Canavan's leukodystrophy is associated with defects in cochlear neurodevelopment and deafness

Neurology

Volumn 60, Issue 10, 2003, Pages 1702-1704

  Ishiyama, Gail ^a   Lopez, Ivan ^a   Baloh, Robert W ^a   Ishiyama, Akira ^a  

^a UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ASPARTOACYLASE;

ARTICLE; AUTOSOMAL RECESSIVE DISORDER; CANAVAN DISEASE; CASE REPORT; CORTI ORGAN; HEARING IMPAIRMENT; HISTOPATHOLOGY; HUMAN; INFANT; PERCEPTION DEAFNESS; PRIORITY JOURNAL; SPIRAL GANGLION; TEMPORAL BONE;

EID: 0038137161     PISSN: 00283878     EISSN: None     Source Type: Journal    
DOI: 10.1212/01.WNL.0000065893.60879.D3     Document Type: Article

References (10)

1. Baslow MH, Resnik TR. Canavan's disease: analysis of the nature of the metabolic lesions responsible for development of the observed clinical symptoms. J Mol Neurosci 1997;9:109-125.
2. Adachi M, Schneck L, Cara J, Volk BW. Spongy degeneration of the central nervous system (Van Bogaert and Bertrand type; Canavan's's disease). Hum Pathol 1973;4:331-347.
3. Canavan M. Schilder's encephalitis periaxialis diffusa. Report of a child aged sixteen and one-half months. Arch Neurol Psychiatry 1931;25:229.
4. Gorlin RJ, Toriello HV, Cohen MM. Hereditary hearing loss and its syndromes. New York: Oxford University Press. 1995.
5. Tsai G, Coyle JT. N-acetylaspartate in neuropsychiatric disorders. Prog Neurobiol 1995;46:531-540.
6. Knipper M, Kopschall I, Rohbock K, et al. Transient expression of NMDA receptors during rearrangement of AMPA-receptor-expressing fibers in the developing inner ear. Cell Tissue Res 1997;287:23-41.
7. Ishiyama G, Lopez I, Williamson R, Acuna D, Ishiyama A. Subcellular immunolocalization of NMDA receptor subunit NR-1, 2A, 2B in the rat vestibular periphery. Brain Res 2002;935:16-23.
8. Schuknecht HF. Pathology of the ear. Second edition. Philadelphia: Lea and Febiger, 1993.
9. Webster M, Webster DB. Spiral ganglion neuron loss following organ of Corti loss: a quantitative study. Brain Res 1981;212:17-30.
10. Yamada S, Tanaka Y, Sameshima M, Furiuchi M. Effects of starvation and feeding on tissue N-acetylhistidine levels in Nile tilapia Oreochrontis niloticus. Comp Biochem Physiol 1994;109:277-283.