This article discusses the presence of consciousness in children born with hydranencephaly, a rare condition where the cerebral hemispheres are absent or severely compromised. While it is widely believed that these children lack consciousness, the article reviews evidence that some hydranencephalic children exhibit behaviors indicating basic levels of consciousness, such as recognizing faces, responding preferentially to familiar people/stimuli, and learning associations. However, whether they experience reflective consciousness remains controversial. The article concludes the evidence for some level of consciousness in these children is more convincing than arguments against it.

1. 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 post­
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 post­
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 fetal.anesthesia and its effect? At what post­
fert ilizati on age?
b. Page :
9. Cortex:
a. Does t he articl e relate t o the asserte d need f or cort ical involvement to
exp eri ence pain? How?

2. 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."

3. SYNAPSE 62:553-556 (2008)
Short Communication
The Presence of Consciousness in the
Absence of the Cerebral Cortex
MAJID BESHKAR·
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 cere­
bral hemispheres are either absent or severely compromised. It is widely believed that
children with hydranencephaly are not conscious; and therefore, are routinely classi­
fied 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 implica­
tions in terms of both clinical and theoretical neuroscience. Synapse 62:553-556,
2008. () 2008 Wiley-Liss. Inc.
INTRODUCTION interrupts the visual radiations! for instance), and are
Hydranencephaly is a rare neurological condition in not even locally functional. On autopsy, such tissue
which the cerebral hemispheres either fail to develop may be found to be gliotic on microscopic examination
for genetic-developmental reasons or are massively or to exhibit other structural anomalies indicating
compromised by trauma of a physical, vascular, toxic, loss of function (Merker, 2007).
hypoxic-ischemic, or infectious nature at some stage An infant born with hydranencephaly may initially
of their development (Merker, 2007). The disorder, present no conspicuous symptoms and occasionally
which occurs in less than 1 per 10,000 births world­ the condition is not diagnosed until several months
wide, is characterized by near-total or total absence of postnatally, when developmental milestones are mis­
the cerebral cortex and basal ganglia. The thalami , sed. In the course of the first year of life, these
pons, cerebral peduncles, and cerebellum are usually infants typically develop a variety of complications
present, as may be a small amount of occipital-lobe, that always include motoric ones (tonus, spasticity,
frontal -lobe, and tempor al-lobe tissue. The damaged cerebral palsy). Although survival beyond 6 months is
brain tissues undergo extensive resorption , and are rare with hydranencephaly, prolonged survival even
replaced by cerebrospinal fluid filling otherwise empty up to 34 years can occur (Counter, 2007).
meninges lining a normally shaped skull. Hydranen­ It is widely believed among neurologists that chil­
cephaly must be distinguished from the even more dren with hydranencephaly are not conscious. How­
severe neurological condition called anencephaly, a ever, there are some pieces of evidence that these
disorder of the neural tube beginning very early in children are able to experience at least some simple
developmen t, which leads to virtually no brain devel ­ levels of consciousness (Fig. 1). Scientific literature
opment. about this issue is very poor, and there are only two
The amount of brain ti ssue that each child with published accounts describing observations to the
hydranen cephaly h s varies from child to child. Ma ny effect that children with hydranencephaly experience
of the children a re missing most of their brain ti ssues conscious states .
above the brainstem. However, the loss of cerebral
h eroisph res mu st be massive to be designated h ydra­ Corresponde nco to: No. 2, Sahe'l 1. Eas l Arghava n. Saadal Ab"rl , Tehron,
I I'un . E~ ma l l: mnjid .bcs h klu-@yu hfJo.com
nencephaly, although it. is seldom compl ete. While
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spared, these coTtic I remnant are most proba bly n ot t'u blil)h online 24 Apnl 2O(1B In Wiley 11lu;>r&ient'e hvwwinterscience_wilc;.­
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4. 554 M.BESHKAR
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 hydranen­
cephalic 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, consis­
tently distinguishing close family members from
others. At the approach or touch of strangers she
assumed a fearful affect, became tense and withdrew,
Fig. 1. Nikki, a hydranencepbalic girl, in the arms of her but relaxed to the touch and voice of mother. The
mother. She has turned toward someone sitting on her left (you can more familiar someone was, the more she w(mld
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 relax, move spontaneously, and vocalize. She was
indebted to Bjorn Merker for receiving permission from Niki's aware of her mother's presence and became upset if
mother on my behalf. 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
CONSCIOUSNESS IN HYDRANENCEPHALICS remained indifferent.
Shewmon et a1. (1999) reported the cases of four Another child developed a liking for puppies and
children aged 5-17, with hydranencephaly involving small children; and her face became animated when­
complete or nearly complete absence of cerebral cor­ ever she saw them. She also showed some orientation
tex. The authors observed that these children pos­ capacities: when called, she would raise her head,
sessed a variety of cognitive capacities that were in­ look at the person, and smile. Furthermore, when an
dicative of ordinary consciousness, including person object she was tracking was suddenly taken behind
recognition, social interaction (smiling when spoken her, she would turn in search of it. She was very
to, giggling when played with, vocalization with socially interacting and clearly enjoyed being with
music therapist), functional vision (object discrimina­ people and even played with them. The authors
tion, fascination with own reflection), musical prefer­ observed that in an occasion the subject attempted to
ences, orienting- toward and smiling at someone call­ imitate monosyllables and even uttered "ah-ah" when
ing, appropriate affective responses, goal-directed coaxed to say "mama." She also demonstrated some
motor behavior (scooting on back to reach a goal), and degree of body awareness. For example, if her face
associative learning (using limited receptive vocabu­ hurt, she would stroke it with her hand. The most
lary to correctly look at an object)~ On the basis of interesting of her cognitive abilities was her capacity
their observations, the authors concluded that each of of associative learning. The authors observed that she
the children they assessed was conscious by the crite­ startled and stiffened when a vacuum cleaner or hair
ria of a standard neurological examin ation . dryer with a loud unpleasant noise was turned on.
One of thes children was able to visually interact Mter several such experiences, she also stiffened in
with the en vironment, scooting around the house anticipation if either object (though switched off) was
while avoiding collision with walls and furniture . brought near. Moreover, she developed a small recep­
More in teresting was the observation that he became tive vocabulary, including "bunny rabbit" (a stuffed
fascinate d with h is own reflection in a mirror; and de­ toy), "Michael" (a family friend), and "Pocahontas"
spite efforts to distract him, he kept turning back to (an image on her T-shirt); with coaxing and repetition
the mirror, ex ploring it intently, and smiling. The of the question , "Where is [one of these]?" she cor­
capacity to recognize oneself in the mirror is com­ rectly looked at the object or person.
monly considered to be an indicator of self- a wareness, Another subject exhibited some limited form of non­
an d is usually explored in animals by the so-called verbal communication by making cooing sounds,
mirror test. This hydranencepha lic chil d wa appa­ expre sions of sadness or pai n , smiling in the
rently as interested in bis r fle tion as any in fant or presence of caregivers , an d indicatin g preferences
animal that passes the m inor test. Although the through facial ex pression s a nd R broad smile. He was
authors interpreted thi' particul ar behavior as an in­ able to distingui h h is moth er 's voice from that of his
dication of consciousness, ther are some> counteT­ father
'ynapse

5. MO'
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,~ CONSCIOUSNESS IN THE ABSENCE OF CEREBRAL CORTEX
In a recent article, Merker (2007) has described his seem to require reflective consciousness and self­
555
! first-hand observations of the behavior of hydranence­ awareness. However, it should be mentioned that
j phalic children, as well as his impressions gleaned Merker used the term "consciousness" in its most ba­
i• from the reports of parents of such children. On the sic and general sense; and, in this context conscious­
I
basis of his observations, Merker argues that "these ness is more than reflective consciousness or self­
children are not only awake and often alert, but show awareness.
responsiveness to their surroundings in the ' form of Watkins and Rees (2007) argue that responsiveness
~ ',
emotional , or orienting reactions to environmental to the environment is a capacity exhibited by almost
, events." He observed that hydranencephaIic children any organism with a central nervous system, and
i~
­
were able to "express pleasure by smiling and laugh­
ter, and aversion by 'fussing,' arching of the back and
cannot be definitely taken as a sign of consciousness.
Furthermore, the behaviors of hydranencephalic chil­
:! crying." Furthermore, the children responded differ­ dren seem to lack the element of intentionality and
entially to the voice of familiars, and showed prefer­ "many of the reported behaviors could be generated
ence for certain situations and stimuli over others, unconsciously or reflexively."
l :' such as a particular familiar toy. More interestingly,
: I some of these children demonstrated the capacity of
J, :i-
taking behavioral initiatives in the form of instru­ CONCLUSION
mental behaviors, such as making noise by kicking
,Whether or not children born with hydranence­
trinkets hanging in a special frame constructed for
phaly have consciousness is still controversial. How­
the purpose, or activating favorite toys by switches.
ever, the body of evidence in favor of the presence of
Merker concludes that hydranencephalic children
consciousness in these patients seems to be more con­
"give proof of being not only awake, but of the kind of
vincing than evidence and argu-ments against con­
responsiveness to their surroundings that qualifies
sciousness in such children.
as conscious by the criteria of ordinary neurological
examination." Finally, it is noteworthy that the outstanding web­
site of the International Hydranencephaly Group
According to Merker, another piece of evidence for
(www.hydranencephaly.com) provides comprehensive
the existence of consciousness in hydranencephalic
source of information about this disorder. The results
children comes from the fact that these children are
of an informal, nonscientific survey conducted by this
subject to the seizures of absence epilepsy. Seizures of
group revealed surprising findings that are contrary
this type are characterized by lapses in consciousness
to what is generally assumed by most neurologists
and a lack of response toward external stimuli. In a
regarding the presence of consciousness in hydranen­
typical episode of absence seizure, the patient sud­
cephalic children. When parents of such children
denly becomes unresponsive in the midst of normal
were asked "is your child aware of hislher surround­
activities. Ongoing activities may continue in the
ings?," 74% of the surveyed parents answered "Yes,"
form of automatisms or they may arrest for the dura­
2.46% answered "No," and 14.8% answered "Some­
tion of the seizure episode. At the end of such a sei­
times." When asked "is your child aware of objects?"
zure, which may last no more than a few seconds, the
"2t0.74% answered "Yes," 17.28% answered "No," and
patient, who typically remains upright throughout,
38.27% answered "Sometimes."
sometimes actively moving, resumes conscious activ­
The rarity of scientific observations similar to those
ities where they were interrupted, has amnesia for ,
reported by parents could be partly due to the fact
what transpired during the episode, and may have no
that hydranencephalic children are extremely sensi­
knowledge that the episode took place at all. Merker
tive to and easily disturbed by changes in environ­
argues that "episodes of absence in this form of epi­
ment and routine daily activities. In unusual and dis­
lepsy represent a basic affliction of consciousness,"
turbing situations, the children often fail to manifest
and concludes "the fact that these children exhibit
any cognitive functions that parents often report.
such episodes would seem to be a weighty piece of
When examined after medical stabilization has taken
evidence r egarding their conscious status."
place, and in the setting of the home environment
•
Merker 's arguments for the presence of conscious­
ness in hydranencephalic children have not remained
upon which these medically fragile children are cru­
cially dependent, th ey give proof of being conscious
unchallenged. Behrendt (2007) believes that "signs of
(Merker, 2007 ).
pleasure or excitement exhibited by anencephalic chil­
dren are not necessarily in dicative of conscious expe­
rience" and they m ay be regarded as automatic be­
havioral reactions activated by appropriate stim uli. ACKNOWLEDG MENT
In opposition to the notion that hydranencephalic I am indebted to Bjorn Merker for provid ing the
children have consciousness, Morin (2007) argues pi ture of Niki an d receiving penllission from Niki's
that behaviors exhibited b these patients do not mother.
f,) Synapse'
I

6. 556 M . BESHKAR
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Counter SA. 2007. Brainstem mediation of the stapedius muscle 374.
reflex in hydranenccphaly. Acta Oto-Laryngol 127:498-504. Watkins S, Rees G. 2007. The human superior colliculus: Neither
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