorientation, fully guidable, severe memory disturbances, rest of primitive motor patterns, slight signs of spasticity, prompt finalized movements, marked diffuse and local cerebral lesions possible, symptoms of bed rest syndrome
Phase 8	Phase of the end of remission state; beginning of defect state, undisturbed consciousness, normal sleep–wake rhythm, increased fatigue phases, marked symptoms of diffuse and/or local cerebral lesions (neurological, cognitive behavior deficits) possible, symptoms of Bed Rest symptoms, remarks of different handicaps

Table 2 RemiPro—six levels[3] [4]

Sleep–wake level	The child reacts occasionally in participative situations with one or more of the following abilities: a. The child shows during this participative situation one or more vegetative reactions such as increased muscle tone, perspiration, blushing, increases in pulse, blood pressure, and/or breathing. The person who carries out this activity considers this as positive b. The child relaxes during this participative situation and appears to feel more comfortable (e.g., decreased muscle tone or pulse), improved breathing (e.g., increased oxygen saturation, deeper and calmer breathing) c. The child appears more awake during this participative situation which can be observed by brief pausing, accidental fixation, or changes in facial or eye expression
Perception level	The child reacts repeatedly in participative situations with one or more of the following abilities. The observable abilities indicate that the child perceives his or her environment a. The child turns towards a participatory situation or person for 2 to 3 seconds b. The child smiles or shows other expressions of enjoyment such as making sounds c. The child makes involuntary movements during this participative situation
Communication level	The child intentionally carries out individual actions during participatory situations. The child interacts self-initiated with his/her environment. Characteristics of this level are attempting trial and error The performance of first self-initiated actions is the child's ability
Independence level	The child demonstrates growing independence in a familiar environment. The presence of a support person is needed to successfully complete the activity and/or for safety reasons. The child needs individual support to participate in a group The performance of daily activities is the child's ability
Group level	The child works in a structured group together with others. He/she can cope independently with the daily activities, in doing so he/she still can be a bit slow and now and then might need some additional support. In unfamiliar situation or with complex activities, the child needs support The performance of daily activities is the child's ability
Participation level	The child/adolescent participates in all areas of life that are normal for his/her age and context (concept of activity according to International Classification of Functioning [ICF]). The child/adolescent develops its being in all areas of life, which are important to him/her in a manner equal to children/adolescents without a health-related handicap of the body function or body structure or the activity (concept of participation according to ICF)

Without having studied systematically, we observed in the 1990s that

1. Our patients with KBP mainly had a severe TBI (not hypoxic!), and all had recovered from a prolonged disorder of consciousness, often fulfilling the criteria of a persistent vegetative state (apallic syndrome, nowadays: unresponsive wakefulness syndrome)

2. None of our patients persisted in KBP (usually lasting 1–4 weeks). As a rule, we calculated the duration of KBP double as long as phase 1 of IRS

3. Patients with a pronounced KBP after TBI often had a particularly good outcome

4. As discussed in ref.,[1] it was also often difficult for us to characterize “increased sexual activity” as a core symptom for KBS in infants and young children

5. Seizures were frequent in our patients with KBP, but we think these were mainly correlated to the cortical damage and not linked to an obvious lesion in the limbic system.

• References

• 1 Juliá-Palacios N, Boronat S, Delgado I, Felipe A, Macaya A. Pediatric Klüver-Bucy syndrome: report of two cases and review of the literature. Neuropediatrics 2018; 49 (02) 104-111
• 2 von Wild K, Laureys ST, Gerstenbrand F, Dolce G, Onose G. The vegetative state--a syndrome in search of a name. J Med Life 2012; 5 (01) 3-15
• 3 Romein E, Hessenauer M, Kluger G. Remi-Pro- eine standardisierte und valide Methode zur Dokumentation des Remissionsverlaufs in der Rehabilitation von Kindern und Jugendlichen im „Wachkoma“. In: Leben im Koma: Interdisziplinäre Perspektiven auf das Problem des Wachkomas. Ed. Jox RJ, Borasio GD, Kühlmeyer K. Kohlhammer. Stuttgart: Thieme Verlag; 2011: 75-106
• 4 Romein E, Hessenauer M. Teilhabe von Kindern im Wachkoma erfassen. Ergopraxis. Stuttgart: Thieme; 2017: 33-35