Abstract：
:Four patients with the classic form and 1 patient with the connatal form of Pelizaeus-Merzbacher disease were studied with magnetic resonance imaging, electroencephalography, and multimodal evoked potentials, including brainstem auditory evoked potentials, somatosensory evoked potentials, and visual evoked potentials. Comparisons between these findings were made. It was determined that the neurophysiologic studies, particularly brainstem auditory evoked potentials, are of value in early diagnosis of Pelizaeus-Merzbacher disease; brainstem auditory evoked potentials with only normal wave I may be a relatively reliable clue suggesting the classic form of Pelizaeus-Merzbacher disease in patients with nystagmus and chronic progressive encephalopathy. Magnetic resonance imaging allows an accurate assessment of the degree of hypomyelination; however, the clinical severity of various forms of Pelizaeus-Merzbacher disease seemed to be independent of the age of onset and the amount of residual myelin. The following may be distinguishing features between the connatal and classic forms of Pelizaeus-Merzbacher disease: hypoplasia of the cerebellum and brainstem, and diffuse brain atrophy on magnetic resonance imaging; optic atrophy with abnormal visual evoked potential; seizure disorder with abnormal electroencephalography, and/or auditory nerve impairment with abnormal wave I of brainstem auditory evoked potentials in the early stage of the disease.
journal_name
Pediatr Neuroljournal_title
Pediatric neurologyauthors
Wang PJ,Young C,Liu HM,Chang YC,Shen YZdoi
10.1016/0887-8994(94)00124-ksubject
Has Abstractpub_date
1995-01-01 00:00:00pages
47-53issue
1eissn
0887-8994issn
1873-5150pii
088789949400124Kjournal_volume
12pub_type
杂志文章abstract：:Serial examination of the cerebral hemispheres of 20 sudden infant death syndrome victims revealed high incidence of leukomalacia (40%), leptomeningeal glioneuronal heterotopias (70%) at the base of the cerebrum, and astrogliosis (65%) in the white matter and medulla reticular formation compared with 20 age-matched co...
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：:Ketoacidosis is one of the common complications of Type I insulin-dependent diabetes mellitus. Several neurologic (cerebral) deficiencies have been associated with diabetic ketoacidosis, including cerebral edema with increased intracranial pressure resulting in coma; partial and generalized seizures; and cerebrovascul...
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
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pub_type： 杂志文章,随机对照试验
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更新日期：2011-08-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章
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更新日期：2003-10-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章,评审
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更新日期：2002-01-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章
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更新日期：1997-04-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章
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更新日期：2003-09-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章
doi：10.1016/0887-8994(86)90037-8
更新日期：1986-01-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章
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更新日期：1993-11-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章
doi：10.1016/j.pediatrneurol.2013.08.016
更新日期：2014-01-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章
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更新日期：1993-05-01 00:00:00
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pub_type： 杂志文章
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journal_title：Pediatric neurology
pub_type： 杂志文章
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更新日期：1987-01-01 00:00:00
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journal_title：Pediatric neurology
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更新日期：1987-05-01 00:00:00
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pub_type： 杂志文章,多中心研究
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更新日期：2018-06-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章
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更新日期：2013-12-01 00:00:00
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更新日期：2004-04-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章
doi：10.1016/j.pediatrneurol.2007.01.009
更新日期：2007-05-01 00:00:00
abstract：:The clinical course and autopsy findings of 2 patients with measles encephalitis that occurred during the 1988-1989 Houston epidemic are reported. A previously healthy 25-month-old boy had serologically-proved measles, hemophagocytic syndrome, and acute disseminated demyelinating encephalitis. A 19-year-old male with ...
journal_title：Pediatric neurology
pub_type： 杂志文章
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更新日期：1990-03-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章,评审
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更新日期：1987-03-01 00:00:00
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journal_title：Pediatric neurology
pub_type： 杂志文章
doi：10.1016/s0887-8994(97)00011-8
更新日期：1997-04-01 00:00:00
abstract：:Although it is widely believed that herbal products are beneficial to the health, some herbal products can result in serious adverse effects, such as epileptic seizures, especially in children who are particularly susceptible. Sage oil contains well-known convulsant substances such as thujone, camphor, and cineole in ...
journal_title：Pediatric neurology
pub_type： 杂志文章
doi：10.1016/j.pediatrneurol.2011.05.012
更新日期：2011-10-01 00:00:00
abstract：:A 12-year-old girl with clinically established tuberous sclerosis complex, and without signs of neurofibromatosis type 1, developed a right retro-ocular optic nerve tumor. After rapid growth for 1 year after its discovery, the optic nerve tumor demonstrated modest progression. The patient received the mammalian target...
journal_title：Pediatric neurology
pub_type： 杂志文章
doi：10.1016/j.pediatrneurol.2010.01.016
更新日期：2010-06-01 00:00:00
