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Normal pressure hydrocephalus in myotonic dystrophy.

P B Christensen1

  • 1Department of Neurology, Aalborg Hospital, Denmark.

European Neurology
|January 1, 1988
PubMed
Summary

Normal pressure hydrocephalus (NPH) may be a late complication of myotonic dystrophy (MD). This study suggests NPH in elderly MD patients could stem from the progressive ventricular enlargement seen in the disease.

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Area of Science:

  • Neurology
  • Genetics
  • Neurosurgery

Background:

  • Myotonic dystrophy (MD) is a progressive genetic disorder.
  • Normal pressure hydrocephalus (NPH) is a neurological condition characterized by enlarged ventricles.

Observation:

  • Two elderly siblings with myotonic dystrophy presented with symptoms of NPH.
  • Cerebral imaging (CT scan) and infusion tests confirmed NPH in these siblings.
  • Eleven younger family members with MD were evaluated; two showed cerebral ventricular enlargement on CT scans, despite lacking neurological symptoms.

Findings:

  • The study observed a potential link between myotonic dystrophy and normal pressure hydrocephalus.
  • Progressive cerebral ventricular enlargement was noted in some MD patients, suggesting a possible underlying mechanism for NPH.
  • This indicates that NPH might be a late-onset complication in individuals with myotonic dystrophy.

Implications:

  • These findings suggest that NPH should be considered in the differential diagnosis of elderly patients with myotonic dystrophy.
  • Further research is warranted to elucidate the specific mechanisms connecting MD and NPH.
  • This could lead to earlier diagnosis and improved management strategies for MD patients at risk of developing NPH.

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