MICROGYRIA
 
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MICROGYRIA

 

DEFINITION:

A disorder of neuronal migration characterized by an excess of secondary sulcation of the cerebral hemispheres resulting in a brain with a complex convolutional pattern.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • infancy to childhood
  • risk factors:
    • a circulatory disorder in utero (see below)
  • associated anomalies:
    • other migration disorders
      • grey matter heterotopias

PATHOGENESIS:

1. Background

  • an insult prior to 24 weeks gestational age does not affect primary sulcation but leads to an excess of secondary and tertiary sulcation which results in gyri that are small and numerous
  • the insult alters the circulation to the affected areas at a specific time and interferes with secondary and teritary sulcation

2. Insults

1. Infections

  • congenital CMV infection

2. Genetic or Chromosomal Disorders

  • Meckel-Gruber Syndrome
  • Bloch-Sulzberger Syndrome
  • Fukuyama's cerebromuscular dystrophy
  • Thanatophoric Dysplasia
  • Inherited Disorders of Metabolism
    • Zellweger's Syndrome
    • Neonatal adrenoleukodystrophy (NALD)

3. Toxic or Environmental

  • carbon monoxide

PATHOLOGY:

  • affected cortical areas have the appearance of 4-6 month old fetal brains and can be focal or generalized
  • two different layering abnormalities: four-layered and unlayered
  • other areas affected: decreased amount of white matter and deforma-tions of the cerebellum are also present

CLINICAL FEATURES:

1. Neurologic Manifestations

  • infantile hypotonia
  • seizure disorder
  • developmental delay
  • mental retardation
  • motor dysfunction
    • hypotonia
    • spasticity

INVESTIGATIONS:

1. Imaging Studies

1. CT/MRI

  • 1. Cerebral Cortex
    • a complex convolutional pattern
    • relatively numerous and small gyri
    • relatively numerous secondary & tertiary sulci

    • MANAGEMENT:

    1. Team Approach

    • periodic multidisciplinary follow-up
      • Paediatrics, Neurology, Ortho, OT, PT

    2. Supportive

    • anticonvulsants
    • genetic counselling

     

     

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