Acute axonal neuropathy in maple syrup urine disease

Muscle and Nerve

Volumn 24, Issue 2, 2001, Pages 284-287

  Kleopa, Kleopas A ^a   Raizen, David M ^a   Friedrich, Christopher A ^b   Brown, Mark J ^a   Bird, Shawn J ^a  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b UNIVERSITY OF PENNSYLVANIA   (United States)

Author keywords

Branched chain amino acids; Maple syrup urine disease; Wallerian degeneration

Indexed keywords

ADULT; ARTICLE; CASE REPORT; DECARBOXYLATION; DIET RESTRICTION; DIETARY INTAKE; ELECTRODIAGNOSIS; FEMALE; HUMAN; MAPLE SYRUP URINE DISEASE; METABOLIC REGULATION; MUSCLE WEAKNESS; NERVE BIOPSY; NERVE FIBER TRANSPORT; PERIPHERAL NEUROPATHY; POLYNEUROPATHY; PRIORITY JOURNAL; SURVIVAL;

ACUTE DISEASE; ADULT; AXONS; ELECTROPHYSIOLOGY; FEMALE; HUMANS; MAPLE SYRUP URINE DISEASE; MUSCLE WEAKNESS; NEURAL CONDUCTION; SURAL NERVE; WALLERIAN DEGENERATION;

EID: 0035140484     PISSN: 0148639X     EISSN: None     Source Type: Journal    
DOI: 10.1002/1097-4598(200102)24:2<284::AID-MUS140>3.0.CO;2-U     Document Type: Article

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4. Further observations on the biochemical lesion in maple syrup urine disease
5. An acute axonal form of Guillain-Barré polyneuropathy
6. Severe axonal degeneration in acute Guillain-Barré syndrome: Evidence of two different mechanisms?
7. Acute motor axonal neuropathy: An antibody-mediated attack on axolemma
8. Abnormal dendritic development in maple syrup urine disease
9. Maple syrup disease: Isolation and identification of organic acids in the urine
10. A new syndrome: Progressive familial infantile cerebral dysfunction associated with an unusual urinary substance
11. Is demyelination a feature of maple syrup urine disease?
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14. Maple syrup urine disease with particular reference to dietotherapy
15. Biochemical mechanisms underlying primary degeneration of axons
16. Metal neuropathy
17. Effect of α-ketoisocaproate and leucine on the in vivo oxidation of glutamate and glutamine in the rat brain
18. Elevation of amino acids in interstitial space of the rat brain following infusion of large neutral amino and keto acids by microdialysis: Leucine infusion