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Unilateral Cerebellar Hypoplasia

  • On the basis of MRI findings, cerebellar malformations can be divided into those associated with hypoplasia and those with dysplasia; each type can show either focal or diffuse malformations.
  • Focal cerebellar hypoplasia can be further subdivided into isolated vermis hypoplasia or hypoplasia of one of the cerebellar hemispheres.
  • Pathologic evidence of cerebellar injury due to birth asphyxia is well described. Because of its high metabolic activity, the vermis is the structure that is most commonly involved.
  • The clear demonstration of cerebellar hypoplasia associated with hypoplasia or aplasia of the cerebellar or vertebral arteries favors the concept of an intrauterine vascular etiology for cerebellar hypoplasia/aplasia. Genetic mutations with somatic mosaicism may also have a role to play.
  • Also, as in our case, unilateral cerebellar hypoplasia may be an incidental finding in a patient with no previous evidence of neuromuscular or metabolic disease and no past history of trauma or anoxia.
  • Cases with unilateral cerebellar hypoplasia can present with ataxia, grand mal seizures, persistent headache, or psychomotor retardation without cerebellar symptomatology.
  • Key Diagnostic Features: vermian or cerebellar atrophy
  • Causes of unilateral cerebellar atrophy: cerebellitis, unilateral ischemic infarction, unilateral traumatic brain injury/concussion, posterior fossa surgery, unexplained origin
November 19, 2012
A 5-year-old boy with frequent falls and speech abnormalities. Significant past history of low birth weight and neonatal distress. On examination, the boy had ataxic gait, slurred speech, and plantar extensor reflex with exaggerated deep tendon reflexes on left side.
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