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THE CHIARI MALFORMATIONS
  1. Donald M Hadley
  1. Correspondence to:
 Professor Donald M Hadley, Department of Neuroradiology, Institute of Neurological Sciences, Southern General Hospital, 1345 Govan Road, Glasgow UK;
 gota27{at}udcf.gla.ac.uk

https://doi.org/10.1136/jnnp.72.suppl_2.ii38

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    Dr Hans Chiari1 first described three hindbrain disorders associated with hydrocephalus in 1891. They have neither an anatomical nor embryological correlation with each other, but they all involve the cerebellum and spinal cord and are thought to belong to the group of abnormalities that result from failure of normal dorsal induction. These include neural tube defects, cephaloceles, and spinal dysraphic abnormalities. Symptoms range from headache, sensory changes, vertigo, limb weakness, ataxia and imbalance to hearing loss. Only those with a type I Chiari malformation may be born grossly normal. The abnormalities are best shown on midline sagittal T1 weighted magnetic resonance imaging (MRI),2 but suspicious features on routine axial computed tomographic brain scans (an abnormal IVth ventricle, a “full” foramen magnum, and absent cisterna magna) should be recognised and followed up with MRI.

    CHIARI I

    This is the mildest of the hindbrain malformations and is characterised by displacement of deformed cerebellar tonsils more than 5 mm caudally through the foramen magnum.3 The brainstem and IVth ventricle retain a relatively normal position although the IVth ventricle may be small and slightly distorted (fig 1). A number of subgroups have …

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