Images suggesting normal pressure hydrocephalus are frequently found in DM1

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Japanese researchers analyzed brain MRI scans of 112 patients with DM1 and compared them to 50 brain MRI scans of controls. From the study of the z-Evans index, a recently developed parameter, they conclude that: 

  • dilation of the ventricles and sylvian valleys is frequent in DM1, even at a presymptomatic stage; 
  • these images are comparable to those of hydrocephalus at normal pressure;
  • their importance does not correlate with the number of CTG triplets or the gender;
  • dilation occurs preferentially in elderly patients.

Further neuropsychological studies may be able to distinguish between the consequences of these dynamic disturbances of the CSF and the evolution of DM1.

 

Cerebral ventriculomegaly in myotonic dystrophy type 1: normal pressure hydrocephalus-like appearances on magnetic resonance imaging. Iida S, Seino H, Nagahata F, et al. BMC Neurosci. 2021 Oct 18;22(1):62.

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