title: Long-term diffusion impairment of cerebral white matter in a degenerative disease of the central and peripheral nervous system: reflection of chronic excitotoxicity?
abstract: The authors report an abnormal prolonged restricted magnetic resonance imaging (MRI) proton diffusion that persisted for more than 2 years in a 6.5-year-old boy with a progressive neurological disease characterized by developmental retardation, peripheral polyneuropathy, and bilateral optical nerve atrophy. The long-term restricted magnetic resonance imaging proton diffusion observed in diffusion-weighted magnetic resonance images indicates chronic metabolic tissue impairment in the affected white matter, whereas measurable lactate accumulation in proton magnetic resonance spectroscopy was absent, and no respiratory complex abnormality was found in muscle tissue. These findings are suggestive of a chronically disturbed regulation of energy supply triggering a "slow onset" excitotoxicity, causing chronic hypoxia and leading to slow cell death as has been postulated in certain neurodegenerative processes.
authors.affiliation: Institute of Diagnostic and Interventional Neuroradiology, Hannover Medical School, Hannover, Germany. blum.katrin@mh-hannover.de
authors.forename: Katrin Sabine
authors.initials: KS
authors.lastname: Blum
authors.forename: Christian
authors.initials: C
authors.lastname: Hagel
authors.forename: Eva
authors.initials: E
authors.lastname: Neuen-Jacob
authors.forename: Peter
authors.initials: P
authors.lastname: Herkenrath
authors.forename: Jens
authors.initials: J
authors.lastname: Fiehler
authors.forename: Alfried
authors.initials: A
authors.lastname: Kohlschütter
authors.forename: Heinrich
authors.initials: H
authors.lastname: Lanfermann
authors.forename: Xiao-Qi
authors.initials: XQ
authors.lastname: Ding
mesh_terms: D001921:Brain / Q000378:metabolism / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D006801:Humans; D008297:Male; D009123:Muscle Hypotonia / Q000378:metabolism / Q000473:pathology*; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology*; D009413:Nerve Fibers, Myelinated / Q000378:metabolism / Q000473:pathology*; D019636:Neurodegenerative Diseases / Q000378:metabolism / Q000473:pathology*