engleski

Cystic megalencephalic leukoencephalopathy - clinical, MRI/MRS and EEG findings

Van der Knaap et al. (1995) described recently a new entity of heredodegenerative white matter disease, based on clinical and morphological criteria as visualized by MRI. We report od 5 further cases of this entity. Three out of 5 patients have pregressive neuromotor disorders and suffer from epilepsy, demonstrating distinctive EEG findings with slow high-voltage background activity combined with spikes and spike-wave complexes, as well as marked photoparoxysmal response of generalized discharges of spikes, polispikes and waves. These three children have extensive lesions to the cerebral white matter on MRI, particulary the huge subcortical cysts frontally and temporally. Localized proton MRS in these patients revealed complete loss of all brain metabolities within altered white matter and cysts. In contrast, the other two children follow the milder clinical course and morphology, showing MR spectra with only decrease of creatine.

Megalencephalic leukoencephalopathy; Magnetic Resonance Imaging; Magnetic Resonance Spectroscopy

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