DEGENERESCENCE SPONGIEUSE FAMILIALE DU SYSTEME NERVEUX CENTRAL
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| Title | DEGENERESCENCE SPONGIEUSE FAMILIALE DU SYSTEME NERVEUX CENTRAL |
| Authors | |
| Abstract | A case o f fami l ial spongy degenerat ion o f cent r a l nervaus sys tem i s repor ted . Cl inical manifestation ,hi s topathologi c changes and pat hogene sis have been disc us sed . The patie nt 15 a ´) months o:d baby "´ho deve:oped nervous disorde r s as hypotonia and sucking di fficu lty short :y a f t e r birth . From t he second month he had hypert oni c and s pas t ic attacks and fina:ly di ~d in r espiratoy dis t r ess . The baby ´s parents are c l os e re lative s and t heir fi - rst chi l d had developed s i mi l ar c linical s ymptoms and di - e d at 4 months of age .Microscopical l y , pathologic change s i nclude spong i osis o f the enti re whi t e matte r o f t he central nervous system associated with diffuse gliosi s Al zheime r t ype 11 . Re cent ultrastructural and biochemical studies have demonst rated unique fe ature that are consist ent ,with t he accumulati on of excessive f l uid within t he as t rocyti c c ytoplasm and myelin l ame l l ae. |
| Publisher | Tehran University of Medical Sciences |
| Date | 2012-10-20 |
| Source | Acta Medica Iranica Vol 23, No 3-4 (1981) |