Çocuk Sağlığı ve Hastalıkları Dergisi
2013 , Vol 56 , Num 3
Demyelination mechanisms in subacute sclerosing panencephalitis (SSPE): an experimental study
Hacettepe Üniversitesi Tıp Fakültesi 1Pediatri Uzmanı, 2Pediatri Yardımcı Doçenti, 3Pediatri Profesörü
*İletişim: serbeken@gmail.com
Subacute sclerosing panencephalitis (SSPE) is a chronic disease caused by the
measles virus. Although its pathogenesis is unclear, the brain tissue shows
inflammatory infiltration, neuronal loss, gliosis, and demyelination. The latter
can be due to endogenous metabolic toxins, oxidative damage, and antibodyor
complement- mediated myelin breakdown. In this study, the presence of
demyelinating soluble substances (cytokine, antibody, complement) in the
cerebrospinal fluid (CSF) of SSPE patients was investigated experimentally
by injection of CSF samples from patients into mice. Adult Swiss albino mice
received an intraperitoneal injection of 0.5 ml CSF from three SSPE patients
and three control patients for two consecutive days. To disrupt the blood brain
barrier, pertussis toxin was given intraperitoneally before the CSF injections.
A total of 14 mice were followed for 6 days (n=7) or 21 days (n=7). Daily
examinations were done for tail tone, gait, and paralysis. After the followup
period, the brain and sciatic nerves were studied histopathologically
(hematoxylin-eosin, myelin stainings and TUNEL method) for demyelination
and apoptosis. None of the mice developed clinical signs during the follow-up.
Histopathological examination revealed no findings of neuronal degeneration,
inflammation or demyelination. Rare apoptotic cells were observed in all
groups. Sciatic nerves revealed no difference in morphology, myelin thickness
or myelin morphology between groups. These results provided no evidence
supporting the presence of soluble substances causing demyelination in the
CSF. Demyelination might be caused by oligodendrocyte death during disease
progression or by other inflammatory mediators released at later phases of
the disease in SSPE.
Anahtar Kelimeler :
SSPE, kızamık, demiyelinizasyon, oligodendrosit, histopatoloji.