Abstract:
:Current evidence suggests that the effects of lithium on metabolic and signaling pathways in the brain may vary depending on the specific clinical condition or disease model. For example, lithium increases levels of cerebral N-acetyl aspartate in patients with bipolar disorder but does not appear to affect N-acetyl aspartate levels in normal human subjects. Conversely, lithium significantly decreases whole-brain levels of N-acetyl aspartate in a rat genetic model of Canavan disease in which cerebral N-acetyl aspartate is chronically elevated. While N-acetyl aspartate is a commonly used surrogate marker for neuronal density and correlates with neuronal viability, grossly elevated whole-brain levels of N-acetyl aspartate in Canavan disease are associated with dysmyelination and mental retardation. This report describes the first clinical application of lithium in a human subject with Canavan disease. Spectroscopic and clinical changes were observed over the time period in which lithium was administered, which reversed during a 2-week wash-out period after withdrawal of lithium. This investigation reports decreased N-acetyl aspartate levels in the brain regions tested and magnetic resonance spectroscopic values that are more characteristic of normal development and myelination, suggesting that a larger, controlled trial of lithium may be warranted as supportive therapy for Canavan disease by decreasing abnormally elevated N-acetyl aspartate.
journal_name
Pediatr Neuroljournal_title
Pediatric neurologyauthors
Janson CG,Assadi M,Francis J,Bilaniuk L,Shera D,Leone Pdoi
10.1016/j.pediatrneurol.2005.04.015subject
Has Abstractpub_date
2005-10-01 00:00:00pages
235-43issue
4eissn
0887-8994issn
1873-5150pii
S0887-8994(05)00257-2journal_volume
33pub_type
杂志文章abstract:BACKGROUND:Cerebrospinal fluid opening pressure is elevated with central nervous system infection and vasculitis, but has not been studied in inflammatory demyelinating disease. This retrospective study sought to determine whether children with demyelinating disease demonstrate elevated cerebrospinal fluid opening pres...
journal_title:Pediatric neurology
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abstract::Earlier reports on T2-weighted magnetic resonance imaging (MRI) in the classical form of Pelizaeus-Merzbacher disease seemed to divide the patterns of the high-intensity lesions in the white matter into three subtypes: type I, diffusely hemispheric and corticospinal; type II, diffusely hemispheric without brainstem le...
journal_title:Pediatric neurology
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doi:10.1016/s0887-8994(97)00212-9
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journal_title:Pediatric neurology
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abstract:BACKGROUND:Individuals with Duchenne muscular dystrophy have an increased risk of long bone fractures. Such fractures are sometimes associated with brain dysfunction due to fat embolism syndrome, although this syndrome has seldom been documented in muscular dystrophy patients. PATIENT DESCRIPTION:We describe a child w...
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abstract::Neurophysiologic intraoperative monitoring, using somatosensory, brainstem auditory, and visual evoked potentials, transcranial electric motor stimulation, and electromyography, is typically used during complex surgeries involving the motor and sensory cortex, brainstem, cranial nerves, spinal cord, nerve root, periph...
journal_title:Pediatric neurology
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abstract::Insufficient nutrition is known to lead to disturbances in postnatal myelin formation. This study aims to demonstrate that early myelination is altered in human twin pregnancies. Five brains of twins with a symmetric blood supply and three brains of twins with chronic fetal-fetal transfusion syndrome (one hypervolemic...
journal_title:Pediatric neurology
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更新日期:1998-10-01 00:00:00
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
pub_type: 杂志文章,评审
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
pub_type: 杂志文章
doi:10.1016/j.pediatrneurol.2015.12.016
更新日期:2016-06-01 00:00:00
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journal_title:Pediatric neurology
pub_type: 杂志文章
doi:10.1016/0887-8994(85)90010-4
更新日期:1985-01-01 00:00:00
abstract::Proteolipid protein (PLP) is the major myelin protein of the central nervous system and is widely believed to play an important structural role in maintaining the myelin compaction. We have studied the early developmental changes of PLP with immunohistochemical methods. Our data demonstrate for the first time a compar...
journal_title:Pediatric neurology
pub_type: 杂志文章
doi:10.1016/s0887-8994(97)00099-4
更新日期:1997-10-01 00:00:00
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journal_title:Pediatric neurology
pub_type: 杂志文章
doi:10.1016/j.pediatrneurol.2009.03.015
更新日期:2009-09-01 00:00:00
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journal_title:Pediatric neurology
pub_type: 杂志文章
doi:10.1016/j.pediatrneurol.2010.11.020
更新日期:2011-05-01 00:00:00
abstract:BACKGROUND:We identified a group of patients with ATP1A3 mutations at residue 756 who display a new phenotype, distinct from alternating hemiplegia of childhood, rapid-onset dystonia-parkinsonism, and cerebellar ataxia, areflexia, pes cavus, optic atrophy, sensorineural hearing loss syndromes. METHODS:Four patients wi...
journal_title:Pediatric neurology
pub_type: 杂志文章
doi:10.1016/j.pediatrneurol.2017.04.022
更新日期:2017-08-01 00:00:00
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
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journal_title:Pediatric neurology
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abstract::Tourette syndrome is a neurologic disorder characterized by both motor and vocal tics. Recently, two variants, including a single-base deletion resulting in a truncated protein and a 3'-untranslated-region variant altering a binding site for micro-RNA in the Slit and Trk-like 1 gene, were found to be a genetic cause o...
journal_title:Pediatric neurology
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