Uner Tan Research Articles

UNERTAN SYNDROME: REVIEW AND REPORT OF FOUR NEW CASES

The aim of this study was to describe additional patients (n= 3) exhibiting the Unertan syndrome (UTS), resident in rural areas of Canakkale. The 8th and 9th families with the UTS, with a total of 16 members affected, have not been previously characterized. A single, non-familial case (Bayramic, Canakkale) occurred during early childhood after an infection with a high fever. Three cases of familial UTS were located in another village in Canakkale. All cases exhibited the three key symptoms of the UTS: (1) walking on all four extremities, (2) primitive language (only sounds), and (3) rudimentary intelligence. Magnetic resonance imaging showed mild vermial hypoplasia in the non-familial case, while there was cerebellar and vermial hypoplasia with a flattened cerebral cortex in the familial cases. Dexterity of the fingers was normal. The man from the latter family was fond of dragging a wooden box after fastening a string around his belt. The collective observations and clinical findings suggest two etiologies for the UTS: (1) genetic, via autosomal recessive transmission and (2) viral, likely the poliovirus affecting the cerebro-cerebellar structures. At a minimum, vermial hypoplasia is suggested to cause fully developed UTS. The subjects could stand upright and even walk bipedally, despite difficulties in balance (ataxia), but they preferred quadrupedal walking. The main difficulty with gait was maintaining a dynamic-asymmetric lateral balance and initiation of the first step during standing. Apparently, a quadrupedal gait in an adult is a developmental regression with absence of the higher control mechanisms for asymmetric lateral balance during bipedal walking. It is suggested that these individuals exhibit ancestral traits; the quadrupedal gait with rudimentary intelligence and primitive speech resulting from viral infections and/or genetic damage may cause devolution (reverse evolution), a manifestation of an experiment of nature as well as experimental studies in animal models. The results support the notion of punctuated evolution, in contrast to Darwin's theory of graded evolution. Dragging a wooden box using a string wrapped around the belt may also be an example of reverse evolution with regard to tool making, illuminating the very long-lasting period before the invention of the wheel. Herein, there has been no intent to insult or injure these individuals affected by the UTS; rather, this is an endeavor to better understand the mystery of human evolution. Go to the publisher's online edition of International Journal of Neuroscience for the following free supplemental resource: video clips.

UNERTAN SYNDROME: A CASE SERIES DEMONSTRATING HUMAN DEVOLUTION

A large family with six individuals exhibiting the Unertan syndrome (UTS) was identified residing in southern Turkey. All of the individuals had mental impairments and walked on all four extremities. The practice of intra-familial marriages suggested that the UTS may be an autosomal recessive disorder, similar to previously described cases. The inferior portions of the cerebellum and vermis were absent as evidenced by MRI and CT scans. The height and head circumference of those affected were within normal ranges. Barany's test suggested normal vestibular system function. The subjects could not name objects or their close relatives. The males (n = 4) could understand simple questions and commands, but answered questions with only one or two sounds. The females (n = 2) were superior to the males with respect to language skills and walking, suggesting an association between walking and speaking abilities. One male exhibited three walking patterns at the same time: quadripedal, tiptoe, and scissor walking. Another male used two walking styles: quadripedal and toe-walking. It is emphasized that there are important differences between the UTS and the disequilibrium syndrome. It is suggested that the inability to walk upright in those affected with the UTS may be best explained by a disturbance in lateral-balance mechanisms, without being related to the cerebello-vestibular system. An interruption of locomotor development during the transition from quadripedality to bipedality may result in habitual walking on all four extremities and is normal in some children. Because quadripedal gait is an ancestral trait, individuals with the UTS, exhibiting a manifestation of reverse evolution in humans, may be considered an experiment of nature, useful in understanding the mechanisms underlying the transition from quadripedality to bipedality during human evolution. The proposed mutant gene or gene pool playing a role in human quadrupedality may also be responsible for human bipedality at the same time. Herein there is no intent to insult or injure; rather, this report is an endeavor to better understand human beings. Supplementary materials are available for this article. Go to the publisher's online edition of International Journal of Neuroscience for the following free supplemental resource(s): video clips.

“Unertan Syndrome” in two Turkish Families in Relation to Devolution and Emergence of Homo Erectus: Neurological Examination, MRI, and pet Scans

“Unertan syndrome” consists of two main symptoms: quadrupedal gait and primitive cognitive abilities including language and conscious experience. To assess the central mechanisms involved in this syndrome, the authors performed MRI and PET scans on affected and unaffected individuals from both families. All affected individuals were also subjected to neurological examination. To assess the integrity of the peripheral and central vestibular system, Barany's caloric test was applied to the affected individuals. Brain MRI and PET scans were performed on normal subjects (n = 7) and patients (n = 7). Right- and left-cerebral and cerebellar areas, including the vermial and callosal areas, were measured on the MRI scans using a computer cursor. Quadrupedal gait, mental retardation, dysartric speech, nystagmus, severe truncal ataxia, hyperreflexia, astasia, and abasia were observed in the affected individuals from both families. Cerebellum and vermis were atrophic in the MRI and PET scans of the first family. In contrast, the brain MRI seemed to be normal in the MRI and PET scans of affected individuals from the second family. The caloric test revealed central vestibular damage in patients from the first family and peripheral vestibular damage in patients from the second family. The results suggest that “Unertan syndrome,” discovered in two unrelated families, may be caused by peripheral or central vestibular damage resulting from different genetic defects. Cerebellar hypoplasia may not be a prerequisite for the emergence of this syndrome. Primitive mental abilities may be explained by damage within the vestibulo-cerebellar system, whereas the quadrupedal gait may be due to a genetic defect within the higher brain centers that suppress the atavistic brain networks controlling quadrupedal gait and helped in the emergence of the habitual bipedal gait during human evolution. This retarded development of human locomotion—devolution—may illuminate the brain mechanisms responsible for the transition from quadrupedality to bipedality in human evolution.

A Special Case of Anencephaly in an Early-Born Baby with an Exagerated Prognastic Face: Further Example for Human Devolution

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 ). 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.

My Scientific Odyssey: Uner Tan

Professor Uner Tan was born at Unye which is at the Black Sea cost on May 1st, 1937. He graduated from secondary school at Corum and started higher education at Ege University, Faculty of Medicine, at 1956. He continued his higher education at Goettingen University and graduated from this faculty simultaneously from the neuroscience PhD program at Max-Planck Institute at 1966. He returned to Turkey at 1969 and worked at Hacettepe University (Ankara), Ataturk University (Erzurum), Black Sea Technical University (Trabzon) and Cukurova University (Adana) by timely order and he was retired at 2004. Professor Tan is a Member of Turkish Academy of Sciences, Professional Member of American Academy of Neuropsychology, and Advisory Member of World Innovation Foundation.

A New Theory on the Evolution of Human Mind. Unertan Syndrome: Quadrupedality, Primitive Language, and Severe Mental Retardation

Please see suppl. section for video, Download mpg (4483 KB) The recently discovered “UNERTAN SYNDROME” consists of quadrupedal gait, severe mental retardation, and primitive language. This syndrome can be considered as devolution of human being, throwing a light into the transition from quadrupedality to bipedality with co-evolution of human mind. The genetic nature of this syndrome supports the punctuated evolution during transition from quadrupedality to bipedality. In light of Tan’s psychomotor theory, accentuating the major role of the motor system in human mind, a new theory was suggested for the human evolution. Namely, the unique behavioral trait of man, the emergence of the habitual bipedality with Homo erectus (1.6 million and 250.000 tears ago) may be coupled with a resistive mind, which forced man to stand up against the gravitational forces with consequent success in tool making and hunting, using free hands for survival. The second stage in the evolution of modern human beings may be coupled with the emergence of language (circa 40.000 years ago), playing a major role in the origins of human mind.

A WRIST-WALKER EXHIBITING NO “UNER TAN SYDNROME”: A THEORY FOR POSSIBLE MECHANISMS OF HUMAN DEVOLUTION TOWARD THE ATAVISTIC WALKING PATTERNS

After discovering two families with handicapped children exhibiting the “Uner Tan syndrome,” the author discovered a man exhibiting only wrist-walking with no primitive mental abilities including language. According to his mother, he had an infectious disease with high fever as a three months old baby; as a result, the left leg had been paralyzed after a penicilline injection. This paralysis most probably resulted from a viral disease, possibly poliomyelitis. He is now (2006) 36 years old; the left leg is flaccid and atrophic, with no tendon reflexes; however, sensation is normal. The boy never stood up on his feet while maturing. The father forced him to walk upright using physical devices and making due exercises, but the child always rejected standing upright and walking in erect posture; he always preferred wrist-walking; he expresses that wrist-walking is much more comfortable for him than upright-walking. He is very strong now, making daily body building exercises, and walking quite fast using a “three legs,” although he cannot stand upright. Mental status, including the language and conscious experience, is quite normal. There was no intra-familiar marriage as in the two families mentioned earlier, and there is no wrist-walking in his family and relatives. There were no cerebellar signs and symptoms upon neurological examination. The brain-MRI was normal; there was no atrophy in cerebellum and vermis. It was concluded that there may be sporadic wrist-walkers exhibiting no “Uner Tan Syndrome.” The results suggest that the cerebellum has nothing to do with human wrist-walking, which may rather be an atavistic trait appearing from time to time in normal individuals, indicating a live model for human reverse evolution. It was concluded that pure quadrupeds may sporadically appear due to random fluctuations in genotypes and/or environmental factors (hormonal or nutritional); the human development following the human evolution may be stopped in the stage of transition from quadrupedality to bipedality. That is, the activity of the philogenetically youngest supraspinal centers for bipedal walking responsible for suppression of the older supraspinal centers for quadrupedal gait may be interrupted at the atavistic level due to genetic and/or environmental factors. Consequently, it is assumed that these individuals prefer their natural wrist-walking to move around more quickly and efficiently.

EVIDENCE FOR “UNER TAN SYNDROME” AS A HUMAN MODEL FOR REVERSE EVOLUTION

“Uner Tan Syndrome” was further studied in a second family. There was no cerebellar atrophy, except a mild vermial atrophy in MRI scans of the affected individuals. This is not, however, the pathogenesis of the “Uner Tan Syndrome”, since in the first and second families there were bipedal men exhibiting very similar MRI scans. The second family may also be considered a live model for reverse evolution in human beings. The present work provided evidence for a reverse evolution: (i) quadrupedality; (ii) primitive mental abilities including language; (iii) curved fingers during wrist-walking of the quadrupedal woman; (iv) arm to leg ratios being close to those of the human-like apes. The quadrupedal individuals were raised in separate places, so that they could not imitate each other, excluding the socio-cultural factors contributing to the habitual quadrupedal gait. The results are consistent with the single gene theory, suggesting a single gene controlling multiple behavioral traits, and the psychomotor theory, and a co-evolution of the human mind, an emergent property of the motor system expressed by human language.