Abstract：
:Classic maple syrup disease can be managed to allow a benign neonatal course, normal growth, and low hospitalization rates. The majority of affected infants that are prospectively managed have good neurodevelopmental outcome; however, acute metabolic intoxication and neurologic deterioration can develop rapidly at any age. Each episode is associated with a risk for cerebral edema, cerebrovascular compromise, and brain herniation. High plasma leucine and, possibly, alpha-ketoisocaproate are the principal neurotoxins in maple syrup disease. Plasma levels rise rapidly in association with net protein catabolism provoked by common infections and injuries. Transient periods of maple syrup disease encephalopathy appear fully reversible, leaving no clinically detectable neurologic sequelae. In contrast, prolonged amino acid imbalance, particularly if occurring during the critical period of brain development, leads to neuronal hypoplasia, a paucity of synapses, and undermyelination. Stagnated maturation and inadequate nutritional maintenance of brain structure have lifelong neurologic and behavioral consequences. Core elements of effective long-term therapy include screening and identification of asymptomatic newborns, frequent plasma amino acid monitoring, careful attention to branched-chain amino acid nurtriture, prevention of cerebral essential amino acid deficiencies, adequate provision of essential omega-3 class fatty acids and micronutrients deficient in commercial formulas, methods for home monitoring of metabolic control, and a commitment to lifelong therapy. Recognizing the risk for acute leucine intoxication depends on anticipating effects of common childhood infection and physiologic stresses on whole body protein turnover. Successful management of metabolic decompensation is based on the use of home sick-day regimens, rapid availability of branched-chain amino acid-free hyperalimentation solutions for hospitalized children, prevention of hyponatremia in patients with leucinosis, and frequent adjustments of intravenous therapies guided by plasma amino acid levels and indices of metabolic and clinical response.
journal_name
Curr Treat Options Neuroljournal_title
Current treatment options in neurologyauthors
Strauss KA,Morton DHdoi
10.1007/s11940-003-0039-3subject
Has Abstractpub_date
2003-07-01 00:00:00pages
329-341issue
4eissn
1092-8480issn
1534-3138journal_volume
5pub_type
杂志文章abstract：:Vision disturbances following traumatic brain injury (TBI) include anomalies of accommodation, version, vergence (nonstrabismic, as well as strabismic), photosensitivity, visual field integrity, and ocular health. Traumatic brain injury patients with complex diplopia patterns, noncomitant strabismic anomalies, and adv...
journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-002-0027-z
更新日期：2002-07-01 00:00:00
abstract：OPINION STATEMENT:Infantile spasms (IS) represent a major therapeutic challenge, as cessation of spasms and normalization of the electroencephalogram (elimination of hypsarrhythmia) are mandatory to prevent cognitive deterioration in previously healthy infants, or to preserve neurocognitive function among those neurolo...
journal_title：Current treatment options in neurology
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journal_title：Current treatment options in neurology
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-006-0012-z
更新日期：2006-05-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章,评审
doi：10.1007/s11940-019-0604-z
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-002-0011-7
更新日期：2002-11-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章,评审
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更新日期：2019-01-23 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章,评审
doi：10.1007/s11940-017-0468-z
更新日期：2017-09-01 00:00:00
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journal_title：Current treatment options in neurology
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更新日期：2004-01-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
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更新日期：1999-11-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
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更新日期：2012-04-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-015-0370-5
更新日期：2015-09-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-003-0028-6
更新日期：2003-09-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-007-0016-3
更新日期：2007-07-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
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更新日期：2011-08-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-008-0044-7
更新日期：2008-11-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-012-0168-7
更新日期：2012-04-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-015-0373-2
更新日期：2015-09-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
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更新日期：2004-09-01 00:00:00
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journal_title：Current treatment options in neurology
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：
更新日期：2007-09-01 00:00:00
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journal_title：Current treatment options in neurology
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更新日期：2010-05-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-010-0077-6
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-012-0177-6
更新日期：2012-08-01 00:00:00
abstract：OPINION STATEMENT:Myotonic dystrophy (DM1) is the most common form of adult muscular dystrophy. It is a multisystem disorder with a complex pathophysiology. Although inheritance is autosomal dominant, disease variability is attributed to anticipation, a maternal expansion bias, variable penetrance, somatic mosaicism, a...
journal_title：Current treatment options in neurology
pub_type： 杂志文章,评审
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-008-0009-x
更新日期：2008-03-01 00:00:00
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journal_title：Current treatment options in neurology
pub_type： 杂志文章
doi：10.1007/s11940-000-0034-x
更新日期：2000-11-01 00:00:00
abstract：OPINION STATEMENT:Paraneoplastic brain stem encephalitis can occur as an isolated clinical syndrome or, more often, may be part of a more widespread encephalitis. Different antineuronal autoantibodies, such as anti-Hu, anti-Ri, and anti-Ma2 can be associated with the syndrome, and the most frequent tumors are lung and ...
journal_title：Current treatment options in neurology
pub_type： 杂志文章
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