Naslov
Beneficial effect of oral creatine intake in the case of "Vacuolating megalencephalopathy with subcortical cysts"

Autori
Mejaški-Bošnjak, Vlatka ; Fumić, Ksenija ; Bešenski, Nada ; Hanefeld, Folker ; Wilken B ; Frahm Jens

Vrsta, podvrsta i kategorija rada
Radovi u zbornicima skupova, cjeloviti rad (in extenso), znanstveni

Izvornik
3rd European Paediatric Neurology Society / Evrard, Philippe ; Richelme, Christian ; Tardieu, Marc - Bolonja : Monduzzi Editore, 1999, 29-32

Skup
3rd European Paediatric Neurology Society Congress

Mjesto i datum
Nica, Francuska, 07.11.1999. - 10.11.1999

Vrsta sudjelovanja
Predavanje

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
Creatine; Megalencephalic leukoencephalopathy

Sažetak
We report on 7 years old boy with "Vacuolating megalencephalopathy with subcortical cysts" (van der Knaap 1995) who recived oral creatine 2g twice a day for two years. The boy had clinical, neurophychiological and MRI/MRS assessment at age of 5 yrs, showing at neurological examination mild ataxia, brisk tendon reflexes with ankle clonus, swallowing problems and dysarthric speech, as well as marked macrocephaly. Proton MRS examination showed reduced spectra of N-acetyl aspartic acid and creatine within altered white matter indicating axonal pathology and loss. Afterwords oral intake of creatine was started and continued onwards. At the age of 7 years the boy underwent MRI/MRS reexamination showing ameliorate findings which was in accordance with his intellectual prosgress and regression of some deviant neurological signs as well as stabilization of head growth.

Izvorni jezik
Engleski

POVEZANOST RADA