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Spinal cord MRI in adult polyglucosan body disease

C Negishi1, G Sze

  • 1Department of Diagnostic Radiology, Yale University, School of Medicine, New Haven, CN 06510.

Journal of Computer Assisted Tomography
|September 1, 1992
PubMed

Insights

Adult polyglucosan body disease can cause severe spinal cord atrophy. MRI may show atrophy without signal changes, confirmed by autopsy findings of corpora amylacea.

Area of Science:

  • Neurology
  • Radiology
  • Pathology

Background:

  • Adult polyglucosan body disease (APBD) is a rare genetic disorder.
  • It typically presents with progressive neurological deficits.

Observation:

  • A 67-year-old male patient exhibited a chronic, progressive neurological decline.
  • Magnetic Resonance Imaging (MRI) revealed significant atrophy of the entire spinal cord.
  • Notably, no signal abnormalities were detected on long TR (TR) images.

Findings:

  • Autopsy confirmed severe spinal cord atrophy.
  • Histopathology demonstrated diffuse gliosis.
  • Numerous corpora amylacea (polyglucosan bodies) were identified.

Implications:

  • This case highlights the MRI appearance of APBD, characterized by atrophy without signal changes.
  • It underscores the importance of correlating imaging findings with autopsy in diagnosing rare neurological disorders.
  • Understanding the MRI characteristics aids in the diagnosis and management of APBD.

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