Abstract
Recently, a disorder characterized by childhood ataxia and diffuse cerebral white matter abnormalities has been described in detail by Hanefeld et al. (1993) and Schiffmann et al. (1992, 1994). Apart from progressive cerebellar ataxia, seizures and optic atrophy were noted. Cognitive functions were relatively preserved. MRI showed diffuse high signal intensity of the hemispheral white matter on T2-weighted images.
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References
Hanefeld F, Holzbach U, Kruse B, Wilichowski E, Christen HJ, Frahm J (1993) Diffuse white matter disease in three children: an encephalopathy with unique features on magnetic resonance imaging and proton magnetic resonance spectroscopy. Neuropediatr 24: 244–248
Henkes HE, Deutman AF, Busch HFM (1972) Behr disease. In: Vinken PG, Bruyn GW (eds) Handbook of clinical neurology, vol 13. North Holland, Amsterdam, pp 88–93
Horoupian DS, Zucker DK, Moshe S, Peterson HDC (1979) Behr syndrome: a clinicopathologic report. Neurology 29: 323–327
Landrigan PJ, Berenberg W, Bresnan M (1973) Behrs syndrome: familial optic atrophy, spastic diplegia and ataxia. Dev Med Child Neurol 15: 41–47
Marzan K, Barron TF (1994) MRI abnormalities in Behr syndrome. Pediatr Neurol 10: 247–248
Monaco F, Pirisi A, Sechi GP, Mutani R (1979) Complicated optic atrophy ( Behrs disease) associated with epilepsy and amino acid imbalance. Eur Neurol 18: 101–105
Schiffmann R, Trapp BD, Moller JR, Kaye EM, Parker CC, Brady RO, Barton NW (1992) Childhood ataxia with diffuse central nervous system hypomyelination. Ann Neurol 32: 484
Schiffmann R, Moller JR, Trapp BD, Shih HHL, Farrer RG, Katz DA, Alger JR, Parker CC, Hauer PE, Kaneski CR, Heiss JD, Kaye EM, Quarles RH, Brady RO, Barton NW (1994) Childhood ataxia with diffuse central nervous system hypomyelination. Ann Neurol 35: 331–340
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© 1995 Springer-Verlag Berlin Heidelberg
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van der Knaap, M.S., Valk, J. (1995). Childhood Ataxia with Diffuse Cerebral Hypomyelination. In: Magnetic Resonance of Myelin, Myelination, and Myelin Disorders. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-03078-3_49
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DOI: https://doi.org/10.1007/978-3-662-03078-3_49
Publisher Name: Springer, Berlin, Heidelberg
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