NEURONAL MIGRATION DISORDERS
 
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NEURONAL MIGRATION DISORDERS

 

DEFINITION:

A group of disorders characterized by the abnormal migration of neurons during embryogenesis resulting in structurally abnormal cerebral hemispheres.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • infancy to childhood
  • risk factors:
    • familial - autosomal recessive and dominant, x-linked
    • acquired - due to an in utero insult to the developing brain, i.e., CMV, intraventricular hemorrhage, hypoxic ischemic encephalopathy, PVL

PATHOGENESIS:

1. Background

  • the Neuronal Migration Disorders represent a group of disorders where metabolic, chromosomal, hypoxic-ischemic, and/or toxic environmental factors result in a process where there is a general or topical loss of control over neuronal migration after closing of the neural tube and formation of the telencephalic vesicles
  • this loss of control results in either cell death or improper positioning of functional cell groups -> failed or improper neuronal connections -> functional neurologic deficiencies
  • clinical variability is due to the various etiologies, sites affected, and gestational age at the time of the insult

2. Disorders

  • Schizencephaly
  • Porencephaly
  • Lissencephaly (Agyria)
  • Macrogyria (Pachygyria)
  • Microgyria (Micropolygyria)
  • Neuronal Heterotopias
  • Agenesis of the Corpus Callosum
  • Agenesis of the Cranial Nerves

3. Associated Anomalies

  • the Neuronal Migration Disorders may be associated with:

    1. Other Anomalies

    • induction disorders (myelomeningocele)
    • proliferation disorders (microcephaly)
    • other neuronal migration disorders

    2. Syndromes

    • Meckel-Gruber Syndrome
    • Miller-Dieker Syndrome
    • Neonatal Adrenoleukodystrophy
    • Walker-Warburg Syndrome
    • Zellweger's Syndrome

CLINICAL FEATURES:

1. Neurological Manifestations

  • infantile hypotonia
  • seizures
  • developmental delay
  • mental retardation
  • microcephaly
  • motor dysfunction
    • hypotonia, spasticity, and/or hemiparesis

INVESTIGATIONS:

1. Imaging Studies

1. CT/MRI

  • abnormal structural appearance of the cerebral hemispheres
  • a migration disorder may appear in isolation or with other disorders of induction, proliferation, or migration

MANAGEMENT:

1. Supportive

  • a multidisciplinary approach involving:
    • Paediatrics, Neurology, PT, OT
    • seizure control
    • supplemental and/or special education

 

 

 

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