Effect of topiramate on enlargement of head in Canavan disease: A new option for treatment of megalencephaly

Turkish Journal of Pediatrics

Volumn 46, Issue 1, 2004, Pages 67-71

  Topçu, Meral ^a   Yalnizoǧlu, Dilek ^a   Saatçi, Işil ^a   Haliloǧlu, Göknur ^a   Topaloǧlu, Haluk ^a   Şenbil, Nesrin ^b   Önol, Saniye ^a   Coşkun, Turgay ^a  

^a HACETTEPE UNIVERSITY   (Turkey)

^b DR SAMI ULUS CHILDREN S HOSPITAL   (Turkey)

Author keywords

Canavan disease topiramate; Leukoencephalopathy; Megalencephaly

Indexed keywords

ANTICONVULSIVE AGENT; TOPIRAMATE;

ALEXANDER DISEASE; ARTICLE; AUTOSOMAL RECESSIVE DISORDER; BRAIN DEGENERATION; CANAVAN DISEASE; CASE REPORT; CHILDHOOD DISEASE; CLINICAL FEATURE; DEGENERATIVE DISEASE; DISEASE COURSE; DRUG EFFECT; FEMALE; FOLLOW UP; HEAD CIRCUMFERENCE; HUMAN; LEUKOENCEPHALOPATHY; MALE; MYELIN SHEATH; NERVE FIBER; NEUROLOGIC DISEASE; PRESCHOOL CHILD; SEIZURE; SPASTICITY; SYMPTOMATOLOGY;

ANTICONVULSANTS; CANAVAN DISEASE; FEMALE; FOLLOW-UP STUDIES; FRUCTOSE; HUMANS; INFANT; MAGNETIC RESONANCE IMAGING; MALE; TREATMENT OUTCOME;

EID: 1542500954     PISSN: 00414301     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (13)

1. Baslow MH, Resnik TR. Canavan disease: Analysis of the nature of the metabolic lesions responsible for development of the observed clinical symptoms. J Mol Neurosci 1997; 9: 109-126.
2. Baslow MH. Canavan's spongiform leukodystrophy: A clinical anatomy of a genetic metabolic CNS disease. J Mol Neurosci 2000; 15: 61-69.
3. Matalon R, Matalon KM. Biochemistry and molecular biology of Canavan disease. Neurochem Res 1999; 24: 507-513.
4. Matalon R, Matalon KM. Canavan disease prenatal diagnosis and genetic counseling. Obstet Gynecol Clin North Am 2002; 29: 297-304.
5. Fink DJ. Gene therapy for Canavan disease? Ann Neurol 2000; 48: 9-10.
6. Leone P, Janson CG, Blaniuk L, et al. Aspartoacylase gene transfer to the mammalian central nervous system with therapeutic implications for Canavan disease. Ann Neurol 2000; 48: 27-38.
7. Baslow MH, Suckow RF, Hungund BL. Effects of ethanol and alcohol dehydrogenase inhibitors on the reduction of N-acetylaspartate levels of brain in mice in vivo: A search for substances that may have therapeutic value in the treatment of Canavan disease. J Inherit Metab Dis 2000; 23: 684-692.
8. Glauser TA. Topiramate. Epilepsia 1999, 40 (Suppl): S71-S80.
9. Matalon R, Michals K, Sebesta D, Deanching M, Gashhoff P, Casanova J. Aspartoacylase deficiency and N-acetyl aspartic aciduria in patients with Canavan disease. Am J Med Genet 1988; 29: 463-471.
10. Baslow MH. Molecular water pumps and the aetiology of Canavan disease: A case of sorcerer's apprentice. J Inherit Metab Dis 1999; 22: 99-101.
11. Nyhan WL, Ozand PT. Atlas Metabolic Diseases. London: Chapman and Hall Medical; 1998: 637-639.
12. Matalon R, Michals-Matalon K. Spongy degeneration of the brain, Canavan disease: Biochemical and molecular findings. Front Biosci 2000; 5: D307-311.
13. Kitada K, Akimitsu T, Shigematsu Y, et al. Accumulation of N-acetyl-L-aspartate in the brain of the tremor rat, a mutant exhibiting absence-like seizure and spongioform degeneration in the central nervous system. J Neurochem 2000; 74: 2512-2519.