Changes in the auditory nerve brainstem evoked responses in a case of maple syrup urine disease

Developmental Medicine and Child Neurology

Volumn 46, Issue 3, 2004, Pages 184-186

  Geal Dor, Miriam ^a   Adelman, Cahtia ^a   Levi, Haya ^a   Goitein, Kalman ^a   Sohmer, Haim ^b  

^a HADASSAH HEBREW UNIVERSITY MEDICAL CENTER   (Israel)

^b HEBREW UNIVERSITY   (Israel)

Author keywords

[No Author keywords available]

Indexed keywords

BRANCHED CHAIN AMINO ACID;

ACUTE DISEASE; ARTICLE; BIRTH WEIGHT; BRAIN EDEMA; BRAIN STEM; CASE REPORT; COCHLEAR NERVE; DIALYSIS; DIET THERAPY; EVOKED BRAIN STEM AUDITORY RESPONSE; FEMALE; FOLLOW UP; GESTATIONAL AGE; HEMISPHERIC DOMINANCE; HUMAN; MAPLE SYRUP URINE DISEASE; NEUROLOGIC EXAMINATION; NEWBORN; PATHOPHYSIOLOGY; PHYSIOLOGY; PRIORITY JOURNAL; RESPIRATORY FAILURE;

ACUTE DISEASE; BRAIN EDEMA; BRAIN STEM; COCHLEAR NERVE; DOMINANCE, CEREBRAL; EVOKED POTENTIALS, AUDITORY, BRAIN STEM; FEMALE; FOLLOW-UP STUDIES; HUMANS; INFANT, NEWBORN; MAPLE SYRUP URINE DISEASE; NEUROLOGIC EXAMINATION; RESPIRATORY INSUFFICIENCY;

EID: 1342282773     PISSN: 00121622     EISSN: None     Source Type: Journal    
DOI: 10.1017/S0012162204000325     Document Type: Article

References (12)

1. Adelman C, Levi H, Linder N, Sohmer H. (1990) Neonatal auditory brain-stem response threshold and latency: 1 hour to 5 months. Electroencephalogr Clin Neurophysiol 77: 77-80.
2. American Academy of Neurology. (1995) Practice parameters for determining brain death in adults (summary statement). The Quality Standards Subcommittee of the American Academy of Neurology. Neurology 45: 1012-1014.
3. Backhouse O, Leitch RJ, Thompson D, Kriss A, Charris D, Clayton P, Russell-Eggitt I. (1999) A case of reversible blindness in maple syrup urine disease. Br J Ophthalmol 83: 250-251.
4. Brismar J, Aqeel A, Brismar G, Coates R, Gascon G, Ozand P. (1990) Maple syrup urine disease: findings on CT and MR scans of the brain in 10 infants. AJNR 11: 1219-1228.
5. Edelmann L, Wasserstein MP, Kornreich R, Sansaricq C, Snyderman SE, Diaz GA. (2001) Maple syrup urine disease: identification and carrier-frequency determination of a novel founder mutation in the Ashkenazi Jewish population. Am J Hum Genet 69: 863-868.
6. Felber SR, Sperl W, Chemelli A, Murr C, Wendel U. (1993) Maple syrup urine disease: metabolic decompensation monitored by proton magnetic resonance imaging and spectroscopy. Ann Neurol 33: 396-401.
7. Goitein KJ, Fainmesser P, Sohmer H. (1983) Cerebral perfusion pressure and auditory brain-stem responses in childhood CNS diseases. Am J Dis Child 137: 777-781.
8. Korein J, Sansaricq C, Kalmijn M, Honig J, Lange B. (1994) Maple syrup urine disease: clinical, EEG, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity. Int J Neurosci 79: 21-45.
9. Levin ML, Scheimann A, Lewis RA, Beaudet AL. (1993) Cerebral edema in maple syrup urine disease. J Pediatr 122: 167-168.
10. Sohmer H, Gafni M, Goitein K, Fainmesser P. (1983) Auditory nerve-brain stem evoked potentials in cats during manipulation of the cerebral perfusion pressure. Electroencephalogr Clin Neurophysiol 55: 198-202.
11. Sohmer H, Gafni M, Havatselet G. (1984) Persistence of auditory nerve response and absence of brain-stem response in severe cerebral ischaemia. Electroencephalogr Clin Neurophysiol 58: 65-72.
12. Taccone A, Schiaffino MC, Cerone R, Fondelli MP, Romano C. (1992) Computed tomography in maple syrup urine disease. Eur J Radiol 14: 207-212.