E-Pearl of the Week: Astrocyte sparing in Lafora disease

May 17, 2011

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May 17, 2011

Astrocyte sparing in Lafora disease


Lafora bodies are a pathologic hallmark of progressive myoclonic epilepsy, or Lafora disease. These inclusion bodies are accumulations of abnormal glycogen (polyglucosans) which are insoluble and precipitate in neuronal cytoplasm. This abnormal glycogen is also produced inside astrocytes but doesn't accumulate because astrocytes have glycogen phosphorylase and it is quickly degraded. Thus, Lafora bodies are seen in neurons and not in astrocytes. Lafora bodies can also be found in other glycogen storage cells such as heart, sweat glands, liver and muscles.

References

  1. Striano P, Zara F, Turnbull J, et al. Typical Progression of myoclonic epilepsy of the Lafora type. Nat Clin Pract Neurol 2008: 4; 106–111.

Submitted by:
Aamir Hussain, MD

Disclosure: Dr. Hussain has nothing to disclose.

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