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lüll MR imaging and localized proton MR spectroscopy in late infantile neuronal ceroid lipofuscinosis Seitz D; Grodd W; Schwab A; Seeger U; Klose U; Nagele TAJNR Am J Neuroradiol 1998[Aug]; 19 (7): 1373-7PURPOSE: Late juvenile neuronal ceroid lipofuscinosis (NCL) is a lysosomal neurodegenerative disorder caused by the accumulation of lipopigment in neurons. Our purpose was to characterize the MR imaging and spectroscopic findings in three children with late infantile NCL. METHODS: Three children with late infantile NCL and three age-matched control subjects were examined by MR imaging and by localized MR spectroscopy using echo times of 135 and 5. Normalized peak integral values were calculated for N-acetylaspartate (NAA), choline, creatine, myo-inositol, and glutamate/glutamine. RESULTS: MR imaging revealed volume loss of the CNS, most prominently in the cerebellum. The T2-weighted images showed a hypointense thalamus and hyperintense periventricular white matter. Proton MR spectra revealed progressive changes, with a reduction of NAA and an increase of myo-inositol and glutamate/glutamine. In long-standing late infantile NCL, myo-inositol became the most prominent resonance. Lactate was not detectable. CONCLUSION: MR imaging in combination with proton MR spectroscopy can facilitate the diagnosis of late infantile NCL and help to differentiate NCL from other neurometabolic disorders, such as mitochondrial or peroxisomal encephalopathies.|*Magnetic Resonance Imaging[MESH]|*Magnetic Resonance Spectroscopy[MESH]|Aspartic Acid/analogs & derivatives/analysis[MESH]|Brain Diseases/diagnosis[MESH]|Brain/metabolism/pathology[MESH]|Case-Control Studies[MESH]|Cerebellum/metabolism/pathology[MESH]|Cerebral Ventricles/metabolism/pathology[MESH]|Child[MESH]|Child, Preschool[MESH]|Choline/analysis[MESH]|Creatine/analysis[MESH]|Diagnosis, Differential[MESH]|Female[MESH]|Glutamic Acid/analysis[MESH]|Glutamine/analysis[MESH]|Humans[MESH]|Inositol/analysis[MESH]|Lactates/analysis[MESH]|Male[MESH]|Mitochondrial Encephalomyopathies/diagnosis[MESH]|Neuronal Ceroid-Lipofuscinoses/*diagnosis/metabolism/pathology[MESH]|Peroxisomal Disorders/diagnosis[MESH]|Thalamus/metabolism/pathology[MESH] |