<> "The repository administrator has not yet configured an RDF license."^^ . <> . . "Two New Cases of Uner Tan Syndrome: One Man\r\nwith Transition from Quadrupedalism to Bipedalism;\r\nOne Man with Consistent Quadrupedalism"^^ . "Background: Uner Tan syndrome, first described in\r\n2005, consists of three main symptoms: habitual\r\nlocomotion on all four extremities, impaired\r\nintelligence, and dysarthric or no speech. This\r\nextremely rare syndrome shows an autosomal\r\nrecessive inheritance due to consanguineous\r\nmarriages between parents. In general, there is a\r\ncerebellovermial hypoplasia with a mild gyral\r\nsimplification in the cerebral cortex. Truncal ataxia is\r\nthe main neurological finding, causing difficulty in\r\nstanding and upright walking on two legs.\r\nMethods: As soon as the new cases came to light, the\r\nfamily was visited. After taking the basic information\r\nabout the family members, the traditional neurological\r\nexaminations were performed, MRI scans of the\r\npatients and of a healthy family member were taken in\r\na hospital nearest to the residence (a small village\r\nnear Kars, Turkey). The patients were also filmed to\r\nanalyze their walking patterns.\r\nResults: Two individuals (case 1, 44y; case 2, 43y)\r\namong six siblings from a family with consanguineous\r\nparents exhibited Uner Tan syndrome, with\r\nquadrupedalism, impaired intelligence, and dysarthric\r\nspeech. Their pedigree suggested an autosomal\r\nrecessive inheritance. MRI scans showed inferior\r\ncerebellovermial and pontobulbar hypoplasia.\r\nCase 1 did not display hypotonia, while case 2 had\r\nbeen hypotonic for two years after birth. Case 2’s\r\nhypotonia progressively disappeared, and at three\r\nyears old he started to sit, and could walk on all fours\r\nby the age of four. Case 1 was quadrupedal for 20\r\nyears, and then walked upright with the aid of a\r\nwalking stick. Tendon reflexes were normal in case 1\r\nbut hyperactive in the lower extremities of case 2;\r\nBabinsky was absent in case 1 but bilaterally present\r\nin case 2. There was no nystagmus, no strabismus,\r\nand no pes pedus in either case. Cognition was\r\nseverely impaired in both.\r\nConclusion: The emergence of quadrupedalism\r\nduring development, with a transition into bipedalism\r\nin case 1, and the emergence of the quadrupedalism\r\nafter a full hypotonia and no locomotion in case 2,\r\nwere considered as examples of the processes of\r\nadaptive self-organization, from the viewpoint of\r\ndynamic systems theory."^^ . "2010-09-17" . . "1" . "9" . . "WebmedCentral"^^ . . . "WebmedCentral NEUROLOGY:WMC00645"^^ . . . . . . . . . . . "Uner"^^ . "Tan "^^ . "Uner Tan "^^ . . "Uner"^^ . "Tan"^^ . "Uner Tan"^^ . . . . . . "Two New Cases of Uner Tan Syndrome: One Man\r\nwith Transition from Quadrupedalism to Bipedalism;\r\nOne Man with Consistent Quadrupedalism (PDF)"^^ . . . . . . . . . . . . "Article_WMC00645CAIQO8QE.pdf"^^ . . . "Two New Cases of Uner Tan Syndrome: One Man\r\nwith Transition from Quadrupedalism to Bipedalism;\r\nOne Man with Consistent Quadrupedalism (Image (JPEG))"^^ . . . . . . "preview.jpg"^^ . . . "Two New Cases of Uner Tan Syndrome: One Man\r\nwith Transition from Quadrupedalism to Bipedalism;\r\nOne Man with Consistent Quadrupedalism (Indexer Terms)"^^ . . . . . . "indexcodes.txt"^^ . . "HTML Summary of #7035 \n\nTwo New Cases of Uner Tan Syndrome: One Man \nwith Transition from Quadrupedalism to Bipedalism; \nOne Man with Consistent Quadrupedalism\n\n" . "text/html" . . . "Neurology" . .