MYELINATION PATTERNS ON MAGNETIC RESONANCE OF CHILDREN WITH DEVELOPMENTAL DELAY

Magnetic resonance (MR) imaging was performed in 30 children with unexplained developmental delay who had associated neurological abnormalities such as seizures, spasticity, hypotonia, ataxia or poor vision. No child had a history of regression, preterm birth or neonatal cerebral injury. CT scans were performed before MR in all cases and were either normal or showed only mild atrophy. At least two MR sequences were obtained for all patients. Nine children had delayed or absent myelination on MR, one had patchy white‐matter abnormalities, and in one patient myelination was topographically normal, but of inappropriately low signal intensity. MR was abnormal in six of seven children who had abnormal brainstem auditory evoked potentials (BAEP), and was normal in nine of 11 patients who had a normal BAEP. MR may have a useful rôle in demonstrating abnormal white‐matter maturation in children with unexplained neurodevelopmental delay, particularly when abnormalities are found on BAEP studies.

[1]  A. Krumholz,et al.  Maturation of the brain-stem auditory evoked potential in premature infants. , 1985, Electroencephalography and clinical neurophysiology.

[2]  A. Zimmerman,et al.  Connatal Pelizaeus-Merzbacher disease: an autosomal recessive form. , 1987, Pediatric neurology.

[3]  H I Goldberg,et al.  Immature brain: spin-echo pulse sequence parameters for high-contrast MR imaging. , 1987, Radiology.

[4]  E. Brett,et al.  Value of computerised tomography in children with non-specific mental subnormally. , 1982, Archives of disease in childhood.

[5]  A. Riela,et al.  Magnetic Resonance Imaging in Pediatric Neurologic Disorders , 1987, Journal of child neurology.

[6]  I. Awad,et al.  Incidental lesions noted on magnetic resonance imaging of the brain: prevalence and clinical significance in various age groups. , 1987, Neurosurgery.

[7]  R. Worth,et al.  Brain stem auditory, visual and somatosensory evoked potentials in leukodystrophies. , 1982, Electroencephalography and clinical neurophysiology.

[8]  G. McKhann Metachromatic Leukodystrophy: Clinical and Enzymatic Parameters , 1984, Neuropediatrics.

[9]  T. H. Newton,et al.  MRI of normal brain maturation. , 1986, AJNR. American journal of neuroradiology.

[10]  B. Brody,et al.  Sequence of Central Nervous System Myelination in Human Infancy. I. An Autopsy Study of Myelination , 1987, Journal of neuropathology and experimental neurology.

[11]  A. Koeppen,et al.  Defective biosynthesis of proteolipid protein in pelizaeus‐merzbacher disease , 1987, Annals of neurology.

[12]  R E Steiner,et al.  Periventricular leukomalacia: evaluation with MR imaging. , 1986, Radiology.

[13]  B. Jones,et al.  Triphalangeal thumbs associated with hypoplastic anemia. , 1973, Pediatrics.

[14]  R. Grossman,et al.  MR imaging of white matter disease in children. , 1988, AJR. American journal of roentgenology.

[15]  M. Osbakken,et al.  Magnetic resonance imaging in leukodystrophies of childhood. , 1985, Pediatric neurology.

[16]  M. Baraitser,et al.  Microcephaly, mental retardation, cataracts, and hypogonadism in sibs: Martsolf's syndrome. , 1989, Journal of medical genetics.

[17]  R. Curless Computed tomography of GM1 gangliosidosis. , 1984, The Journal of pediatrics.

[18]  G. Bydder,et al.  Serial MR imaging in neonatal cerebral injury. , 1987, AJNR. American journal of neuroradiology.

[19]  J. Aicardi,et al.  Pelizaeus-Merzbacher Disease: Clinical and Nosological Study , 1986, Journal of child neurology.

[20]  D. Norman,et al.  Normal maturation of the neonatal and infant brain: MR imaging at 1.5 T. , 1988, Radiology.

[21]  P. Kaplan,et al.  Leukoencephalopathy among native indian infants in northern Quebec and Manitoba , 1988, Annals of neurology.

[22]  R. Galamboš,et al.  Brain stem auditory evoked responses in human infants and adults. , 1974, Archives of otolaryngology.

[23]  C. McArdle,et al.  Abnormalities of the neonatal brain: MR imaging. Part II. Hypoxic-ischemic brain injury. , 1987, Radiology.

[24]  M. Ehrlich,et al.  MRI of the central nervous system in neonates and young children. , 1986, AJNR. American journal of neuroradiology.

[25]  N. Soroker,et al.  Magnetic resonance imaging in head injured patients with normal late computed tomography scans. , 1987, Surgical neurology.

[26]  K. Tsuchiya,et al.  Magnetic resonance imaging in Pelizaeus-Merzbacher disease. , 1988, Pediatric neurology.

[27]  C. McArdle,et al.  Developmental features of the neonatal brain: MR imaging. Part I. Gray-white matter differentiation and myelination. , 1987, Radiology.

[28]  O. Markand,et al.  Usefulness of BAER studies in the early diagnosis if Pelizaeus‐Merzbacher disease , 1983, Neurology.

[29]  McKhann Gm Metachromatic leukodystrophy: clinical and enzymatic parameters. , 1984 .