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A Special Case of Anencephaly in an Early-Born Baby with an Exagerated

Prognastic Face: Further Example for Human Devolution
Üner Tan a
a
Çukurova University, Faculty of Sciences, Adana, Turkey

Online Publication Date: 01 June 2008

To cite this Article Tan, Üner(2008)'A Special Case of Anencephaly in an Early-Born Baby with an Exagerated Prognastic Face:
Further Example for Human Devolution',International Journal of Neuroscience,118:6,751 — 760
To link to this Article: DOI: 10.1080/00207450701668004
URL: http://dx.doi.org/10.1080/00207450701668004

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 International Journal of Neuroscience, 118:751–760, 2008
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ISSN: 0020-7454 / 1543-5245 online

DOI: 10.1080/00207450701668004

A SPECIAL CASE OF ANENCEPHALY IN AN

EARLY-BORN BABY WITH AN EXAGERATED
PROGNASTIC FACE: FURTHER EXAMPLE FOR
HUMAN DEVOLUTION
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ÜNER TAN
Çukurova University
Faculty of Sciences
Adana, Turkey

A 7-month-old baby was born in a village near Iskenderun (Turkey) where “Unertan
Syndrome” with quadrupedality and primitive cognitive abilities was discovered.
The clinical diagnosis was anencephaly. However, his head did not show the classical
symptoms of anencephaly because it was covered with bony structures. The baby
has an ape-like, prognasthic head with low-set ears and flapped ear flaps. The other
parts of the body were similar to humans with broad shoulders and a short neck.
This may be a further example of human devolution, which was first reported by
Tan (2005, 2006a,b,c). A genetic defect affecting the head development including
brain may be responsible for the reappearance of the ape-like head in a human
being. This human devolution, or evolution in reverse, suggests that the same gene
or gene-pool as well as the interactions between genes may be responsible for the
transition from our ancestors into human beings with regard to an orthognasthic
head, and brain development.

Keywords anencephaly, brain, devolution, evolution, premature, prognathism

INTRODUCTION
The present work deals with an early-born baby with a clinical diagnosis of
anencephaly, which is a congenital defect affecting the formation of the brain

Received 22 November 2007.

This study is partly supported by the Turkish Academy of Sciences, Ankara, Turkey.
Address correspondence to Prof. Dr. Uner Tan, Çukurova University, Faculty of Sciences,
Department of Physics Adana 01330, Turkey. E-mail: unertan37@yahoo.com
751
 752 Ü. TAN

and the skull bones. The brain often lacks part or all of the cerebrum. There
is no bony covering over the back of the head. Anencephaly occurs in about
1,000 to 2,000 births in the United States each year. The incidence of the
anencephalic births between 1898 and 1991 has been reported to be 0.52% in
Istanbul Cerrahpasa Medical School (see Tasdelen et al., 1996).
The well-known main symptoms of anencephaly are absence of bony
covering over the back of the head along with missing bones around the front
and sides of the head. The present case did, however, not fit to the main
symptoms of anencephaly. From the inspection of the head, a very small brain
(microcephaly) was apparent especially in the parieto-occipital regions of the
skull, which was entirely covered with bony structures.
Interestingly, this baby was born with an ape-like head exhibiting an
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exaggerated prognasthic face and flat head, similar to human ancestors.

Therefore, this case can be considered as an example for human devolution,
which is the opposite of evolution (see Tan, 2005, 2006a,b,c). The devolution
is explained in terms of a passing down or descent through successive stages
of time or a process (The American Heritage
R
Dictionary of the English
Language), meaning to regress or degenerate, to evolve backward (Urban
Dictionary). This is the act of evolution in reverse (see Porter & Crandall, 2003).
According to the American Heritage Dictionary, devolution or degeneration
in biology mean retrograde (biological) evolution; a continuing process of
degeneration or breaking down in contrast to evolution. Reverse evolution as
a change in character state to one resembling an ancectral state (Porter &
Crandall, 2003) will be referred to “devolution,” throughout the present work,
as first suggested by Tan (2005, 2006 a,b,c). The importance of this biological
devolution or evolution in reverse as an influential evolutionary phenomenon
is recently well illustrated in a review article by Porter and Crandall (2003).
The present work is aimed to report and analyze this an early-born baby with
an ape-like head—protruding jaw and face, flat skull—and a human-like body
with normal arms and legs, that is, half ape-half human being, with regard to
human devolution.

METHOD
The baby was born as he was seven months old in a small village near
Iskenderun, near another small village where the individuals exhibiting the
“Unertan Syndrome” were born.
The baby had an unusual facial appearance. The shoulder were larger than
normal. The body weight was 1,100 g and the body length was 35 cm. This is
 HUMAN DEVOLUTION 753

the first child of a non-consanguineous young family. Prenatal ultrasonography

was not performed because of the low socioeconomic status of the parents. The
family history was not contributory; there was not a similar early-born baby
in their relatives. The baby died one hour after birth within the incubator. The
parents buried the baby immediately after death. Permission for authopsy of the
cerebrospinal structures was not available. Therefore no DNA analysis could
be performed.

RESULTS
The male baby with an ape-like head and a human-like body is illustrated in
Figure 1. Except the head, the body seems to be normal (Figure 1). The baby
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has exophtalmic eyes, broad shoulders, short neck, and thick arms. The jaw and
face are markedly protruded (prognathism; see the picture in the middle) like
in apes, and in most animals. The head is flattened, especially in the posterior
regions, and entirely covered with bony structures with long hairs. The back of
the baby seemed to be quite normal, except the short and thick neck (Figure 1).
There were also no anomalies in the spine of the baby.
Most of the human beings have markedly decreased facial projection
(orthognasthic face). However, there are also some humans exhibiting projected
jaw and face, although they may not be as exaggerated as the baby presented in
the present work, and in apes. Figure 2 presents an example of African children
with prognasthic facial appearances, which was published by Hrdlicka in 1928.
A marked prognathism is typical in many non-human primates. Figure 3
shows the facial prognathism in baby monkeys and a squirrel, similar to the
exaggerated prognathism in the half-ape and half-human baby, contrary to the
markedly decreased facial projection in normal human beings.
A comparison of prognasthic primate skulls in chronologiacl order is
illustrated in Figure 4. As visualized in Figure 4, the brain size increases,
whereas facial and dentognathic size decreases during phylogenesis. That is,
there is an inverse trends in absolute cranio-facial size during chronological
evolution of hominidea. There is a gradual shift in craniofacial structures from
a ancestral pattern with a strong facial prognathism to a facial retraction in
modern human beings (see Polanski & Franciscus, 2006).

DISCUSSION
A 7-month-old premature baby with an ape-like head is presented for the first
time in the scientific literature. According to the gynecologist’s diagnosis, it
 754 Ü. TAN
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Figure 1. Pictures from the half-ape, half-human baby: whole body (above), half body (middle),
back (below).
 HUMAN DEVOLUTION 755

Figure 2. African children with prognastic faces. From Hrdlicka (1928).

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was a case of anencephaly. In the author’s opinion, however, it may merely be

a special case of anencephaly, because the baby did not exhibit the classical
symptoms of this syndrome.
The forebrain, meninges, vault of the skull, and scalp all fail to form in
anencephaly (see Koukoura et al., 2006), resulting from a failed closure of the
rostral end of the neural tube leading to a total or partial absence of the cranial
vault and cerebral hemisphere (Detrait et al., 2005). The half-human, half-ape
baby did not exhibit these classical symptoms of anencephaly. His head was
covered with bones and hairs, exhibiting no openings in the cranium and spinal
cord. The frontal part of the brain seemed to be existent, whereas the posterior
part of the brain seemed to be underdeveloped (microcephaly). This case may
be a special case of skin-covered anencephaly (see Lemire, 1988).
The congenital absence of the cranial vault with cerebral hemispheres
completely missing or decreased to small masses in anencephaly (see Lomholt
et al., 2004) may be associated with different genotypic deviations such as
ring chromosome 13 and trisomy (Chen et al., 2001; Hahm et al., 1999). Of 16
anencephaly cases, 5 belong to environmental factors such as maternal diabetes,
hyperthermia, fetal influenze, fetal aminopterin, and sodium valproate (see
Lynch, 2005). It is frequently reported that a folic acid supplementation reduces
the risk of anencephaly. Moreover, genes involved in folate metabolism may be
involved in anencephaly, from both genetic and an environmental viewpoints
(see Boyles et al., 2006). In the case of the half-human, half-ape baby, both the
genetic and environmental factors may play a role in ethiogenesis, because the
parents living in a small village are very low in educational and socioeconomical
level (see Mitchell, 2005).
 756 Ü. TAN
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Figure 3. Markedly increased facial projections in monkeys (above) and a squirrel (below).

The most interesting feature of the baby presented in the present work
is the ape-like (prognasthic) appearance of his face with low-set ears and
flapped ear flaps (see Figure 1). Otherwise, his body, legs, and arms seemed
to be normal except broad shoulders and relatively strong and long arms. The
 HUMAN DEVOLUTION 757
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Figure 4. Human ancestors with marked projections of facial and dentognathic appearances,
compared to markedly decreased facial projection in homo sapiens.
 758 Ü. TAN

prognasthic face structures are typical for human ancestors. As depicted in

Figure 4, the human ancestors, like apes, have marked projections of facial and
dentognathic appearances, compared to markedly decreased facial projection
in homo sapiens.
Regarding these features in the human ancestors and modern humans,
the half-ape and haf-human baby may represent ancestral traits in the skull.
This case cannot be a simple congenital anomaly, because the occurrence of
such a coincidence would be impossible to be a random event. In fact, this
case seems to be a further example for human devolution (see Tan, 2005, Tan,
2006a,b,c) or evolution in reverse (see Porter & Crandall, 2003). Accordingly,
Rushton and Ankney (2000) have reported a pattern of descending prognathism
in African, European, and East-Asian skulls and proposed that these structures
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shrank over the course of evolution to allow greater brain size, that is, an
evolution from prognathism to orthognathism. Now one sees the reverse, that
is, from orthognatism to prognathism. The traces of prognathism can indeed
be sporadically observed in some human beings in less developed forms.
Because the most neural tube defects including anencephaly may be attributed
to chromosome anomalies (see Lynch, 2005), it is plausible to suggest that a
causative gene or gene-pool might have caused the proposed devolution in the
half-ape baby.
It is plausible that the same gene or gene pool responsible from the
development of the ape-like head might also be responsible for the transition
from a prognasthic face in apes to an orthognastic face in homo sapiens.
This hypothesis is consistent with the theories of punctuated evolution, being
long periods of very little evolution interrupted by periods of relatively rapid
changes, in contrast to Darwin’s gradual evolution, being a gradual rate of
evolution. The punctuated evolution may occur if beneficial mutations sweep
successively through the population (Elena et al., 1996). Some hotspots were
indeed found in human genome for acquiring duplicated DNA sequences—but
only at specific time-points of evolution. Accordingly, long periods of genomic
stasis may be punctuated by relatively short episodes of duplicate activity
(Minghetti & Dugaiczyk, 1993). Mezhzherin (1997) has analyzed the genetic
differentiation of taxa from three Holarctic and three Afrotropical phyla of
small mammals, and found that (i) the distributions of fixed gene differences
are relatively independent; (ii) the speciation process is discontinuous, being
consistent with punctuated evolution.
Similar to the experimental studies on the evolution in reverse, Hrdlicka
(1928) has first reported children running on all fours, exhibiting prognasthic
faces (see Figure 2). Nearly 70 years later, Tan has discovered adult individuals
 HUMAN DEVOLUTION 759

exhibiting some ancestral traits, such as quadrupedal gait, and primitive

cognitive abilities including language and conscious experience (see Tan,
2005, 2006a,b,c). This was the first description of evolution in reverse in
human beings, referred to “Unertan Syndrome.” The results suggested that the
gene defective in these individuals might be the ancestor-gene responsible for
the transition from quadrupedality to bipedality during human evolution. This
hypothesis is consistent with the reverse evolution in mice. Accordingly, Tvrdik
and Capecchi (2006) have inserted the Hoxb1 autoregulatory enhancer into the
Hoxa1 promotor in mice and found that the newly generated Hoxa1 gene can
deliver the functionality of both paralogs in these mice. Thus, an ancestral gene
existed nearly 500 million years ago could be reconstructed experimentally.
A similar genetic mechanism including a single-gene influence on brain (see
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Wahlsten, 1999) might have caused an ancestral pattern in the individuals

exhibiting the “Unertan Syndrome,” and in the half-ape, half-human human
being presented in the present work.

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