?url_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Adc&rft.title=Two+New+Cases+of+Uner+Tan+Syndrome%3A+One+Man%0D%0Awith+Transition+from+Quadrupedalism+to+Bipedalism%3B%0D%0AOne+Man+with+Consistent+Quadrupedalism&rft.creator=Tan%2C+Prof.+Dr.++Uner&rft.subject=Neurology&rft.description=Background%3A+Uner+Tan+syndrome%2C+first+described+in%0D%0A2005%2C+consists+of+three+main+symptoms%3A+habitual%0D%0Alocomotion+on+all+four+extremities%2C+impaired%0D%0Aintelligence%2C+and+dysarthric+or+no+speech.+This%0D%0Aextremely+rare+syndrome+shows+an+autosomal%0D%0Arecessive+inheritance+due+to+consanguineous%0D%0Amarriages+between+parents.+In+general%2C+there+is+a%0D%0Acerebellovermial+hypoplasia+with+a+mild+gyral%0D%0Asimplification+in+the+cerebral+cortex.+Truncal+ataxia+is%0D%0Athe+main+neurological+finding%2C+causing+difficulty+in%0D%0Astanding+and+upright+walking+on+two+legs.%0D%0AMethods%3A+As+soon+as+the+new+cases+came+to+light%2C+the%0D%0Afamily+was+visited.+After+taking+the+basic+information%0D%0Aabout+the+family+members%2C+the+traditional+neurological%0D%0Aexaminations+were+performed%2C+MRI+scans+of+the%0D%0Apatients+and+of+a+healthy+family+member+were+taken+in%0D%0Aa+hospital+nearest+to+the+residence+(a+small+village%0D%0Anear+Kars%2C+Turkey).+The+patients+were+also+filmed+to%0D%0Aanalyze+their+walking+patterns.%0D%0AResults%3A+Two+individuals+(case+1%2C+44y%3B+case+2%2C+43y)%0D%0Aamong+six+siblings+from+a+family+with+consanguineous%0D%0Aparents+exhibited+Uner+Tan+syndrome%2C+with%0D%0Aquadrupedalism%2C+impaired+intelligence%2C+and+dysarthric%0D%0Aspeech.+Their+pedigree+suggested+an+autosomal%0D%0Arecessive+inheritance.+MRI+scans+showed+inferior%0D%0Acerebellovermial+and+pontobulbar+hypoplasia.%0D%0ACase+1+did+not+display+hypotonia%2C+while+case+2+had%0D%0Abeen+hypotonic+for+two+years+after+birth.+Case+2%E2%80%99s%0D%0Ahypotonia+progressively+disappeared%2C+and+at+three%0D%0Ayears+old+he+started+to+sit%2C+and+could+walk+on+all+fours%0D%0Aby+the+age+of+four.+Case+1+was+quadrupedal+for+20%0D%0Ayears%2C+and+then+walked+upright+with+the+aid+of+a%0D%0Awalking+stick.+Tendon+reflexes+were+normal+in+case+1%0D%0Abut+hyperactive+in+the+lower+extremities+of+case+2%3B%0D%0ABabinsky+was+absent+in+case+1+but+bilaterally+present%0D%0Ain+case+2.+There+was+no+nystagmus%2C+no+strabismus%2C%0D%0Aand+no+pes+pedus+in+either+case.+Cognition+was%0D%0Aseverely+impaired+in+both.%0D%0AConclusion%3A+The+emergence+of+quadrupedalism%0D%0Aduring+development%2C+with+a+transition+into+bipedalism%0D%0Ain+case+1%2C+and+the+emergence+of+the+quadrupedalism%0D%0Aafter+a+full+hypotonia+and+no+locomotion+in+case+2%2C%0D%0Awere+considered+as+examples+of+the+processes+of%0D%0Aadaptive+self-organization%2C+from+the+viewpoint+of%0D%0Adynamic+systems+theory.&rft.publisher=WebmedCentral&rft.contributor=Tan+%2C+Prof.Dr.+Uner&rft.date=2010-09-17&rft.type=Journal+(Paginated)&rft.type=PeerReviewed&rft.format=application%2Fpdf&rft.identifier=http%3A%2F%2Fcogprints.org%2F7035%2F1%2FArticle_WMC00645CAIQO8QE.pdf&rft.identifier=++Tan%2C+Prof.+Dr.+Uner++(2010)+Two+New+Cases+of+Uner+Tan+Syndrome%3A+One+Man+with+Transition+from+Quadrupedalism+to+Bipedalism%3B+One+Man+with+Consistent+Quadrupedalism.++%5BJournal+(Paginated)%5D+++++&rft.relation=http%3A%2F%2Fcogprints.org%2F7035%2F