
sclerosing leukoensephalopathy ulla poolisistik lipomembranus austiodisplaasiya

sclerosing leukoensephalopathy ulla poolisistik lipomembranus austiodisplaasiya ennum ariyappedunna nasu-hackola rogam neratheyulla demensia multifocal boonn sisttukalum ulla oru apoorva rogamaanu. finnish, japanese janasamkhyayil ettavum kooduthal kaanapedunna "trem 2" allengil "tyrobe" jeenil function mutationukalude autosomal resessive nashtam moolamaanu ithu sambhavikkunnath.
adayaalangalum lakshanangalum.
eee avasthayil naalu gattangal thirichariyappedunnu. aadya ghattam (olinjirikkunna ghattam) lakshanangalo adayaalangalo kanikkunnilla. eee ghattam saadhaaranayaayi 20-kalude thudakkam vare neendunilkkum. ithine thudarnnu oossiyas ghattam varunnu. kaikaalukalude neelamulla ellukale saadhaaranayaayi badhikkunna aavarthichulla asthi vedanayaanu ithinte savisheshatha. ithine thudarnnu saadhaaranayaayi eee asthikalude rogalakshanaparamaaya odivukal sambhavikkunnu. moonnaam ghattam (aadyakaala neurological) adayaalappeduthunnathu oormma nashtathodoppam oru frondall loob syndrominte (euphoria, eakaagrathayude abhavam, vidhinirnnayathinte nashtam, saamoohika thadassangal) lakshanangalude thudakkamaanu. apasmaram undaayekkaam. eee ghattam saadhaaranayaayi 20 kalude avasaanathilum 30 kalude thudakkathilumaanu aarambhikkunnath. avasana ghattatthinte savisheshatha kadinamaaya demensia pakshaakhaathavumaanu. maranam saadhaaranayaayi 40kalude avasaanathilo 50kalude thudakkathilo sambhavikkunnu.
janithakashasthram.
tyro protein tyrosine kainas binding protein ("tyrobe") jeenileyum myelooid cellukal 2 ("trem 2") jeenil prakadippikkunna triggering resepterileyum mutationukalumaayi eee avastha bandhappettirikkunnu. "tyrobe" chromosome 19 (19q13.12) nte neelamulla kaiyilum "trem 2" chromosome 6 (6p21.1) nte cheria kaiyilumanu sthithi cheiyunnathu. 
pathopiciology.
ithu manassilaakunnillenkilum microglia ulppedunnuvennu thonnunnu.
roganirnayam.
klinikkal adisthaanathil eee syndrome samshayikkappedaam. tyrobe", "trem 2" jeenukal krameekarichaanu roganirnayam sthaapikkunnathu.
anweshanangal.
ex-reey asthisisttukaludeyum austioporosisukaludeyum saannidhyam kaanikkunnu. thalachorinte sii. tii allengil emm. aarr. aii. thalachorinte frontotemporal lobukalil tissue nashtappedunnathu kaanikkunnu. basel gangliayude calcification saadhaaranamaanu. eeg saadhaaranayaayi thudakkathil saadhaaranamaanu, pakshe pinneedu mandagathiyilaakkukayum prakopippikkukayum cheiyunnu.
chikitsa.
eee avasthaykku prathyeka chikilsayilla. maanejment pinthunaykkunnu.
epidemiology.
saahithyathil vivarichirikkunna ~ 200 kesukal ulla eee avastha apoorvamaayi kanakkaakkappedunnu. finsil janasamkhyayude vyaapanam 2 x 10−6 aanennu kanakkaakkappedunnu.
charithram.
1973laanu eee avastha aadyamaayi vivarichathu.

