Abstract
We report clinical and neurophysiological findings in six children (three female, three male) with type I lissencephaly and three children (all female) with type II lissencephaly (Walker-Warburg syndrome). In type I lissencephaly the diagnosis is based only on electroencephalographic (EEG) signs, whereas in type II lissencephaly the diagnosis rests on clinical signs. In type I lissencephaly the EEG typically shows high α-β activity, which is not seen in type II lissencephaly.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Aicardy J (1989) The lissencephaly syndromes. Int Pediatr 4:118–126
Barkovich AJ, Koch THK, Carrol CL (1991) The spectrum of lissencephaly: report of ten patients analysed by magnetic resonance imaging. Ann Neurol 30:139–146
Barth PG (1987) Disorders of neuronal migration. Can J Neurol Sci 14:1–16
Barth PG (1992) Migrational disorders of the brain. Curr Opin Neurol Neurosurg 5:339–343
Bordarir C, Aicardi J, Gouttieres F (1984) Congenital hydrocephalus and eye abnormalities with severe developmental brain defects: Warburg's syndrome. Ann Neurol 16:60–65
Damska M, Wisnierski K, Sher J (1983) Lissencephaly: two distinct clinopathological types. Brain Dev 5:302–310
De Rijk-van Andel JF, Artts WFM, Barth PG, Loonan MCB (1990) Diagnostic features and clinical signs of 21 patients with lissencephaly type I. Dev Med Child Neurol 32:707–717
Dieker H, Edwards RH, ZuRhain G, Chou SM, Opitz JM (1969) The lissencephaly syndrome. Birth Defects 5:53–64
Dobyns WB (1987) Developmental aspects of lissencephaly and the lissencephaly syndromes. Birth Defects 23:225–241
Dobyns WB, Stratton RF, Greenberg F (1984) Syndromes with lissencephaly. I. Miller-Dieker and Norman-Roberts syndromes and isolated lissencephaly. Am J Med Genet 18:509–526
Dobyns WB, Pagon RA, Armstrong D, Curry CJR, Greenberg F, Grix A, Holmes LB, Laxova R, Michels VV, Robinow M, Zimmerman RL (1989) Diagnostic criteria for Walker-Warburg syndrome. Am J Med Genet 32:195–210
Ferris GS, Happel LT, Duncan MC (1977) Cerebral cortical isolation in infantile neuroaxonal dystrophy. Electroencephalogr Clin Neurophysiol 43:168–182
Gastaut H, Pindard N, Raybaud C, Aicardi J, Zifkin B (1987) Lissencephaly (agyria-pachygyria): clinical findings and serial EEG studies. Dev Med Child Neurol 29:167–180
Grant S, Heel RC (1991) Vigabatrin. A review of its pharmacodynamic and pharmacokinetic properties, and therapeutic potential in epilepsy and disorders of motor control. Drugs 41:889–926
Hakamada S, Watanabe K, Hara K, Miyazaki S (1979) The evolution of electroencephalographic features in lissencephaly syndrome. Brain Dev 1:277–283
Jones KL, Gilbert EF, Kaveggia EG, Opitz JM (1980) The Miller-Dieker syndrome. Pediatrics 66:277–281
Kimura S, Kobayashi T, Sasaki Y, Hara M, Nishino T, Miyake S, Iwamoto H, Misugi N (1992) Congenital polyneuropathy in Walker-Warburg syndrome. Neuropediatrics 23:14–17
Leyten QH, Gabreels FJM, Renir WO, Ter Lack HJ, Sengers RCA, Mullaart RA (1989) Congenital muscular dystrophy. J Pediatr 115:214–221
Oostra BA, De Rijk-van Andel JF, Eyssen HJ, Helley DJJ van, Niermeijer MF (1991) DNA analysis in patients with lissencephaly type I and other cortical dysplasia. Am J Med Genet 40:383–386
Schuierer G, Kurlemann G, Lengerke H-J v (1993) Neuroimaging in lissencephalies. Child's Nerv Syst 9:391–393
Warburg M (1978) Hydrocephaly, congenital retinal nonattachment and congenital falciform fold. Am J Ophthalmol 85:88–94
Williams RS, Swisher CN, Jennings M, Ambler M, Caviness VS (1984) Cerebro-ocular dysgenesis (Walker-Warburg syndrome): neuropathologic and etiologic analysis. Neurology 34:1531–1541
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kurlemann, G., Schuierer, G., Kuchelmeister, K. et al. Lissencephaly syndromes: clinical aspects. Child's Nerv Syst 9, 380–386 (1993). https://doi.org/10.1007/BF00306188
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00306188