Naslov
Cystic leukoencephalopathy in a megalencephalic child: clinical and magnetic resonance imaging/magnetic resonance spectroscopy findings

Autori
Mejaški-Bošnjak, Vlatka ; Bešenski, Nada ; Brockmann, Knut ; Pouwels, Petra JW ; Frahm, J ; Hanefeld, Folker A

Izvornik
Pediatric Neurology (0887-8994) 16 (1997), 4; 347-350

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
Leukoencphalopathy; Megalencephaly; Magnetic resonance imaging; Magnetic resonance spectroscopy

Sažetak
A neurodegenerative disorder chaacterized by megalencephaly since early infancy and slowly progressive symptoms of cerebellar, pyramidal, and extrapyramidal dysfunction, pseudobulbar signs, and epilepsy was detected in an 8-year-old girl with severe neuromotor handicap but preservation of mental and sensory functions. Cranial computed tomography and magnetic resonance imaging revealed brain swelling as well as severe abnormalities of frontal, temporal, and parietal white matter, with an extended cystlike apperance isointense to cerebrospinal fluid. Localized proton magnetic resonance spectroscopy of affected cystic white matter showed a loss of all metabolites, in accordance with a complete disintegration of neuroaxonal and glial tissue. This case is likely a severe variant of a recently described megalencephalic leukoencephalopathy with swelling and discrepantly mild clinical course.

Izvorni jezik
Engleski

POVEZANOST RADA