The Ulaş family of nineteen from rural southern Turkey has been the primary example of the proposed syndrome. Tan described five members as walking with a quadrupedal gait using their feet and the palms of their hands. In infants, where this is a rare but a normal stage prior and sometimes following bipedal walking, such a gait is called “bear crawl”. The affected family members are also severely mentally retarded and their speech is affected. Tan proposed that these are symptoms of Uner Tan syndrome.
In January 2008, Tan reported on another family (four males and two females) located in southern Turkey.
Four other unrelated cases in families are described as having various degrees of UTS. Males may be affected more than females. It is also claimed that some individuals are unaware of time, lack language, have severe mental retardation with no conscious experience, and communicate by using sounds. Two males are unable to stand up, while in other cases, can stand up but cannot make a step when standing. Less severe cases use toe walking, which is a normal phase in child gait development.
Uner Tan syndrome has been linked to intrafamilial marriage and reproduction, which suggests that it is an autosomal recessive disorder.The main characteristic of this syndrome is habitual quadrupedalism, meaning they can stand up straight until they try to move, then they walk on their hands and knees. According to Tan, the syndrome may be placed in its own category under types of cerebellar ataxias. This simply means it is a type of disorder that involves the cerebellum becoming inflamed, resulting in lack of control of voluntary movements. Uner Tan syndrome falls into this category because it has similar symptoms to other cerebellar ataxia disorders such as Disequilibrium Syndrome (DES-H) and Cayman Syndrome. These symptoms include dysarthria, nystagmus, and hypoplasia of the cerebellum and vermis.
By – Nursing Tutor – Ms. Aradhana George
Department of Nursing
College Of Nursing UCBMSH
Uttaranchal (P.G.) College Of Bio-Medical Sciences & Hospital