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MRI and clinical syndrome in dura mater-related Creutzfeldt-Jakob disease

B Meissner1, K Kallenberg, P Sanchez-Juan

  • 1National TSE Reference Center, Dept. of Neurology, University of Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany. epicjd@med.uni-goettingen.de

Journal of Neurology
|January 23, 2009
PubMed
Abstract

Insights

Iatrogenic Creutzfeldt-Jakob disease (iCJD) from dura mater grafts has a long incubation period. MRI scans reveal characteristic brain signal changes, primarily in the cortex and basal ganglia, similar to sporadic CJD.

Area of Science:

  • Neurology
  • Prion Diseases
  • Neuroimaging

Background:

  • Iatrogenic Creutzfeldt-Jakob disease (iCJD) is primarily linked to dura mater grafts and human growth hormone treatments.
  • Limited data exists on the clinical progression of dura mater-associated CJD (DM-CJD).

Purpose of the Study:

  • To describe the clinical and diagnostic features of DM-CJD patients.
  • To emphasize MRI signal alterations in DM-CJD.

Main Methods:

  • Clinical symptoms and diagnostic findings were assessed in ten DM-CJD patients.
  • MRI scans were analyzed for signal increases in cortical and subcortical brain structures.

Main Results:

  • DM-CJD patients had a median incubation of 18 years and disease duration of 7 months.
  • Most patients were methionine homozygous at codon 129 of the prion protein gene (PRNP) and presented with ataxia and psychiatric symptoms.
  • MRI revealed cortical and basal ganglia signal increases in 8/10 patients, and thalamic hyperintensity in 5/10, with thalamic changes correlating with methionine homozygosity.

Conclusions:

  • MRI findings in DM-CJD are largely consistent with those observed in sporadic CJD.
  • The cortex and basal ganglia are the primary brain regions affected in DM-CJD.

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