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We studied 30 patients with juvenile neuronal ceroid lipofuscinosis (JNCL). The patients (aged 6-25 years) and 43 age-matched healthy volunteers underwent MRI. After visual assessment, the signal intensity was measured on T2-weighted images in numerous locations. The thickness of the cortex and corpus callosum and the dimensions of the brain stem were(More)
NCL disorders are progressive brain diseases with an autosomal recessive inheritance in all eleven childhood types. These occure world-wide but may be enriched in some countries. In Finland altogether about 400 patients have been diagnosed during the last forty years. The most common types are the infantile and classic juvenile forms with an incidence of 1:(More)
We investigated cerebral processing of audiovisual speech stimuli in humans using functional magnetic resonance imaging (fMRI). Ten healthy volunteers were scanned with a 'clustered volume acquisition' paradigm at 3 T during observation of phonetically matching (e.g., visual and acoustic /y/) and conflicting (e.g., visual /a/ and acoustic /y/) audiovisual(More)
Hemodynamic activity in occipital, temporal, and parietal cortical areas were recently shown to correlate across subjects during viewing of a 30-minute movie clip. However, most of the frontal cortex lacked between-subject correlations. Here we presented 12 healthy naïve volunteers with the first 72 minutes of a movie ("Crash", 2005, Lions Gate Films)(More)
Six patients with a variant type of Jansky-Bielschowsky (JBVD) disease were examined using 3 different imaging methods. Five of the patients underwent computed tomography, 4 magnetic resonance imaging, and 5 single photon emission computed tomography. All patients had brain atrophy that was most severe in the cerebellum. Magnetic resonance imaging(More)
The purpose of this study was to demonstrate the course of infantile neuronal ceroid-lipofuscinosis with brain magnetic resonance imaging (MRI) in children aged 3 months to 11 years. Twenty-one patients and 46 neurologically normal controls of the same age were examined. The images were evaluated visually; then signal intensities were measured and related(More)
For the majority of families affected by one of the neuronal ceroid lipofuscinoses (NCLs), a biochemical and/or genetic diagnosis can be achieved. In an individual case this information not only increases understanding of the condition but also may influence treatment choices and options. The presenting clinical features prompt initial investigation and(More)
We presented phonetically matching and conflicting audiovisual vowels to 10 dyslexic and 10 fluent-reading young adults during "clustered volume acquisition" functional magnetic resonance imaging (fMRI) at 3 T. We further assessed co-variation between the dyslexic readers' phonological processing abilities, as indexed by neuropsychological test scores, and(More)
Our purpose was to develop a method of measuring the size of the brain stem by routine MRI and to determine brain stem dimensions in a normal population. We examined 174 subjects, aged 4 months to 86 years, with no known brain disease. Sagittal midline diameters of the mesencephalon, pons and medulla oblongata were measured on sagittal T1-weighted images,(More)
Clinical features, magnetic resonance imaging and visual evoked potentials were analysed and correlated in 20 Finnish patients with muscle-eye-brain disease. Significantly enhanced visual evoked potentials were found in 15 patients (giant in 14 of them). Magnetic resonance images were available in 17 cases. The images of 12 patients with giant visual evoked(More)