Adult polyglucosan body disease (APBD): management

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Adult polyglucosan body disease (APBD): management. Polyglucosan bodies are PAS-positive round intra-axonal inclusions.

Pathology

  • Polyglucosan bodies are PAS-positive round intra-axonal inclusions
  • They contain amylopectin-like polysaccharide
  • They are found in myelinated fibers, skin, and muscle
  • The inclusions may be seen in normal older people

Magnetic resonance imaging (MRI): features

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Other investigations

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Treatment

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Synonyms

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References

  1. Mochel F, Schiffman R, Steenweg ME, et al. Adult polyglucosan body disease: natural history and key magnetic resonance imaging findings. Ann Neurol 2012; 72:433-441.
  2. Robertson NP, Wharton S, Anderson J, Scolding NJ. Adult polyglucosan body disease associated with an extrapyramidal syndrome. JNNP 1998; 65:788-790.
  3. Sindern E, Ziemssen F, Ziemssen T, et al. Adult polyglucosan body disease: a postmortem correlation study. Neurology 2003; 61:263-265. 
  4. Mochel F, Schiffmann R, Steenweg ME, et al. Adult polyglucosan body disease: natural history and key magnetic resonance imaging findings. Ann Neurol 2012; 72:433-441.
  5. Busard HL, Gabreëls-Festen AA, Renier WO, et al. Adult polyglucosan body disease: the diagnostic value of axilla skin biopsy. Ann Neurol 1991; 29:448-451.
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