Cerebellar agenesis

Cerebellar agenesis is a rare congenital abnormality which can result from failure to develop normal cerebellar tissue or destruction of normally developed tissue.

For a more general overview of cerebellar malformations, please refer to the article on classification systems for malformations of the cerebellum.

Terminology

Typically small remnants of the cerebellum are present, including cerebellar peduncles and vermian lobules, and as such the term subtotal cerebellar agenesis is often used. Cerebellar agenesis or hypoplasia has previously been referred to as Chiari IV malformation, however this term is now considered obsolete 1, 2

Clinical presentation

While one might expect these individuals to mainly show motor coordination difficulties, the plasticity of the brain is such that motor issues, although present, are not necessarily as severe as in patients with cerebellar lesions acquired after birth. Patients with cerebellar agenesis do however display varying degrees of cognitive impairment in memory, behavior, language and social functioning, and often autism or autism-like behavior is encountered.         

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Article information

rID: 30205
Section: Anatomy
Synonyms or Alternate Spellings:
  • Agenesis of the cerebellum
  • Subtotal cerebellar agenesis

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Cases and figures

  • Case 1: axial
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  • Case 1: coronal
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