the presence of consciousness in the absence of the cerebral cortex
DESCRIPTION
The presence of consciousness in the absence of the cerebral cortexTRANSCRIPT
Article Lead Author Beshkar Majid Date 2008 Article The Presence of Consciousness in the Absence of the Cerebral Cortex
1 Pain felt at
a If the article specifically asserts unborn children feel pain at what postshy
fertilization age
b Page
2 Nociceptors
a Ifthe article states nociceptors are present at what post-fertilization age
b Page
3 Thalamus link
a If the article states nerves link nociceptors to the thalamus at what postshy
fertilization age
b Page
4 Subcortical plate link
a If the article states nerves link to the subcortical plate at what post-fertilization
age
b Page
S Noxious stimuli reaction
a Does the article refer to reaction to noxious stimuli At what post-fertilization
age
b Page
6 Stress hormones
a Does the article refer to increase in stress hormones with noxious stimuli At
what post-fertilization age
b Page
7 Long-term effects
a Does the article describe long term harmful effects from exposure to noxious
st imuli
b Page
8 Fetal anesthesia
a Does the article refer to use of fetalanesthesia and its effect At what postshy
fert ilization age
b Page
9 Cortex
a Does t he article relate to the asserted need for cort ical involvement to
experience pain How
b Page 553 Right Column Last Paragraph However there are some pieces of
evidence that these children [with hydranencephaly] are able to experience at
least some levels of consciousness
Page 554 Left Column First Paragraph Shewmon et al (1999) reported cases
of four children aged 5-17 with hydranencephaly involving complete or nearly
complete absence of cerebral cortex
SYNAPSE 62553-556 (2008)
Short Communication
The Presence of Consciousness in the Absence of the Cerebral Cortex
MAJID BESHKARmiddot Tehran University of Medical Sciences Tehran Iran
KEYWORDS hydranencephaly consciousness vegetative state behavior cerebral cortex
ABSTRACT Hydranencephaly is a rare neurological condition in which the cereshybral hemispheres are either absent or severely compromised It is widely believed that children with hydranencephaly are not conscious and therefore are routinely classishyfied into the diagnostic criteria of vegetative state However ther~ are several pieces of behavioral evidence clearly indicating the presence of consciousness in such patients Here I review these behavioral evidence and argue how qlisclassification of these patients and assigning them a lack of consciousness have far-reaching implicashytions in terms of both clinical and theoretical neuroscience Synapse 62553-556 2008 () 2008 Wiley-Liss Inc
INTRODUCTION
Hydranencephaly is a rare neurological condition in which the cerebral hemispheres either fail to develop for genetic-developmental reasons or are massively compromised by trauma of a physical vascular toxic hypoxic-ischemic or infectious nature at some stage of their development (Merker 2007) The disorder which occurs in less than 1 per 10000 births worldshywide is characterized by near-total or total absence of the cerebral cortex and basal ganglia The thalami pons cerebral peduncles and cerebellum are usually present as may be a small amount of occipital-lobe frontal -lobe and temporal-lobe tissue The damaged brain tissues undergo extensive resorption and are replaced by cerebrospinal fluid filling otherwise empty meninges lining a normally shaped skull Hydranenshycephaly must be distinguished from the even more severe neurological condition called anencephaly a disorder of the neural tube beginning very early in developmen t which leads to virtually no brain devel shyopment
The amount of brain ti ssue that each child with hydranencephaly h s varies from child to child Many of the children a re missing most of their brain ti ssues above the brainstem However the loss of cerebral h eroisph res must be massive to be designated hydrashynencephaly although it is seldom complete While variable remnants of th cerebral cortex may be spared these coTtic I remnant are most proba bly not connected to the thalamus (whi te matter loss often
interrupts the visual radiations for instance) and are not even locally functional On autopsy such tissue may be found to be gliotic on microscopic examination or to exhibit other structural anomalies indicating loss of function (Merker 2007)
An infant born with hydranencephaly may initially present no conspicuous symptoms and occasionally the condition is not diagnosed until several months postnatally when developmental milestones are misshysed In the course of the first year of life these infants typically develop a variety of complications that always include motoric ones (tonus spasticity cerebral palsy) Although survival beyond 6 months is rare with hydranencephaly prolonged survival even up to 34 years can occur (Counter 2007)
It is widely believed among neurologists that chilshydren with hydranencephaly are not conscious Howshyever there are some pieces of evidence that these children are able to experience at least some simple levels of consciousness (Fig 1) Scientific literature about this issue is very poor and there are only two published accounts describing observations to the effect that children with hydranencephaly experience conscious states
Corresponde nco to No 2 Sahel 1 Eas l Arghava n Saadal Abrl Tehron I Iun E~ma l l mnjid bcs h klu-yuhfJocom
RIcevec 23 Octuber 2007 Accepl0Ji 20 JflI1LIJrv ~OOtlt
o I 1 n 100Vy 11 20521
tublil)h online 24 Apnl 2O(1B In Wiley 11lugtrampiente hvwwinterscience_wilcshyL 11I lt l
1)2008 WILlW-LlSS INC
554 MBESHKAR
Fig 1 Nikki a hydranencepbalic girl in the arms of her mother She has turned toward someone sitting on her left (you can see the knee to the right in the picture) This picture shows the alertness of the girl and her smile very well For this picture I am indebted to Bjorn Merker for receiving permission from Nikis mother on my behalf
CONSCIOUSNESS IN HYDRANENCEPHALICS Shewmon et a1 (1999) reported the cases of four
children aged 5-17 with hydranencephaly involving complete or nearly complete absence of cerebral corshytex The authors observed that these children posshysessed a variety of cognitive capacities that were inshydicative of ordinary consciousness including person recognition social interaction (smiling when spoken to giggling when played with vocalization with music therapist) functional vision (object discriminashytion fascination with own reflection) musical prefershyences orienting- toward and smiling at someone callshying appropriate affective responses goal-directed motor behavior (scooting on back to reach a goal) and associative learning (using limited receptive vocabushylary to correctly look at an object)~ On the basis of their observations the authors concluded that each of the children they assessed was conscious by the criteshyria of a standard neurological examination
One of thes children was able to visually interact with the en vironment scooting around the house while avoiding collision with walls and furniture More in teresting was the observation that he became fascinated with h is own reflection in a mirror and deshyspite efforts to distract him he kept turning back to the mirror exploring it intently and smiling The capacity to recognize oneself in the mirror is comshymonly considered to be an indicator of self-a wareness an d is usually explored in animals by the so-called mirror test This hydranencephalic child wa appashyrently as interested in bis r fle tion as any in fant or animal that passes the minor test Although the authors interpreted thi particular behavior as an inshydication of consciousness ther are somegt counteTshy
ynapse
arguments For example Merker argues that Most animals and human infants mirror reactions are social reactions (that is they treat the mirror image as a conspecific and NOT as themselves) so reacting to and being fascinated by a mirror by no means implies self-recognition In the case of [this hydranenshycephalic subject] we cannot even be sure that the behavior was of the nature of social reactions (though the smile raises that possibility) they might simply have been reactions to reflected movements and so on (personal communication)
Another child in this study exhibited discriminative awareness of the environment for example consisshytently distinguishing close family members from others At the approach or touch of strangers she assumed a fearful affect became tense and withdrew but relaxed to the touch and voice of mother The more familiar someone was the more she w(mld relax move spontaneously and vocalize She was aware of her mothers presence and became upset if separated She had favorite pieces and types of music to which she would consistently smile and vocalize in contrast to other music to which she consistently remained indifferent
Another child developed a liking for puppies and small children and her face became animated whenshyever she saw them She also showed some orientation capacities when called she would raise her head look at the person and smile Furthermore when an object she was tracking was suddenly taken behind her she would turn in search of it She was very socially interacting and clearly enjoyed being with people and even played with them The authors observed that in an occasion the subject attempted to imitate monosyllables and even uttered ah-ah when coaxed to say mama She also demonstrated some degree of body awareness For example if her face hurt she would stroke it with her hand The most interesting of her cognitive abilities was her capacity of associative learning The authors observed that she startled and stiffened when a vacuum cleaner or hair dryer with a loud unpleasant noise was turned on Mter several such experiences she also stiffened in anticipation if either object (though switched off) was brought near Moreover she developed a small recepshytive vocabulary including bunny rabbit (a stuffed toy) Michael (a family friend) and Pocahontas (an image on her T-shirt) with coaxing and repetition of the question Where is [one of these] she corshyrectly looked at the object or person
Another subject exhibited some limited form of nonshyverbal communication by making cooing sounds expre sions of sadness or pai n smiling in the presence of caregivers and indicatin g preferences through facial expressions and R broad smile He was able to distingui h h is mother s voice from that of his father
555
MOJ ~
~ CONSCIOUSNESS IN THE ABSENCE OF CEREBRAL CORTEX J t
bull
f)
If j
ibull
I ~
i
~ shy
l I J i-
In a recent article Merker (2007) has described his first-hand observations of the behavior of hydranenceshyphalic children as well as his impressions gleaned from the reports of parents of such children On the basis of his observations Merker argues that these children are not only awake and often alert but show responsiveness to their surroundings in the form of emotional or orienting reactions to environmental events He observed that hydranencephaIic children were able to express pleasure by smiling and laughshyter and aversion by fussing arching of the back and crying Furthermore the children responded differshyentially to the voice of familiars and showed prefershyence for certain situations and stimuli over others such as a particular familiar toy More interestingly some of these children demonstrated the capacity of taking behavioral initiatives in the form of instrushymental behaviors such as making noise by kicking trinkets hanging in a special frame constructed for the purpose or activating favorite toys by switches Merker concludes that hydranencephalic children give proof of being not only awake but of the kind of responsiveness to their surroundings that qualifies as conscious by the criteria of ordinary neurological examination
According to Merker another piece of evidence for the existence of consciousness in hydranencephalic children comes from the fact that these children are subject to the seizures of absence epilepsy Seizures of this type are characterized by lapses in consciousness and a lack of response toward external stimuli In a typical episode of absence seizure the patient sudshydenly becomes unresponsive in the midst of normal activities Ongoing activities may continue in the form of automatisms or they may arrest for the durashytion of the seizure episode At the end of such a seishyzure which may last no more than a few seconds the patient who typically remains upright throughout sometimes actively moving resumes conscious activshyities where they were interrupted has amnesia for what transpired during the episode and may have no knowledge that the episode took place at all Merker argues that episodes of absence in this form of epishylepsy represent a basic affliction of consciousness and concludes the fact that these children exhibit such episodes would seem to be a weighty piece of evidence r egarding their conscious status
Merker s arguments for the presence of consciousshyness in hydranencephalic children have not remained unchallenged Behrendt (2007) believes that signs of pleasure or excitement exhibited by anencephalic chilshydren are not necessarily indicative of conscious expeshyrience and they may be regarded as automatic beshyhavioral reactions activated by appropriate stimuli
In opposition to the notion that hydranencephalic children have consciousness Morin (2007) argues that behaviors exhibited b these patients do not
seem to require reflective consciousness and selfshyawareness However it should be mentioned that Merker used the term consciousness in its most bashysic and general sense and in this context consciousshyness is more than reflective consciousness or selfshyawareness
Watkins and Rees (2007) argue that responsiveness to the environment is a capacity exhibited by almost any organism with a central nervous system and cannot be definitely taken as a sign of consciousness Furthermore the behaviors of hydranencephalic chilshydren seem to lack the element of intentionality and many of the reported behaviors could be generated unconsciously or reflexively
CONCLUSION Whether or not children born with hydranenceshy
phaly have consciousness is still controversial Howshyever the body of evidence in favor of the presence of consciousness in these patients seems to be more conshyvincing than evidence and argu-ments against conshysciousness in such children
Finally it is noteworthy that the outstanding webshysite of the International Hydranencephaly Group (wwwhydranencephalycom) provides comprehensive source of information about this disorder The results of an informal nonscientific survey conducted by this group revealed surprising findings that are contrary to what is generally assumed by most neurologists regarding the presence of consciousness in hydranenshycephalic children When parents of such children were asked is your child aware of hislher surroundshyings 74 of the surveyed parents answered Yes 246 answered No and 148 answered Someshytimes When asked is your child aware of objects
2t074 answered Yes 1728 answered No and 3827 answered Sometimes
The rarity of scientific observations similar to those reported by parents could be partly due to the fact that hydranencephalic children are extremely sensishytive to and easily disturbed by changes in environshyment and routine daily activities In unusual and disshyturbing situations the children often fail to manifest any cognitive functions that parents often report When examined after medical stabilization has taken place and in the setting of the home environment upon which these medically fragile children are crushycially dependent they give proof of being conscious (Merker 2007)
ACKNOWLEDGMENT
I am indebted to Bjorn Merker for providing the pi ture of Niki and receiving penllission from Nikis mother
Synapse I
556 M BESHKAR
REFERENCES Behrendt RP 2007 the hypthalamo--tectoperiaqueductal system
Unconscious underpmnings of conscious behaviour Behav Brain Sci 3085--86
Counter SA 2007 Brainstem mediation of the stapedius muscle reflex in hydranenccphaly Acta Oto-Laryngol 127498-504
Merker B 2007 Consciousness without a cerebral cortex A chalshylenge for neuroscience and medicine Behav Brain Sci 30p3-81
Morin A 2007 Consciousness is more than wakefulness Behav Brain Sci 3099
Shewmon DA Holmes GL Byrne PA 1999 Consciousness in congenitally decorticate children Developmental vegetative state as self-fulfilling prophecy Dev Med Child Neurol 41364shy374
Watkins S Rees G 2007 The human superior colliculus Neither necessary nor sufficient for consciousness Behav Brain Sci 30107
vnnplP
b Page 553 Right Column Last Paragraph However there are some pieces of
evidence that these children [with hydranencephaly] are able to experience at
least some levels of consciousness
Page 554 Left Column First Paragraph Shewmon et al (1999) reported cases
of four children aged 5-17 with hydranencephaly involving complete or nearly
complete absence of cerebral cortex
SYNAPSE 62553-556 (2008)
Short Communication
The Presence of Consciousness in the Absence of the Cerebral Cortex
MAJID BESHKARmiddot Tehran University of Medical Sciences Tehran Iran
KEYWORDS hydranencephaly consciousness vegetative state behavior cerebral cortex
ABSTRACT Hydranencephaly is a rare neurological condition in which the cereshybral hemispheres are either absent or severely compromised It is widely believed that children with hydranencephaly are not conscious and therefore are routinely classishyfied into the diagnostic criteria of vegetative state However ther~ are several pieces of behavioral evidence clearly indicating the presence of consciousness in such patients Here I review these behavioral evidence and argue how qlisclassification of these patients and assigning them a lack of consciousness have far-reaching implicashytions in terms of both clinical and theoretical neuroscience Synapse 62553-556 2008 () 2008 Wiley-Liss Inc
INTRODUCTION
Hydranencephaly is a rare neurological condition in which the cerebral hemispheres either fail to develop for genetic-developmental reasons or are massively compromised by trauma of a physical vascular toxic hypoxic-ischemic or infectious nature at some stage of their development (Merker 2007) The disorder which occurs in less than 1 per 10000 births worldshywide is characterized by near-total or total absence of the cerebral cortex and basal ganglia The thalami pons cerebral peduncles and cerebellum are usually present as may be a small amount of occipital-lobe frontal -lobe and temporal-lobe tissue The damaged brain tissues undergo extensive resorption and are replaced by cerebrospinal fluid filling otherwise empty meninges lining a normally shaped skull Hydranenshycephaly must be distinguished from the even more severe neurological condition called anencephaly a disorder of the neural tube beginning very early in developmen t which leads to virtually no brain devel shyopment
The amount of brain ti ssue that each child with hydranencephaly h s varies from child to child Many of the children a re missing most of their brain ti ssues above the brainstem However the loss of cerebral h eroisph res must be massive to be designated hydrashynencephaly although it is seldom complete While variable remnants of th cerebral cortex may be spared these coTtic I remnant are most proba bly not connected to the thalamus (whi te matter loss often
interrupts the visual radiations for instance) and are not even locally functional On autopsy such tissue may be found to be gliotic on microscopic examination or to exhibit other structural anomalies indicating loss of function (Merker 2007)
An infant born with hydranencephaly may initially present no conspicuous symptoms and occasionally the condition is not diagnosed until several months postnatally when developmental milestones are misshysed In the course of the first year of life these infants typically develop a variety of complications that always include motoric ones (tonus spasticity cerebral palsy) Although survival beyond 6 months is rare with hydranencephaly prolonged survival even up to 34 years can occur (Counter 2007)
It is widely believed among neurologists that chilshydren with hydranencephaly are not conscious Howshyever there are some pieces of evidence that these children are able to experience at least some simple levels of consciousness (Fig 1) Scientific literature about this issue is very poor and there are only two published accounts describing observations to the effect that children with hydranencephaly experience conscious states
Corresponde nco to No 2 Sahel 1 Eas l Arghava n Saadal Abrl Tehron I Iun E~ma l l mnjid bcs h klu-yuhfJocom
RIcevec 23 Octuber 2007 Accepl0Ji 20 JflI1LIJrv ~OOtlt
o I 1 n 100Vy 11 20521
tublil)h online 24 Apnl 2O(1B In Wiley 11lugtrampiente hvwwinterscience_wilcshyL 11I lt l
1)2008 WILlW-LlSS INC
554 MBESHKAR
Fig 1 Nikki a hydranencepbalic girl in the arms of her mother She has turned toward someone sitting on her left (you can see the knee to the right in the picture) This picture shows the alertness of the girl and her smile very well For this picture I am indebted to Bjorn Merker for receiving permission from Nikis mother on my behalf
CONSCIOUSNESS IN HYDRANENCEPHALICS Shewmon et a1 (1999) reported the cases of four
children aged 5-17 with hydranencephaly involving complete or nearly complete absence of cerebral corshytex The authors observed that these children posshysessed a variety of cognitive capacities that were inshydicative of ordinary consciousness including person recognition social interaction (smiling when spoken to giggling when played with vocalization with music therapist) functional vision (object discriminashytion fascination with own reflection) musical prefershyences orienting- toward and smiling at someone callshying appropriate affective responses goal-directed motor behavior (scooting on back to reach a goal) and associative learning (using limited receptive vocabushylary to correctly look at an object)~ On the basis of their observations the authors concluded that each of the children they assessed was conscious by the criteshyria of a standard neurological examination
One of thes children was able to visually interact with the en vironment scooting around the house while avoiding collision with walls and furniture More in teresting was the observation that he became fascinated with h is own reflection in a mirror and deshyspite efforts to distract him he kept turning back to the mirror exploring it intently and smiling The capacity to recognize oneself in the mirror is comshymonly considered to be an indicator of self-a wareness an d is usually explored in animals by the so-called mirror test This hydranencephalic child wa appashyrently as interested in bis r fle tion as any in fant or animal that passes the minor test Although the authors interpreted thi particular behavior as an inshydication of consciousness ther are somegt counteTshy
ynapse
arguments For example Merker argues that Most animals and human infants mirror reactions are social reactions (that is they treat the mirror image as a conspecific and NOT as themselves) so reacting to and being fascinated by a mirror by no means implies self-recognition In the case of [this hydranenshycephalic subject] we cannot even be sure that the behavior was of the nature of social reactions (though the smile raises that possibility) they might simply have been reactions to reflected movements and so on (personal communication)
Another child in this study exhibited discriminative awareness of the environment for example consisshytently distinguishing close family members from others At the approach or touch of strangers she assumed a fearful affect became tense and withdrew but relaxed to the touch and voice of mother The more familiar someone was the more she w(mld relax move spontaneously and vocalize She was aware of her mothers presence and became upset if separated She had favorite pieces and types of music to which she would consistently smile and vocalize in contrast to other music to which she consistently remained indifferent
Another child developed a liking for puppies and small children and her face became animated whenshyever she saw them She also showed some orientation capacities when called she would raise her head look at the person and smile Furthermore when an object she was tracking was suddenly taken behind her she would turn in search of it She was very socially interacting and clearly enjoyed being with people and even played with them The authors observed that in an occasion the subject attempted to imitate monosyllables and even uttered ah-ah when coaxed to say mama She also demonstrated some degree of body awareness For example if her face hurt she would stroke it with her hand The most interesting of her cognitive abilities was her capacity of associative learning The authors observed that she startled and stiffened when a vacuum cleaner or hair dryer with a loud unpleasant noise was turned on Mter several such experiences she also stiffened in anticipation if either object (though switched off) was brought near Moreover she developed a small recepshytive vocabulary including bunny rabbit (a stuffed toy) Michael (a family friend) and Pocahontas (an image on her T-shirt) with coaxing and repetition of the question Where is [one of these] she corshyrectly looked at the object or person
Another subject exhibited some limited form of nonshyverbal communication by making cooing sounds expre sions of sadness or pai n smiling in the presence of caregivers and indicatin g preferences through facial expressions and R broad smile He was able to distingui h h is mother s voice from that of his father
555
MOJ ~
~ CONSCIOUSNESS IN THE ABSENCE OF CEREBRAL CORTEX J t
bull
f)
If j
ibull
I ~
i
~ shy
l I J i-
In a recent article Merker (2007) has described his first-hand observations of the behavior of hydranenceshyphalic children as well as his impressions gleaned from the reports of parents of such children On the basis of his observations Merker argues that these children are not only awake and often alert but show responsiveness to their surroundings in the form of emotional or orienting reactions to environmental events He observed that hydranencephaIic children were able to express pleasure by smiling and laughshyter and aversion by fussing arching of the back and crying Furthermore the children responded differshyentially to the voice of familiars and showed prefershyence for certain situations and stimuli over others such as a particular familiar toy More interestingly some of these children demonstrated the capacity of taking behavioral initiatives in the form of instrushymental behaviors such as making noise by kicking trinkets hanging in a special frame constructed for the purpose or activating favorite toys by switches Merker concludes that hydranencephalic children give proof of being not only awake but of the kind of responsiveness to their surroundings that qualifies as conscious by the criteria of ordinary neurological examination
According to Merker another piece of evidence for the existence of consciousness in hydranencephalic children comes from the fact that these children are subject to the seizures of absence epilepsy Seizures of this type are characterized by lapses in consciousness and a lack of response toward external stimuli In a typical episode of absence seizure the patient sudshydenly becomes unresponsive in the midst of normal activities Ongoing activities may continue in the form of automatisms or they may arrest for the durashytion of the seizure episode At the end of such a seishyzure which may last no more than a few seconds the patient who typically remains upright throughout sometimes actively moving resumes conscious activshyities where they were interrupted has amnesia for what transpired during the episode and may have no knowledge that the episode took place at all Merker argues that episodes of absence in this form of epishylepsy represent a basic affliction of consciousness and concludes the fact that these children exhibit such episodes would seem to be a weighty piece of evidence r egarding their conscious status
Merker s arguments for the presence of consciousshyness in hydranencephalic children have not remained unchallenged Behrendt (2007) believes that signs of pleasure or excitement exhibited by anencephalic chilshydren are not necessarily indicative of conscious expeshyrience and they may be regarded as automatic beshyhavioral reactions activated by appropriate stimuli
In opposition to the notion that hydranencephalic children have consciousness Morin (2007) argues that behaviors exhibited b these patients do not
seem to require reflective consciousness and selfshyawareness However it should be mentioned that Merker used the term consciousness in its most bashysic and general sense and in this context consciousshyness is more than reflective consciousness or selfshyawareness
Watkins and Rees (2007) argue that responsiveness to the environment is a capacity exhibited by almost any organism with a central nervous system and cannot be definitely taken as a sign of consciousness Furthermore the behaviors of hydranencephalic chilshydren seem to lack the element of intentionality and many of the reported behaviors could be generated unconsciously or reflexively
CONCLUSION Whether or not children born with hydranenceshy
phaly have consciousness is still controversial Howshyever the body of evidence in favor of the presence of consciousness in these patients seems to be more conshyvincing than evidence and argu-ments against conshysciousness in such children
Finally it is noteworthy that the outstanding webshysite of the International Hydranencephaly Group (wwwhydranencephalycom) provides comprehensive source of information about this disorder The results of an informal nonscientific survey conducted by this group revealed surprising findings that are contrary to what is generally assumed by most neurologists regarding the presence of consciousness in hydranenshycephalic children When parents of such children were asked is your child aware of hislher surroundshyings 74 of the surveyed parents answered Yes 246 answered No and 148 answered Someshytimes When asked is your child aware of objects
2t074 answered Yes 1728 answered No and 3827 answered Sometimes
The rarity of scientific observations similar to those reported by parents could be partly due to the fact that hydranencephalic children are extremely sensishytive to and easily disturbed by changes in environshyment and routine daily activities In unusual and disshyturbing situations the children often fail to manifest any cognitive functions that parents often report When examined after medical stabilization has taken place and in the setting of the home environment upon which these medically fragile children are crushycially dependent they give proof of being conscious (Merker 2007)
ACKNOWLEDGMENT
I am indebted to Bjorn Merker for providing the pi ture of Niki and receiving penllission from Nikis mother
Synapse I
556 M BESHKAR
REFERENCES Behrendt RP 2007 the hypthalamo--tectoperiaqueductal system
Unconscious underpmnings of conscious behaviour Behav Brain Sci 3085--86
Counter SA 2007 Brainstem mediation of the stapedius muscle reflex in hydranenccphaly Acta Oto-Laryngol 127498-504
Merker B 2007 Consciousness without a cerebral cortex A chalshylenge for neuroscience and medicine Behav Brain Sci 30p3-81
Morin A 2007 Consciousness is more than wakefulness Behav Brain Sci 3099
Shewmon DA Holmes GL Byrne PA 1999 Consciousness in congenitally decorticate children Developmental vegetative state as self-fulfilling prophecy Dev Med Child Neurol 41364shy374
Watkins S Rees G 2007 The human superior colliculus Neither necessary nor sufficient for consciousness Behav Brain Sci 30107
vnnplP
SYNAPSE 62553-556 (2008)
Short Communication
The Presence of Consciousness in the Absence of the Cerebral Cortex
MAJID BESHKARmiddot Tehran University of Medical Sciences Tehran Iran
KEYWORDS hydranencephaly consciousness vegetative state behavior cerebral cortex
ABSTRACT Hydranencephaly is a rare neurological condition in which the cereshybral hemispheres are either absent or severely compromised It is widely believed that children with hydranencephaly are not conscious and therefore are routinely classishyfied into the diagnostic criteria of vegetative state However ther~ are several pieces of behavioral evidence clearly indicating the presence of consciousness in such patients Here I review these behavioral evidence and argue how qlisclassification of these patients and assigning them a lack of consciousness have far-reaching implicashytions in terms of both clinical and theoretical neuroscience Synapse 62553-556 2008 () 2008 Wiley-Liss Inc
INTRODUCTION
Hydranencephaly is a rare neurological condition in which the cerebral hemispheres either fail to develop for genetic-developmental reasons or are massively compromised by trauma of a physical vascular toxic hypoxic-ischemic or infectious nature at some stage of their development (Merker 2007) The disorder which occurs in less than 1 per 10000 births worldshywide is characterized by near-total or total absence of the cerebral cortex and basal ganglia The thalami pons cerebral peduncles and cerebellum are usually present as may be a small amount of occipital-lobe frontal -lobe and temporal-lobe tissue The damaged brain tissues undergo extensive resorption and are replaced by cerebrospinal fluid filling otherwise empty meninges lining a normally shaped skull Hydranenshycephaly must be distinguished from the even more severe neurological condition called anencephaly a disorder of the neural tube beginning very early in developmen t which leads to virtually no brain devel shyopment
The amount of brain ti ssue that each child with hydranencephaly h s varies from child to child Many of the children a re missing most of their brain ti ssues above the brainstem However the loss of cerebral h eroisph res must be massive to be designated hydrashynencephaly although it is seldom complete While variable remnants of th cerebral cortex may be spared these coTtic I remnant are most proba bly not connected to the thalamus (whi te matter loss often
interrupts the visual radiations for instance) and are not even locally functional On autopsy such tissue may be found to be gliotic on microscopic examination or to exhibit other structural anomalies indicating loss of function (Merker 2007)
An infant born with hydranencephaly may initially present no conspicuous symptoms and occasionally the condition is not diagnosed until several months postnatally when developmental milestones are misshysed In the course of the first year of life these infants typically develop a variety of complications that always include motoric ones (tonus spasticity cerebral palsy) Although survival beyond 6 months is rare with hydranencephaly prolonged survival even up to 34 years can occur (Counter 2007)
It is widely believed among neurologists that chilshydren with hydranencephaly are not conscious Howshyever there are some pieces of evidence that these children are able to experience at least some simple levels of consciousness (Fig 1) Scientific literature about this issue is very poor and there are only two published accounts describing observations to the effect that children with hydranencephaly experience conscious states
Corresponde nco to No 2 Sahel 1 Eas l Arghava n Saadal Abrl Tehron I Iun E~ma l l mnjid bcs h klu-yuhfJocom
RIcevec 23 Octuber 2007 Accepl0Ji 20 JflI1LIJrv ~OOtlt
o I 1 n 100Vy 11 20521
tublil)h online 24 Apnl 2O(1B In Wiley 11lugtrampiente hvwwinterscience_wilcshyL 11I lt l
1)2008 WILlW-LlSS INC
554 MBESHKAR
Fig 1 Nikki a hydranencepbalic girl in the arms of her mother She has turned toward someone sitting on her left (you can see the knee to the right in the picture) This picture shows the alertness of the girl and her smile very well For this picture I am indebted to Bjorn Merker for receiving permission from Nikis mother on my behalf
CONSCIOUSNESS IN HYDRANENCEPHALICS Shewmon et a1 (1999) reported the cases of four
children aged 5-17 with hydranencephaly involving complete or nearly complete absence of cerebral corshytex The authors observed that these children posshysessed a variety of cognitive capacities that were inshydicative of ordinary consciousness including person recognition social interaction (smiling when spoken to giggling when played with vocalization with music therapist) functional vision (object discriminashytion fascination with own reflection) musical prefershyences orienting- toward and smiling at someone callshying appropriate affective responses goal-directed motor behavior (scooting on back to reach a goal) and associative learning (using limited receptive vocabushylary to correctly look at an object)~ On the basis of their observations the authors concluded that each of the children they assessed was conscious by the criteshyria of a standard neurological examination
One of thes children was able to visually interact with the en vironment scooting around the house while avoiding collision with walls and furniture More in teresting was the observation that he became fascinated with h is own reflection in a mirror and deshyspite efforts to distract him he kept turning back to the mirror exploring it intently and smiling The capacity to recognize oneself in the mirror is comshymonly considered to be an indicator of self-a wareness an d is usually explored in animals by the so-called mirror test This hydranencephalic child wa appashyrently as interested in bis r fle tion as any in fant or animal that passes the minor test Although the authors interpreted thi particular behavior as an inshydication of consciousness ther are somegt counteTshy
ynapse
arguments For example Merker argues that Most animals and human infants mirror reactions are social reactions (that is they treat the mirror image as a conspecific and NOT as themselves) so reacting to and being fascinated by a mirror by no means implies self-recognition In the case of [this hydranenshycephalic subject] we cannot even be sure that the behavior was of the nature of social reactions (though the smile raises that possibility) they might simply have been reactions to reflected movements and so on (personal communication)
Another child in this study exhibited discriminative awareness of the environment for example consisshytently distinguishing close family members from others At the approach or touch of strangers she assumed a fearful affect became tense and withdrew but relaxed to the touch and voice of mother The more familiar someone was the more she w(mld relax move spontaneously and vocalize She was aware of her mothers presence and became upset if separated She had favorite pieces and types of music to which she would consistently smile and vocalize in contrast to other music to which she consistently remained indifferent
Another child developed a liking for puppies and small children and her face became animated whenshyever she saw them She also showed some orientation capacities when called she would raise her head look at the person and smile Furthermore when an object she was tracking was suddenly taken behind her she would turn in search of it She was very socially interacting and clearly enjoyed being with people and even played with them The authors observed that in an occasion the subject attempted to imitate monosyllables and even uttered ah-ah when coaxed to say mama She also demonstrated some degree of body awareness For example if her face hurt she would stroke it with her hand The most interesting of her cognitive abilities was her capacity of associative learning The authors observed that she startled and stiffened when a vacuum cleaner or hair dryer with a loud unpleasant noise was turned on Mter several such experiences she also stiffened in anticipation if either object (though switched off) was brought near Moreover she developed a small recepshytive vocabulary including bunny rabbit (a stuffed toy) Michael (a family friend) and Pocahontas (an image on her T-shirt) with coaxing and repetition of the question Where is [one of these] she corshyrectly looked at the object or person
Another subject exhibited some limited form of nonshyverbal communication by making cooing sounds expre sions of sadness or pai n smiling in the presence of caregivers and indicatin g preferences through facial expressions and R broad smile He was able to distingui h h is mother s voice from that of his father
555
MOJ ~
~ CONSCIOUSNESS IN THE ABSENCE OF CEREBRAL CORTEX J t
bull
f)
If j
ibull
I ~
i
~ shy
l I J i-
In a recent article Merker (2007) has described his first-hand observations of the behavior of hydranenceshyphalic children as well as his impressions gleaned from the reports of parents of such children On the basis of his observations Merker argues that these children are not only awake and often alert but show responsiveness to their surroundings in the form of emotional or orienting reactions to environmental events He observed that hydranencephaIic children were able to express pleasure by smiling and laughshyter and aversion by fussing arching of the back and crying Furthermore the children responded differshyentially to the voice of familiars and showed prefershyence for certain situations and stimuli over others such as a particular familiar toy More interestingly some of these children demonstrated the capacity of taking behavioral initiatives in the form of instrushymental behaviors such as making noise by kicking trinkets hanging in a special frame constructed for the purpose or activating favorite toys by switches Merker concludes that hydranencephalic children give proof of being not only awake but of the kind of responsiveness to their surroundings that qualifies as conscious by the criteria of ordinary neurological examination
According to Merker another piece of evidence for the existence of consciousness in hydranencephalic children comes from the fact that these children are subject to the seizures of absence epilepsy Seizures of this type are characterized by lapses in consciousness and a lack of response toward external stimuli In a typical episode of absence seizure the patient sudshydenly becomes unresponsive in the midst of normal activities Ongoing activities may continue in the form of automatisms or they may arrest for the durashytion of the seizure episode At the end of such a seishyzure which may last no more than a few seconds the patient who typically remains upright throughout sometimes actively moving resumes conscious activshyities where they were interrupted has amnesia for what transpired during the episode and may have no knowledge that the episode took place at all Merker argues that episodes of absence in this form of epishylepsy represent a basic affliction of consciousness and concludes the fact that these children exhibit such episodes would seem to be a weighty piece of evidence r egarding their conscious status
Merker s arguments for the presence of consciousshyness in hydranencephalic children have not remained unchallenged Behrendt (2007) believes that signs of pleasure or excitement exhibited by anencephalic chilshydren are not necessarily indicative of conscious expeshyrience and they may be regarded as automatic beshyhavioral reactions activated by appropriate stimuli
In opposition to the notion that hydranencephalic children have consciousness Morin (2007) argues that behaviors exhibited b these patients do not
seem to require reflective consciousness and selfshyawareness However it should be mentioned that Merker used the term consciousness in its most bashysic and general sense and in this context consciousshyness is more than reflective consciousness or selfshyawareness
Watkins and Rees (2007) argue that responsiveness to the environment is a capacity exhibited by almost any organism with a central nervous system and cannot be definitely taken as a sign of consciousness Furthermore the behaviors of hydranencephalic chilshydren seem to lack the element of intentionality and many of the reported behaviors could be generated unconsciously or reflexively
CONCLUSION Whether or not children born with hydranenceshy
phaly have consciousness is still controversial Howshyever the body of evidence in favor of the presence of consciousness in these patients seems to be more conshyvincing than evidence and argu-ments against conshysciousness in such children
Finally it is noteworthy that the outstanding webshysite of the International Hydranencephaly Group (wwwhydranencephalycom) provides comprehensive source of information about this disorder The results of an informal nonscientific survey conducted by this group revealed surprising findings that are contrary to what is generally assumed by most neurologists regarding the presence of consciousness in hydranenshycephalic children When parents of such children were asked is your child aware of hislher surroundshyings 74 of the surveyed parents answered Yes 246 answered No and 148 answered Someshytimes When asked is your child aware of objects
2t074 answered Yes 1728 answered No and 3827 answered Sometimes
The rarity of scientific observations similar to those reported by parents could be partly due to the fact that hydranencephalic children are extremely sensishytive to and easily disturbed by changes in environshyment and routine daily activities In unusual and disshyturbing situations the children often fail to manifest any cognitive functions that parents often report When examined after medical stabilization has taken place and in the setting of the home environment upon which these medically fragile children are crushycially dependent they give proof of being conscious (Merker 2007)
ACKNOWLEDGMENT
I am indebted to Bjorn Merker for providing the pi ture of Niki and receiving penllission from Nikis mother
Synapse I
556 M BESHKAR
REFERENCES Behrendt RP 2007 the hypthalamo--tectoperiaqueductal system
Unconscious underpmnings of conscious behaviour Behav Brain Sci 3085--86
Counter SA 2007 Brainstem mediation of the stapedius muscle reflex in hydranenccphaly Acta Oto-Laryngol 127498-504
Merker B 2007 Consciousness without a cerebral cortex A chalshylenge for neuroscience and medicine Behav Brain Sci 30p3-81
Morin A 2007 Consciousness is more than wakefulness Behav Brain Sci 3099
Shewmon DA Holmes GL Byrne PA 1999 Consciousness in congenitally decorticate children Developmental vegetative state as self-fulfilling prophecy Dev Med Child Neurol 41364shy374
Watkins S Rees G 2007 The human superior colliculus Neither necessary nor sufficient for consciousness Behav Brain Sci 30107
vnnplP
554 MBESHKAR
Fig 1 Nikki a hydranencepbalic girl in the arms of her mother She has turned toward someone sitting on her left (you can see the knee to the right in the picture) This picture shows the alertness of the girl and her smile very well For this picture I am indebted to Bjorn Merker for receiving permission from Nikis mother on my behalf
CONSCIOUSNESS IN HYDRANENCEPHALICS Shewmon et a1 (1999) reported the cases of four
children aged 5-17 with hydranencephaly involving complete or nearly complete absence of cerebral corshytex The authors observed that these children posshysessed a variety of cognitive capacities that were inshydicative of ordinary consciousness including person recognition social interaction (smiling when spoken to giggling when played with vocalization with music therapist) functional vision (object discriminashytion fascination with own reflection) musical prefershyences orienting- toward and smiling at someone callshying appropriate affective responses goal-directed motor behavior (scooting on back to reach a goal) and associative learning (using limited receptive vocabushylary to correctly look at an object)~ On the basis of their observations the authors concluded that each of the children they assessed was conscious by the criteshyria of a standard neurological examination
One of thes children was able to visually interact with the en vironment scooting around the house while avoiding collision with walls and furniture More in teresting was the observation that he became fascinated with h is own reflection in a mirror and deshyspite efforts to distract him he kept turning back to the mirror exploring it intently and smiling The capacity to recognize oneself in the mirror is comshymonly considered to be an indicator of self-a wareness an d is usually explored in animals by the so-called mirror test This hydranencephalic child wa appashyrently as interested in bis r fle tion as any in fant or animal that passes the minor test Although the authors interpreted thi particular behavior as an inshydication of consciousness ther are somegt counteTshy
ynapse
arguments For example Merker argues that Most animals and human infants mirror reactions are social reactions (that is they treat the mirror image as a conspecific and NOT as themselves) so reacting to and being fascinated by a mirror by no means implies self-recognition In the case of [this hydranenshycephalic subject] we cannot even be sure that the behavior was of the nature of social reactions (though the smile raises that possibility) they might simply have been reactions to reflected movements and so on (personal communication)
Another child in this study exhibited discriminative awareness of the environment for example consisshytently distinguishing close family members from others At the approach or touch of strangers she assumed a fearful affect became tense and withdrew but relaxed to the touch and voice of mother The more familiar someone was the more she w(mld relax move spontaneously and vocalize She was aware of her mothers presence and became upset if separated She had favorite pieces and types of music to which she would consistently smile and vocalize in contrast to other music to which she consistently remained indifferent
Another child developed a liking for puppies and small children and her face became animated whenshyever she saw them She also showed some orientation capacities when called she would raise her head look at the person and smile Furthermore when an object she was tracking was suddenly taken behind her she would turn in search of it She was very socially interacting and clearly enjoyed being with people and even played with them The authors observed that in an occasion the subject attempted to imitate monosyllables and even uttered ah-ah when coaxed to say mama She also demonstrated some degree of body awareness For example if her face hurt she would stroke it with her hand The most interesting of her cognitive abilities was her capacity of associative learning The authors observed that she startled and stiffened when a vacuum cleaner or hair dryer with a loud unpleasant noise was turned on Mter several such experiences she also stiffened in anticipation if either object (though switched off) was brought near Moreover she developed a small recepshytive vocabulary including bunny rabbit (a stuffed toy) Michael (a family friend) and Pocahontas (an image on her T-shirt) with coaxing and repetition of the question Where is [one of these] she corshyrectly looked at the object or person
Another subject exhibited some limited form of nonshyverbal communication by making cooing sounds expre sions of sadness or pai n smiling in the presence of caregivers and indicatin g preferences through facial expressions and R broad smile He was able to distingui h h is mother s voice from that of his father
555
MOJ ~
~ CONSCIOUSNESS IN THE ABSENCE OF CEREBRAL CORTEX J t
bull
f)
If j
ibull
I ~
i
~ shy
l I J i-
In a recent article Merker (2007) has described his first-hand observations of the behavior of hydranenceshyphalic children as well as his impressions gleaned from the reports of parents of such children On the basis of his observations Merker argues that these children are not only awake and often alert but show responsiveness to their surroundings in the form of emotional or orienting reactions to environmental events He observed that hydranencephaIic children were able to express pleasure by smiling and laughshyter and aversion by fussing arching of the back and crying Furthermore the children responded differshyentially to the voice of familiars and showed prefershyence for certain situations and stimuli over others such as a particular familiar toy More interestingly some of these children demonstrated the capacity of taking behavioral initiatives in the form of instrushymental behaviors such as making noise by kicking trinkets hanging in a special frame constructed for the purpose or activating favorite toys by switches Merker concludes that hydranencephalic children give proof of being not only awake but of the kind of responsiveness to their surroundings that qualifies as conscious by the criteria of ordinary neurological examination
According to Merker another piece of evidence for the existence of consciousness in hydranencephalic children comes from the fact that these children are subject to the seizures of absence epilepsy Seizures of this type are characterized by lapses in consciousness and a lack of response toward external stimuli In a typical episode of absence seizure the patient sudshydenly becomes unresponsive in the midst of normal activities Ongoing activities may continue in the form of automatisms or they may arrest for the durashytion of the seizure episode At the end of such a seishyzure which may last no more than a few seconds the patient who typically remains upright throughout sometimes actively moving resumes conscious activshyities where they were interrupted has amnesia for what transpired during the episode and may have no knowledge that the episode took place at all Merker argues that episodes of absence in this form of epishylepsy represent a basic affliction of consciousness and concludes the fact that these children exhibit such episodes would seem to be a weighty piece of evidence r egarding their conscious status
Merker s arguments for the presence of consciousshyness in hydranencephalic children have not remained unchallenged Behrendt (2007) believes that signs of pleasure or excitement exhibited by anencephalic chilshydren are not necessarily indicative of conscious expeshyrience and they may be regarded as automatic beshyhavioral reactions activated by appropriate stimuli
In opposition to the notion that hydranencephalic children have consciousness Morin (2007) argues that behaviors exhibited b these patients do not
seem to require reflective consciousness and selfshyawareness However it should be mentioned that Merker used the term consciousness in its most bashysic and general sense and in this context consciousshyness is more than reflective consciousness or selfshyawareness
Watkins and Rees (2007) argue that responsiveness to the environment is a capacity exhibited by almost any organism with a central nervous system and cannot be definitely taken as a sign of consciousness Furthermore the behaviors of hydranencephalic chilshydren seem to lack the element of intentionality and many of the reported behaviors could be generated unconsciously or reflexively
CONCLUSION Whether or not children born with hydranenceshy
phaly have consciousness is still controversial Howshyever the body of evidence in favor of the presence of consciousness in these patients seems to be more conshyvincing than evidence and argu-ments against conshysciousness in such children
Finally it is noteworthy that the outstanding webshysite of the International Hydranencephaly Group (wwwhydranencephalycom) provides comprehensive source of information about this disorder The results of an informal nonscientific survey conducted by this group revealed surprising findings that are contrary to what is generally assumed by most neurologists regarding the presence of consciousness in hydranenshycephalic children When parents of such children were asked is your child aware of hislher surroundshyings 74 of the surveyed parents answered Yes 246 answered No and 148 answered Someshytimes When asked is your child aware of objects
2t074 answered Yes 1728 answered No and 3827 answered Sometimes
The rarity of scientific observations similar to those reported by parents could be partly due to the fact that hydranencephalic children are extremely sensishytive to and easily disturbed by changes in environshyment and routine daily activities In unusual and disshyturbing situations the children often fail to manifest any cognitive functions that parents often report When examined after medical stabilization has taken place and in the setting of the home environment upon which these medically fragile children are crushycially dependent they give proof of being conscious (Merker 2007)
ACKNOWLEDGMENT
I am indebted to Bjorn Merker for providing the pi ture of Niki and receiving penllission from Nikis mother
Synapse I
556 M BESHKAR
REFERENCES Behrendt RP 2007 the hypthalamo--tectoperiaqueductal system
Unconscious underpmnings of conscious behaviour Behav Brain Sci 3085--86
Counter SA 2007 Brainstem mediation of the stapedius muscle reflex in hydranenccphaly Acta Oto-Laryngol 127498-504
Merker B 2007 Consciousness without a cerebral cortex A chalshylenge for neuroscience and medicine Behav Brain Sci 30p3-81
Morin A 2007 Consciousness is more than wakefulness Behav Brain Sci 3099
Shewmon DA Holmes GL Byrne PA 1999 Consciousness in congenitally decorticate children Developmental vegetative state as self-fulfilling prophecy Dev Med Child Neurol 41364shy374
Watkins S Rees G 2007 The human superior colliculus Neither necessary nor sufficient for consciousness Behav Brain Sci 30107
vnnplP
555
MOJ ~
~ CONSCIOUSNESS IN THE ABSENCE OF CEREBRAL CORTEX J t
bull
f)
If j
ibull
I ~
i
~ shy
l I J i-
In a recent article Merker (2007) has described his first-hand observations of the behavior of hydranenceshyphalic children as well as his impressions gleaned from the reports of parents of such children On the basis of his observations Merker argues that these children are not only awake and often alert but show responsiveness to their surroundings in the form of emotional or orienting reactions to environmental events He observed that hydranencephaIic children were able to express pleasure by smiling and laughshyter and aversion by fussing arching of the back and crying Furthermore the children responded differshyentially to the voice of familiars and showed prefershyence for certain situations and stimuli over others such as a particular familiar toy More interestingly some of these children demonstrated the capacity of taking behavioral initiatives in the form of instrushymental behaviors such as making noise by kicking trinkets hanging in a special frame constructed for the purpose or activating favorite toys by switches Merker concludes that hydranencephalic children give proof of being not only awake but of the kind of responsiveness to their surroundings that qualifies as conscious by the criteria of ordinary neurological examination
According to Merker another piece of evidence for the existence of consciousness in hydranencephalic children comes from the fact that these children are subject to the seizures of absence epilepsy Seizures of this type are characterized by lapses in consciousness and a lack of response toward external stimuli In a typical episode of absence seizure the patient sudshydenly becomes unresponsive in the midst of normal activities Ongoing activities may continue in the form of automatisms or they may arrest for the durashytion of the seizure episode At the end of such a seishyzure which may last no more than a few seconds the patient who typically remains upright throughout sometimes actively moving resumes conscious activshyities where they were interrupted has amnesia for what transpired during the episode and may have no knowledge that the episode took place at all Merker argues that episodes of absence in this form of epishylepsy represent a basic affliction of consciousness and concludes the fact that these children exhibit such episodes would seem to be a weighty piece of evidence r egarding their conscious status
Merker s arguments for the presence of consciousshyness in hydranencephalic children have not remained unchallenged Behrendt (2007) believes that signs of pleasure or excitement exhibited by anencephalic chilshydren are not necessarily indicative of conscious expeshyrience and they may be regarded as automatic beshyhavioral reactions activated by appropriate stimuli
In opposition to the notion that hydranencephalic children have consciousness Morin (2007) argues that behaviors exhibited b these patients do not
seem to require reflective consciousness and selfshyawareness However it should be mentioned that Merker used the term consciousness in its most bashysic and general sense and in this context consciousshyness is more than reflective consciousness or selfshyawareness
Watkins and Rees (2007) argue that responsiveness to the environment is a capacity exhibited by almost any organism with a central nervous system and cannot be definitely taken as a sign of consciousness Furthermore the behaviors of hydranencephalic chilshydren seem to lack the element of intentionality and many of the reported behaviors could be generated unconsciously or reflexively
CONCLUSION Whether or not children born with hydranenceshy
phaly have consciousness is still controversial Howshyever the body of evidence in favor of the presence of consciousness in these patients seems to be more conshyvincing than evidence and argu-ments against conshysciousness in such children
Finally it is noteworthy that the outstanding webshysite of the International Hydranencephaly Group (wwwhydranencephalycom) provides comprehensive source of information about this disorder The results of an informal nonscientific survey conducted by this group revealed surprising findings that are contrary to what is generally assumed by most neurologists regarding the presence of consciousness in hydranenshycephalic children When parents of such children were asked is your child aware of hislher surroundshyings 74 of the surveyed parents answered Yes 246 answered No and 148 answered Someshytimes When asked is your child aware of objects
2t074 answered Yes 1728 answered No and 3827 answered Sometimes
The rarity of scientific observations similar to those reported by parents could be partly due to the fact that hydranencephalic children are extremely sensishytive to and easily disturbed by changes in environshyment and routine daily activities In unusual and disshyturbing situations the children often fail to manifest any cognitive functions that parents often report When examined after medical stabilization has taken place and in the setting of the home environment upon which these medically fragile children are crushycially dependent they give proof of being conscious (Merker 2007)
ACKNOWLEDGMENT
I am indebted to Bjorn Merker for providing the pi ture of Niki and receiving penllission from Nikis mother
Synapse I
556 M BESHKAR
REFERENCES Behrendt RP 2007 the hypthalamo--tectoperiaqueductal system
Unconscious underpmnings of conscious behaviour Behav Brain Sci 3085--86
Counter SA 2007 Brainstem mediation of the stapedius muscle reflex in hydranenccphaly Acta Oto-Laryngol 127498-504
Merker B 2007 Consciousness without a cerebral cortex A chalshylenge for neuroscience and medicine Behav Brain Sci 30p3-81
Morin A 2007 Consciousness is more than wakefulness Behav Brain Sci 3099
Shewmon DA Holmes GL Byrne PA 1999 Consciousness in congenitally decorticate children Developmental vegetative state as self-fulfilling prophecy Dev Med Child Neurol 41364shy374
Watkins S Rees G 2007 The human superior colliculus Neither necessary nor sufficient for consciousness Behav Brain Sci 30107
vnnplP
556 M BESHKAR
REFERENCES Behrendt RP 2007 the hypthalamo--tectoperiaqueductal system
Unconscious underpmnings of conscious behaviour Behav Brain Sci 3085--86
Counter SA 2007 Brainstem mediation of the stapedius muscle reflex in hydranenccphaly Acta Oto-Laryngol 127498-504
Merker B 2007 Consciousness without a cerebral cortex A chalshylenge for neuroscience and medicine Behav Brain Sci 30p3-81
Morin A 2007 Consciousness is more than wakefulness Behav Brain Sci 3099
Shewmon DA Holmes GL Byrne PA 1999 Consciousness in congenitally decorticate children Developmental vegetative state as self-fulfilling prophecy Dev Med Child Neurol 41364shy374
Watkins S Rees G 2007 The human superior colliculus Neither necessary nor sufficient for consciousness Behav Brain Sci 30107
vnnplP