Northern epilepsy syndrome (NES, CLN8) — MRI and electrophysiological studies
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Cited by (9)
Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways of neuronal ceroid lipofuscinosis 8 mice
2012, Neurobiology of DiseaseCitation Excerpt :However, the data from the milder form of CLN8-related NCL disease, EPMR correlates poorly with our results from the Cln8mnd mouse model. EPMR patients did not show alterations in somatosensory SEF measurements, nor was thalamic hypointensity detected via MRI (Lauronen et al., 2001). Although glial activation coincided with the neuron loss in EPMR autopsy material, it was restricted to the hippocampal subfields CA2/CA3 and has not been explored in detail in other brain regions (Herva et al., 2000; Tyynela et al., 2004).
Ocular Manifestations of Neuronal Ceroid Lipofuscinoses
2021, Seminars in OphthalmologyExpanding the neuroimaging phenotype of neuronal ceroid lipofuscinoses
2020, American Journal of NeuroradiologyStandardized assessment of seizures in patients with juvenile neuronal ceroid lipofuscinosis
2015, Developmental Medicine and Child NeurologyMetabolic, toxic, and inflammatory brain disorders
2012, Pediatric Neuroimaging: Fifth EditionThalami and corona radiata in juvenile NCL (CLN3): A voxel-based morphometric study
2007, European Journal of Neurology
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