The following article was written by Dr Shewmon and
published in Developmental Medicine and Child Neurology in June 1999.
This article has brought hope to many of our families. It is printed
here with the permission of Dr. Shewmon and the publishers of
Developmental Medicine and Child Neurology.
Note: This article is
quite long and technical. The reason for the technical language is that
Dr Shewmon wrote this as a Neurologist for other Neurologists. He feels
that is the way to affect change in the thinking of neurologists. If you
don’t want to read all the technical stuff, read from “Why are these
Cases so Rare” (p. 101) to the end.
Consciousness in Congenitally Decorticate Children:
"Developmental Vegetative State"
as Self-Fulfilling Prophecy
D. Alan Shewmon, MD
Gregory L. Holmes, MD
Paul A. Byrne, MD FAAP
in press, Developmental Medicine and Child Neurology
published June 1999
Address correspondence to Dr. Shewmon
UCLA Medical Center
P.O. Box 95 1752
Los Angeles. CA 90095-1752
According to traditional neurophysiologic theory. consciousness
requires. neocortical functioning. and children born without cerebral
hemispheres necessarily remain indefinitely in a developmental
vegetative state. We report four children, ages 5 to 17 years. with
congenital brain malformations involving total or nearly total absence
of cerebral cortex, but who nonetheless possessed discriminative
awareness. eg; distinguishing familiar from unfamiliar people and
environments, social interaction. functional vision orienting. musical
preference,, appropriate affective· responses. and
associative learning. These abilities may reflect "vertical". plasticity
of brain-stem and diencephalic structures. The relative rarity of
manifest consciousness in congenitally decorticate children could be due
largely to an inherent tendency of the label "developmental vegetative
state" to become a self-fulfilling prophecy
keywords: Congenital decorticate state; consciousness;
developmental disability; hydranencephaly,: vegetative state
abbreviations AAN = American Academy of Neurology: CT = computed
tomography: EEG=electroencephalogram. MRI=magnetic resonance imagine:
PVS = persistent vegetative state
Content of consciousness is widely held to be mediated by the
cerebral cortex, with subcortical structures serving merely an arousal
function (Cranford 1988, Plum and Posner 1983). As the
American Academy of Neurology (AAN) put it (1989):
"Neurologically, being awake but unaware is the result of a
functioning brainstem and the total loss of cerebral cortical
functioning... Pain and suffering are attributes of
consciousness requiring cerebral cortical functioning.."
Equivalent statements have been issued by multiple professional
groups (American Academy of Neurology 1995, American Medical Association
1990. ANA Committee 1993, Dyer 1992, Multi society Task Force 1994a.
For congenital cases, the Medical Task Force on Anencephaly
(l990) similarly opined: "Infants with anencephaly, lacking
functioning cerebral cortex are permanently unconscious (p. 671)....
the suffering associated with noxious stimuli (pain) is a cerebral
interpretation of the stimuli; therefore, infants with
anencephaly presumably cannot suffer (p. 672)"
According to the Multi-Society Task Force on persistent Vegetative
State (PVS) (1994a. 1994b). any form of congenital decortication will
equally yield a "developmental vegetative state" It takes but one
counterexample to disprove a universal rule, We here present four.
The following cases came to the authors' attention in a variety of
ways. With full permission medical records were reviewed, and one
or more authors visited the homes to examine the child and videotape
interactive behaviors in the most familiar environment
Case I (VA)
VA was born full term following a pregnancy complicated by
urinary tract infection. Hydranencephaly had been
diagnosed prenatally by ultrasound. and parents relinquished the
baby for adoption. His examination was unremarkable except for nystagmus
Hydranencephaly was confirmed by computed tomography (CT). which
showed absence of cerebral tissue rostral to the thalamus, save for
small mesial temporal lobe remnants. A thin crescent of tissue extended
from the left middle fossa along the posterolateral aspect of a large
midline cyst with fluid of lower density than the main ruprafentorial
fluid (Fig. I) EEGs showed no electrocerebral activity over the entire
head except for some 50-60 pV theta plus low-amplitude beta in the left
parietal region. corresponding to !he tissue
on CT scan; some tracings also revealed epileptiform discharges in the
VA was discharged to a foster family, who were told he
would remain vegetative and almost certainly require
institutionalization. Over the next two years he remained
severely irritable and was treated with sedatives. By 6 months he had
developed marked diffuse spasticity and prominent obligate tonic
neck and grasp responses.
Hydrocephalus required placement of bilateral ventriculo-peritoneal
shunts at age 4 months. The neurosurgeon observed no brain tissue at all
under the meninges on either side. Increasing difficulty with swallowing
led to placement of a feeding gastrostomy.
At 6 months VA was adopted by a nurse who had especially
bonded with him and subsequently dedicated herself full-time to caring
for three hydranencephalic children From then on he received constant
stimulation and attention from both her and early-intervention
therapists His neurologist from age 6 months to 10 years was author GLH.
When authors DAS and PAB visited VA at home. he was 6 years
old He was small for age and microcephalic. extremely spastic with
sustained clonus everywhere and moderate flexion contractures in all
four limbs. He was well nourished and in excellent
general heal health and was even mainstreamed in a normal nursery
school. At age 10 he died unexpectedly for unknown reasons An autopsy
was not performed.
Vision On examination at age 6 weeks he reacted to
bright light: pupils constricted. but funduscopic examination revealed
bilateral optic atrophy. Flash visual evoked potentials showed ''poor
formation of the major positive component with a relative delay." On
repeat at 6 and 25 months there was a retinal potential but no posterior
waveforms. At 25 months a pediatric neuroophthalmologist confirmed the
optic atrophy but noted that VA was attracted by light in the
left eye's temporal field. there was nystagmus but "not really
wandering vision of the blind." He concluded that VA had some vision
and that only time would tell how much. At age 5 years, a neurosurgeon
noted VA to be "fairly responsive to visual threat."
On examination by DAS at age 6, VA blinked to threat and
closed his eyes to bright light. Although irregular conjugate
nystagmus interfered with fixating, he grossly tracked
objects and faces consistently and changes in affect or movement
indicated active attending. His motor deficit precluded reaching for
objects, but he visually interacted with the environment in other ways.
such as scooting around the house (see below).
object discrimination. From age 3 on, therapists noted
that he distinguished among toys, certain ones eliciting the most
smiling. giggling, and moving. It is unclear whether there preferences
were based on appearance, tactile quality, sound, or a combination
Music Discrimination At age 6 weeks response to sound
was documented. and auditory evoked responses were normal. At 3 years VA
began regular music therapy. Therapists noted how he was consistently
stimulated by music and reacted differently to different types of
music. He distinguished new from familiar pieces and had clear
favorites. While listening to Prokofiev's Peter and the Wolf
during DAS's visit his behavior and facial expressions
appropriately reflected the changing instrumentation and mood.
Goal directed behavior At age 2 the
neuroophthalmologist documented that VA was fairly mobile when supine.
pushing himself around in a circle with his legs. According to mother he
could tell whether the sliding glass door to the sun porch was open and,
if so, scoot through to enjoy the warmth and sunshine. Author PAB
witnessed him scoot around the house. visually avoiding collision with
walls and furniture.
Orientation. He would turn in the direction of someone
calling him and smile.
Socialization. By age 3 VA's irritability had subsided. and
positive affect became predominant. He smiled when spoken to and giggled
when played with. These human interactions were much more intense than.
and qualitatively different from, his positive reactions to favorite
toys and music. He never developed stranger anxiety.
"Mirror test" During the authors'' visit, VA showed
fascination with his own reflection. Despite efforts to distract him, he
kept turning back to it, studying it intently and smiling.
Case 2 (DC)
DC was born full term following an unremarkable pregnancy and
delivery Examination was normal except for a head circumference of 42 cm
and widened sutures. Her entire; head transilluminated. Arteriography
showed poor visualization of anterior and middle cerebral arteries but
normal external carotid and vertebro-basilar systems (posterior
cerebrals not specifically mentioned). During placement of a
ventriculo-atrial shunt, the only subpial tissue found war a thin
fibrous membrane. histiologically hemosiderin-laden and devoid of
Parents were told DC would remain vegetative and probably die
within a few months. so she was institutionalized. Spastic quadriplegia
and cortical blindness soon manifested At age I:1/2. still unresponsive.
she was taken into foster care by the same nurse who adopted
Hydranencephaly was reconfirmed around 51/2 years. when author
GLH became her neurologist CT scan showed no supratentorial parenchyma
above the thalamus, except for a thin left inferior temporo-occipital
remnant and even less on the right (Fig 3). An EEG was isoelectric
except for low-amplitude nondescript activity in the temporo-occipital
She developed brief staring and longer tonic episodes, treated as
seizures although of unclear nature. She ate orally until age 10, when
tube feeding became necessary.
When examined by authors DAS and PAB at age I3. she had marked
positional plagiocephaly, head circumference
of 56 cm, and bilateral optic atrophy. Pupillary reflexes and
extraocular muscles were intact, with wandering gaze and
nystagmus. She had marked spastic quadriplegia with axial hypotonia and
muscle wasting. She could move her right arm and kick with both legs.
Emotion was manifested through facial expression and vocalization.
DC developed regular, brief menstrual periods around age 14- 1 5.
transiently exacerbating her "seizures." She remained healthy
until age 17, when increasing lethargy set in. Shunt malfunction
was suspected, but hospitalization and invasive procedures were
foregone. and she died peacefully at home. No autopsy was
Although motorically and visually,· DC was much more disabled
than her foster brother VA, she exhibited finer, though subtle,
Vision. Light/dark discrimination evidenced early. She had
difficulty sleeping in the dark and "complained" until lights were
turned back on. Around 4 years she began to track objects intermittently
and became upset if her view was blocked. An optometrist found visual
evoked responses to flash. gross checkerboard and bar gratings,
suggesting acuity between 20/600 and 20/200. Waveforms were
simple and at a markedly prolonged latency around 200 msec. Over the
years. mother became convinced that DC sometimes identified her by
purely· visual cues. About half the days DC seemed to see. The visit by
DAS and PAB fell on a "bad" day: she rolled her eyes to bright light but
showed no tracking or optokinetic nystagmus.
Discrimination of Persons. When received into foster care at
age 1 1/2. DC showed no interaction with persons or environment. From
then on she received constant affection and multimodal stimulation from
her new mother and a therapist. For the next half year she remained
unresponsive even to this enriched environment. Gradually, however, both
mother and Iherapist noticed that she seemed more at ease in her own
home than elsewhere.
By age 5 she consistently recognized certain individuals
nonvisually and responded to people differentially according to three
categories: mother, familiar persons. and strangers The more familiar
someone was, the more she would relax, move spontaneously and vocalize.
At age 6 a neurosurgeon described her as happy and very responsive to
At age 12 her neurologist author GLH, noted that she smiled,
turned to sound, and seemed to enjoy music. She was aware of mother's
presence and became upset if separated. On author PAB`s first home
visit, DC grew anxious at his approach and withdrew fearfully when he
gently took hold of her arm, but calmed again to comforting by mother.
During the joint visit of DAS and PAB. she seemed to enjoy being stroked
by mother and was relaxed with a familiar music therapist: but when DAS
approached. speaking soothingly and touching her as gently as possible,
she became tense and apprehensive. with a change in respiratory pattern
and more eye deviation toward mother at the other side of the bed
Musical discrimination Between 3 1/2 and 4 DC first
manifested preference for certain kinds of music (ballad,, rhythmic
dance. or marcher) and particular songs (eg. Send in the
Clowns). as well as dislike for other kinds of music (Mozart,
loud rock), From age 6 to I2 she was visited weekly by a particular
music therapist, who eventually (after more than a )ear)
was accepted into DC's circle of "familiar people", The therapist
confirmed that DC was typically indifferent to most new pieces.
but giggled and kicked to favorite pieces. If the therapist
intentionally made an error or suddenly switched songs in the
middle of a favorite one. DC would change facial expression turn head or
eyes. and cease vocalizing. She preferred live music to recordings of
the same pieces. and responded more to this therapist singing a favorite
song than to an unfamiliar therapist singing the same song She would
also orient toward, and reach out to touch. a nearby sound
DAS's visit coincided with a return of this therapist who had been
away several months. The two quietly entered DC's room, and the
therapist began to sing Send in the Clowns, accompanying herself
on the piano. DC was at first expressionless but seemed to attend. After
45 second. she began to smile and gradually became more animated, with
smiling, vocalizing and movement When DAS played the same version of
Send in the Clowns, DC was less responsive. As mother
predicted, she had no reaction to Mozart and romantic works, but
became animated and vocalized to two bouncy dances from a Bach partita.
She became indifferent again to slower movements from the same work.
Case 3 (OA)
Following an uneventful pregnancy OA was delivered by Cesarean
section for breech presentation with birth weight 3.785 kg and
head circumference 38 cm. Magnetic resonance imaging (MRI)
revealed a gross brain malformation mainly resembling
hydranencephaly but partially alobar holoprosencephaly. The
Supratentorial space was empty except for a thin slab of frontal
lobe a without midline fissure. A repeat scan at 10 months was
Mother was told that OA would forever remain a "vegetable" and most
likely not survive beyond 2 years. A neurologist, after reviewing the
MRI, said that OA’s brain was "like that of a reptile" and that she
would never socially interact. Her hydrocephalus steadily
increased. but shunting was discouraged on the grounds that it would
only lead to "more suffering.' on OA’s part. and institutional
placement was urged. At age 2 months her feeding slowed to only 2
ouncer every 8 hours, and mother was told that OA's few brain
cells were "dying." Despite such negative assessments, mother insisted
that a ventriculo- peritoneal shunt be placed; if has since
functioned well, requiring one revision at age 4 1/2 years.
At 6 months. OA was transferred to a new pediatrician and began
"relaxation and distraction therapy." Soon her initial irritability
subsided and the began to eat well She has remained in excellent health.
An awake EEG at age 5 1/2 showed moderate-amplitude 2-5 Hz
frequencies frontally, but no definite electrocerebral activity
elsewhere amidst much artifact.
OA was 5 1/2 years old when visited by author DAS She had a
happy. engaging affect. Head circumference was 51 1/2 cm. A
hyperactive blink reflex did not habituate to glabellar tapping. though
eye fluttering to a ratchety noise did. Cranial nerves were
unremarkable. She had spastic quadripareris. sustained clonus, palmar
and plantar grasp reflexes, and bilateral Babinski signs. Despite axial
hypotonia she could raise her head and control it somewhat when propped
sitting. A stepping reflex enabled her to "walk" with axillary support.
Vision: After only a few weeks mother suspected that
OA could see. Between 4 and 5 months she began to smile responsively,
and thereafter vision was unquestionable. She was evaluated twice around
age 2 by an ophthalmologist, who noted that fundi uere normal and visual
fixation was -'central. steady and maintained." Acuify was not
estimated. For DAS she demonstrated a nonhabituating "virtual suck"
reflex, in which her mouth would open and tongue protrude at the
approach of any object. She smiled responsively, tracked faces
and toys, and oriented immediately to objects brought into the
Orientation. When called, she would raise her head,
look at the person and smile
0bject Permanence. When an object she was tracking
while held sitting was suddenly whisked behind her, the would turn in
search of it.
Person discrimination Between 3 and 4 months
the manifested a slight preference for mother She never developed
stranger anxiety, but senses if someone is uncomfortable with her and
stiffens. She cried constantly during a visit of a therapist whom mother
described as "condescending" As soon as the therapist left, she stopped
crying and smiled at mother. She developed a liking for puppies and
small children: her eyes "light up" whenever the sees one.
Socialization She clearly enjoys being with
people and even interactively plays. A "conversation" with mother is
documented on video. in which OA attempted to imitate monosyllables and
even uttered "ah-ah" to coaxing to say "mama." In another scene she
attempted to stick out her tongue in an, imitative game with mother,
Musical and emotional discrimination. By history. when OA
hears a happy song she enlivenrs and seems to want more when it ends;
conversely, she tends to cry with sad songs. During DAS's visit
she did enjoy some happy songs, but no sad songs were available for
comparison. Once when a babysitter was crying. OA began to cry
Body awareness. At 6 months OA began to manifest
awareness of her body. eg. if her face was hurt, she would stroke
it with her hand. She enjoys vestibular and vibratory stimuli: eg..
during a car ride, she cried at stop and immediately calmed upon
resumption of motion.
Associative learning. She startled and stiffened when
a vacuum cleaner or hair dryer war turned on. making a loud unpleasant
noise. After several such experiences, she also stiffened in
anticipation if either object (though .'off') was brought near.
She developed a small receptive 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 correctly looked at the
object or person.
Case 4 (RB)
RB was born to a single mother who intended adoption. Because of
abnormally positioned ears a CT scan was obtained, diagnosing
hydranencephaly. On re-examination his head transilluminated. RB was
adopted at 6 weeks by a former nurse who provided a home for
disabled children, She was told that he would die soon and never develop
RB soon demonstrated severe spastic quadriplegia. Despite physical
therapy, diazepam, and baclofen marked flexion contractures developed.
He required gavage feeding for two years, but then ate orally until age
I I, when a gastrostomy tube had to be placed.
RB has always appeared cortically blind, with some response to
darkness and light but no visual tracking. An ophthalmologic examination
revealed severe optic atrophy.
Seizures were suspected, with motionless staring, often progressing
to head deviation and facial twitching for around a minute, followed by
drooling. Phenobarbital was begun and is still taken: seizure frequency
is now around two per month. Three EEGs, at ages 4,. 4 1/2 and 9 1/2
years. all showed no activity in frontal, central and temporal leads.
The occipital region had frequent epileptiform discharges and no change
with eye opening or closing.
At age 10. RB required a permanent tracheostomy for airway
obstruction due to macroglossia. A CT scan at that
time showed occipital lobe remnants
but no other cerebral cortex.
Posterior fossa structures were normal.
Since then, he has remained in
excellent general health, puberty began
around 13 or 14 years.
At ages 9. 12, 14 and 17,
RB was evaluated with the
Vineland Adaptive Behavior Scales,
Age-equivalent scores ranged initially from
4 to 10 months and recently from
I to 5 months. with a decrease in the
Daily Living domain related to inability
to take food orally.
He was 17 years old when
visited by author DAS. Head
circumference was 17 cm Pupils
reacted to light. Eye movements were
roving and nystagmoid, without fixation
or following Extremities had fixed
contractures. and reflexes were hyperactive
with sustained clonus. Facial expression
and slight head turning were his only
means of communication.
Nonverbal communication and affect
.At age 9 a developmental psychologist
observed cooing sounds, expressions of sadness or pain, and smiling in
the presence of caregivers. At age 14. on re-evaluation he cooed and
laughed. and by age 17 he indicated preferences through facial
expressions and a broad smile.
Person recognition. Ability to distinguish
mother became evident around 2 years. when he would stop sucking a
bottle and attend when mother spoke to him but not when others; did.
Ever since he has reacted indifferently to strangers but positively to
her. His eyes often turn toward her voice. even though he does not see.
When at age 10 he was taken to the hospital for upper airway)
obstruction. she had to accompany him in the ambulance: as long as he
heard her reassuring voice and felt her caresses his oxygen saturation
remained tenuously stable, but whenever the stopped. it quickly
This discriminative ability was repeatedly corroborated by the
developmental psychologist,. who noted that at
age 9 RB smiled in response to caregivers, at 12 and 14 he
distinguished mother's from father's voice, and at 17 he enjoyed mother
stroking his face and tolerated pain better in her presence. During
DAS`s visit he was unresponsive to the author's attempts at vocal and
tactile interaction. but smiled consistently to mother's voice
Orientation. At ages 9 and I2 he was documented to turn head
and eyes toward sound and his head toward tactile stimulation.
Musical discrimination RB`s mother frequently exposed
him to music. At age 14 the psychologist noted that he enjoyed music,
particularly with deep sonorities. From 16 on. he has had weekly
sessions with a music therapist. She gradually found that
particular pieces and types of music (eg. with strong rhythms and
high-pitched instruments) elicit positive affect more consistently than
others: he smiles radiantly and sometimes laughs. whereas his affect
blunts when the music stops or less favorite pieces are played.
Environmental sounds elicit no such response. He distinguishes live
instruments and especially enjoys the therapist`s maracas and tambourine
and to hare the stereo speaker placed on his chest. At times he has
followed the sound of her maracas with his eyes as she walked
around his bed
During DAS`s visit the music therapist came, and these differential
responses were observed. when DAS played some classical music RB
remained apathetic, but he smiled and became animated with a tape of
"Favorite" band music accompanied by live maracas.
Each of these children defied a prognosis of permanent vegetative
state, rendered with absolute certainty by multiple physicians,
including pediatric neurologists and neurosurgeons. Any one of these
cases suffices to disprove that all content of consciousness, including
pain and suffering. is necessarily mediated by the cortex. That four
such cases have come to the authors' attention through pure happenstance
suggests that subcortical mediation of consciousness in congenitally
decorticate children might not be so uncommon as the Multi-Society Task
force seeml to imply (1994a (p 1504) These findings therefore raise
important questions about our assumptions regarding consciousness and
Is the cortex necessary for consciousness?
It would far exceed the present scope to enter into a philosophical
discussion of the definition and possible operational criteria for
something so fundamental yet elusive as "consciousness." Suffice it to
emphasize the term's inherent ambiguity. deriving from the
`'bidimensionality" of human consciousness; the simultaneous awareness
of the physical world (including one's own body) and awareness of
that awareness (i.e.. "self-awareness. "reflective awareness") (Plum and
Posner 1983 (p1).
The AAN position statement on PVS (1989) implicitly ascribes to
"consciousness" both dimensions paraphrasing "eyes-open
unconsciousness" as a state in which "at no time is the patient
aware of him,- herself or the environment" (emphasis added).
Similarly, its more recent "practice parameter" defines "vegetative
state" as involving "complete unawareness of the self and the
environment" (emphasis added) (American Academy of Neurology 1995),
Nevertheless. biologists (not to mention animal rights activists) speak
meaningfully of "consciousness" in animals where only the behavioral.
operationally definable, non-reflective "dimension".. is implied.
Unarguably. such "consciousness" is just as properly attributed to the
described here. Were they not humans studied by clinicians but rather
animals studied by ethologists, no one would object to attributing to
them "consciousness" (or ability to "'experience" pain or suffering)
based on their evident adaptive interaction with the environment This
alone is surely remarkable. Even prescinding from the question of
self-awareness. the possession by decorticate children of even
animal-type "consciousness'· thoroughly contradicts prevailing
PVS orthodoxy, which predicts that they should be precisely
vegetative, not sentient and intentionally behaving.
Whether or not their consciousness also has an ··orthogonal"
reflexive dimension is empirically unanswerable. Self awareness cannot
be reduced to mere external manifestations (ie e. linguistic
self-reference): neither can its absence be inferred from mere absence
of such manifestations. especially if the linguistic apparatus is
pathologically or developmentally inadequate. Far example. there is no
reason to suspect that autistic children. global aphasics. and preverbal
infants lack reflective awareness merely because they do not talk about
Some authors claim that the best test for self-awareness in animals
is recognition of their own body in a minor (Korein 1997). Whether
behavior before a mirror reflects true self-awareness of a mind. as
opposed to an extension of body-awareness or even mere
fascination with control over the image's movements (perceived as
extra-self). is debatable. In any case. VA was as interested in his
reflection as any infant or simian who passes the "mirror test.'
Although agnosticism about self-awareness might theoretically be the
only scientifically rigorous position, practical prudence demands giving
the benefit of the doubt
that any member of the species Homo sapiens who is
behaviorally conscious is reflectively so as well. Thus have we
always treated autistics and aphasics. Only recently, however, has the
same enlightened stance been extended to preverbal infants (Anand and
Hickey 1987), and there is no a priori reason not to extend it
also in decorticate children with environmental awareness. After all. we
feel compelled to treat "humanely" laboratory animals with even smaller
Were these children truly decorticate?
One might argue that the remarkableness of these cases is
muted by the fact that none of the children was absolutely devoid
of cortical tissue: they were not truly· "decorticate"·
The proper nomenclature for VA's and OA’s pathology is
admittedly debatable: it is more dysgenetic than classical
hydranencephaly (the end-product of in utrero necrosis of
normally developing hemispheres (Halsey et al 1971, Samar 1992)).
VA had mesial temporal remnants and a large supratentorial cyst
partially lined by tissue capable of generating epileptiform discharges
OA has a sliver of holoprosencephalic frontal lobe. But the point is.
even if these two children were not "decorticate' absolutely. they were
enough so that physicians. including neurologists. predicted a
vegetative outcome absolutely
Even in classical hydranencephaly there is often a thin remnant of
inferior temporo- occipital cortex. (Halsey et al. 1971), as
exemplified in cases DC and RB. Typically this tissue does not mediate
vision because it is severely gliotic and optic radiations are absent.
Thus. despite the cortical remnant. such children are universally
cortically blind, as was RB (and DC on "bad" days). and the literature
does not hesitate to label them "decorticate.. (Berntson et al
1983. Deiker and Bruno 1976. Halsey et al 1968) and to consider
them as necessarily vegetative (Multi-Society Task Force an PVS 1994a (p
The most important differential diagnosis is with "maximal
hydrocephalus." in which the cortex is basically intact though extremely
compressed (Linuma et al 1989. Sutton et al 1980)
Refinements of modern neuroimaging make this distinction less difficult
than it used to be Also. the EEG is relatively normal in maximal
hydrocephalus but virtually flat in hydranencephaly. In each of our four
cares all diagnostic information taken together leaves little ground for
concern over possible misdiagnosis of maximal hydrocephalus.
The main point is that these children's consciousness can be inferred
to be mediated subcortically not, because there were absolutely
zero cortical neurons, but because the few that were present could not
plausibly subserve the totality of their conscious behaviors.
That is why parents were invariably told -with complete confidence by
relevant specialists - that their child would unquestionably remain in a
vegetative state for as long as he or she lived. Experienced
neurologists, to whom the authors have shown the CT and MRI scans with
an invitation to guess the child's Ievel of functioning. also typically
predict vegetative state.
This is not to say that the number and distribution of telencephalic
neurons played no role in these children's cognitive repertoire. The two
with rudimentary limbic structures (VA and OA) were more affective and
sociable than the two with classical hydranencephaly·. and they also had
more motor function. Ironically, they also possessed the most vision
despite total lack oloccipital cortex. in contrast to the other two, who
had little or no vision despite occipital remnants. It seems as unlikely
that the occipital tissue in the latter two mediated their
discriminative affect as that the limbic tissue in the former two
mediated their vision. What is functionally common to all
(consciousness per se) is more logically attributable to
structures common to all (diencephalon and brain stem) than to
idiosyncratic structures (OA's frontal sliver, VA's mesial temporal
tissue, and DC's and RB’s occipital slabs,)
In principle the anatomical substrate of their various cognitive
functions could be clarified noninvasively by high-resolution positron
emission tomography. or less practically by· functional MRI
Unfortunately. logistical and economic obstacles precluded such
tests. and we must make do with inferences from the information
Do subcortical structures possess "vertical" plasticity?
That subcortical mediation of consciousness has been described so far
only in congenital brain malformations suggest that developmental
plasticity may play a role. Although both cortical plasticity for
cortical functions and subcortical plasticity for subcortical
functions ("horizontal" plasticity) have been known for many decades
(Cotman 1985, Finger and Wolf 1988. Flohr and Precht 1981),
subcortical plasticity for supposedly cortical functions ("vertical"
plasticity) has not previously been reported. apart from cases VA
and DC in an abstract (Shewmon and Holmer 1990) and mentioned briefly
elsewhere (Shewmon 1992).
Vertical plasticity must be less robust than horizontal plasticity:
intuitively, potential for compensatory reorganization ought to be
largely related to the degree of microstructural similarity between
sites at issue. But it would be gratuitous to exclude a priori
the very possibility of vertical plasticity. Perhaps the
strongest argument for its role in our cases is that the two children
with vision despite fetal absence of occipital cortex had brain
malformations arising earlier in gestation than the two with no
vision despite occipital remnants. presumably in the latter cases, prior
to telencephalic infarction the visual system had developed so that
relevant subcortical nuclei were already committed to a functional
relationship with occipital cortex whereas in the former the absence of
occipital cortex all along allowed these subcortical nuclei "free rein'·
to organize optimally for functional vision.
If such vertical plasticity can occur with vision there is no reason
to suppose it cannot also occur to some extent with other sensory and
motor modalities and with their mutual interactions mediating
adaptive environmental relatedness. i.e. with consciousness
(at least its behavioral. operationally definable dimension). This
should not be surprising, given:
(I) the primarily subcortical mediation of certain sensory
modalities. especially pain
(Bromm and Desmedt 1995, Lenz 1991, McQuillen 1991, Willis 1989) with
cortex serving a more modulatory role (Talbot et al 1991)
(2) the non-postulation of any cortical representation certain other
sensations. especially visceral ones such as nausea, thirst, etc.
(Brookhart et al 1984, Kandel et al 1991)
(3) the distinction between pyramidal and extrapyramidial
motor systems,. the former governing fine distal activity and the
latter gross, proximal/axial activity (Davidoff 1990, Lawrence and
Kuypers 1968a, 1968b, Sarnat 1989) (with hemispherectomy loss of
individual finger movement is a pyramidal deficit, whereas gait and use
of the paretic arm as a "helper" derive from the extrapyramidal system:
our cases are motorically and anatomically equivalent to bilateral
(4) the role of the nucleus reticularis thalami in attentional focus
and relevance-based pre-cortical sensory "filtering" Crick 1984, Hobson
and Steriade 1986, Scheibel 1984) and
(5) the "distributedness" (both horizontal and vertical) of brain
systems mediating higher functions (Freeman 1990, John 1990,
Mesulam 1990, Pribam 1990)
The hydranencephaly literature documents subcortical mediation of
certain cognitive functions usually attributed cortex, such as
distinguishing mother, associative learning. consolability.
conditioning, orienting, and visual tracking (Aylward et al 1978.
Barnet et al 1966. Bemlron and Micco1976, Berntron et al.
1983, Brackbill 1971, Deiker and Bruno 1976. Francis et al 1984.
Graham et al 1978, Hairy et al. 1968, Nielsen and Sedgwick
1949. Tuber et al 1980).
In the pre-CT scan era Lorber (1965) described a remarkable case of a
boy diagnosed with "hydranencephaly" by pneumoencephalogram who had
developed normally as of 21 months of age. The X-ray seemed to show air
right up against the inner table of the skull. Nevertheless normal
development is so implausible with hydranencephaly yet perfectly in
keeping with maximul hydrocephalus that one cannot help doubting the
sensitivity of the air study. Lorber seated that an EEG -showed evidence
of some electrical activity." but its quality and distribution were not
described. If the EEG was as relatively normal as the child, then surely
this was misdiagnosed hydrocephalus. Lorber concluded. "there ought
to be some cerebrum somewhere, as it is impossible to explain his
progress otherwise At this stage. one can go no further and he
remains... an enigma." fifteen years later. Lorber reported patients
with cortex as.thin as I mm from hydrocephalus, yet
neurologically normal (Lewin 1980) Follow-up on the normally
developing 'hydranencephalic" boy, however, was not provided.
The cases reported are not at all similar to Lorber's; they had gross
brain dysgenesis or bona fide hydranencephaly, and all
were cognitively and motorically severely disabled The
impossibility of their having misdiagnosed maximal hydrocephalus
reinforces more convincingly Lorber's and others speculation that
subcortical structures may play a greater role in consciousness
than is usually assumed (Berntson and Micco 1976,
Some authors have hypothesized primarily subcortical vision in normal
human newborns prior to postnatal encephalization (Bronson 1974, Snyder
et al 1990) Fetuses in utero can distinguish and remember
sounds (DeCasper and Spence 1986, Restak 1986) and term infants prefer
mother's voice to other women's. and women's
voices to men's (DeCasper 1980), even
though they have very low
cerebral cortical metabolism not dissimilar
to adults diagnosed in PVS (Chugani
et al 1987. Levy et al
There is also phylogenetic precedent for subcortical mediation of
some complex behaviors and perceptual functions traditionally
regarded as'` cortical." Eg.. habituation.
learning. and discriminative conditioning have been
observed in decorticate animals (Bromiley 1948.
Finger and Stein 1982 (pp
245-250), Huston and Borbely 1974,
Norman et al 1977, Travis
and Woolsey 1956). Binocular depth
perception is exhibited exquisitely by
falcons. owls, toads and grass
frogs, although they possess little
or no visual cortex (Collet and
Harkens 198t. Fox et al 1997,
Pettigrew and Konishi 1976). and
it can be brought out in cats
following bilateral occipital lobectomy
(Feeney and Hovda 1985. Hovda
et al 1989. Hovda and Villablanca 1990). Feline
vertical plasticity is evidenced in that adult cats bilaterally
hemispherectomized as kittens behave nearly indistinguishably
from normal. in marked contrast to cats hemispherectomized as adults
(which are severely disabled) (Bjursten et al 1976. Burgess and
Villablanca 1986. Burgess et al 1986. Villablanca et al 1986)
This animal evidence is cited, not to imply that cortex and
subcortical structures must have the same role in humans as in animals
and the same potential for plasticity (the perils of cross-species
extrapolations are well known), but rather to emphasize how much more
parsimonious it seems (absent direct data) tentatively to ascribe OA's
visual function, for example, to subcortical pathways known to subserve
vision in animals rather than to her rudimentary frontal lobe: much less
radical reorganization would have to take place. On the other hand, the
cortex's capacity for transmodal reorganization may also be greater than
previously imagined, as evidenced by recent studies of occipital
activation by tactile Braille reading in people blind from an early age
(Büchel et al 1998. Cohen et al 1997). Clearly this
exciting field is wide open for fruitful research.
Why are such cases so rare?
But if consciousness in congenitally decorticate children occurs by
virtue of diencephalic and brains stem plasticity. why should it not
occur in all, or even most. such children? Five possible reasons suggest
1. Susceptibility to unfamiliarity First decorticate children
are extremely sensitive to changes in routine and environment. They are
easily disturbed by rides to doctors' offices and by strange people and
surroundings, in such settings they often involute and fail to manifest
any cognitive functions that parents might report. (This is why home
visits or home videos are a particularly important means of
2. Intermittence of function Secondly, certain functions may
be intermittent even at home (eg; VA's scooting, DC's tracking),
reducing still further their probability of being witnessed during a
brief office visit. let alone in an emergency room or intensive care
3. Physicians' lack of time. Thirdly, the preceding two
obstacles are compounded by the brevity of time that doctors often have
for taking detailed developmental histories and examining for subtle
functions that may not be immediately manifest.
4. Mental filtering Fourthly we
physicians have learned through experience to interpret implausible
parental claims about abilities of severely disabled children as
psychological denial. On the other hand, we should be on guard against a
form of denial ourselves, ignoring evidence inconsistent with our (often
simplistic) theories of brain functioning. We all probably engage in
more selective information-filtering than we would like to admit.
5. Self-fulfilling prophecy But perhaps the most important
reason why such cases are so rare is that the label "developmental
vegetative state" tends to be self-fulfilling. Sensorimotor and
emotional deprivation in even neurologically normal infants leads to
profound apathy, failure to thrive and developmental delay (Dietrich
et al. 1983, Koluchova 1972. Money 1977. Perry et al 199S.
Powell and Bettes 1992. Weston et al 1993). How much more should such
consequences be expected if the deprived infant is severely disabled.
Nevertheless, the uniformity of "vegetative" outcomes in decorticate
infants treated as ”vegetables" is accepted uncritically by many as
"evidence" that congenital decortication necessarily produces a
"developmental vegetative state".` This is analogous to the now
acknowledged tragedy of many potentially functional individuals
with Down syndrome who became victims of
self-fulfilling prognoses of severe mental
retardation (Canning 1978. Zausmer 1978).
Indeed, the parents of all four children reported here were initially
warned by most physicians that their child would unquestionably never
have a mental life. Whether some physicians actually used perjorative
terminology, or parents simply reinterpreted over time the recollection
of those conversations, matters little for understanding the
self-fulfilling tendency of the label "developmental vegetative state.'.
Regardless of the words spoken. parents
were often left with a sense o of not
merely a bleak developmental outlook but
even a dehumanizing attitude toward the
child. On occasion (eg. when the child
was brought to an emergency room
or required intensive care) some
parents were given the impression that
certain physicians felt they were
wasting valuable time and "scarce
resources" on something subhuman or even
sub-animal (i.e, a "vegetable." even if
the word was not used explicitly)
If these children had been kept in institutions (as DC was for the
first 1 1/2 years) or treated at home as "vegetables" (the
prognosis being accepted uncritically by
parents). there can be little
doubt that they would have turned out exactly
as predicted· What surely made
all the difference was that their
parents ignored the prognoses and
advice, and instead followed their
instinct to shower the children with loving stimulation
and affection. Such children and
their families have much to teach
about not only the neurophysiology of
The authors wish to thank Mr.
Stephen Wire for assistance in
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