--- abstract: "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." altloc: [] chapter: ~ commentary: ~ commref: ~ confdates: ~ conference: ~ confloc: ~ contact_email: ~ creators_id: - unertan37@yahoo.com creators_name: - family: Tan given: Uner honourific: 'Prof. Dr. ' lineage: '' date: 2010-09-17 date_type: published datestamp: 2010-10-18 11:04:33 department: ~ dir: disk0/00/00/70/35 edit_lock_since: ~ edit_lock_until: 0 edit_lock_user: ~ editors_id: - 33098227240 editors_name: - family: 'Tan ' given: Uner honourific: Prof.Dr. lineage: '' eprint_status: archive eprintid: 7035 fileinfo: /style/images/fileicons/application_pdf.png;/7035/1/Article_WMC00645CAIQO8QE.pdf full_text_status: public importid: ~ institution: ~ isbn: ~ ispublished: pub issn: ~ item_issues_comment: [] item_issues_count: 0 item_issues_description: [] item_issues_id: [] item_issues_reported_by: [] item_issues_resolved_by: [] item_issues_status: [] item_issues_timestamp: [] item_issues_type: [] keywords: 'Uner Tan syndrome, locomotion, quadrupedalism, speech, ataxia, self-organization' lastmod: 2011-03-11 08:57:45 latitude: ~ longitude: ~ metadata_visibility: show note: ~ number: 9 pagerange: 1-11 pubdom: TRUE publication: WebmedCentral NEUROLOGY:WMC00645 publisher: WebmedCentral refereed: TRUE referencetext: "1. 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The concept of phylogenetic regression.\r\nJ Am Acad Psychoanal 1978; 6:5-35." relation_type: [] relation_uri: [] reportno: ~ rev_number: 19 series: ~ source: ~ status_changed: 2010-10-18 11:04:33 subjects: - neuro-neu succeeds: ~ suggestions: ~ sword_depositor: ~ sword_slug: ~ thesistype: ~ title: "Two New Cases of Uner Tan Syndrome: One Man\r\nwith Transition from Quadrupedalism to Bipedalism;\r\nOne Man with Consistent Quadrupedalism" type: journalp userid: 4979 volume: 1