Developmental Neuropathology
Chapter 27.2: Disorders of Carbohydrate Metabolism: Polyglucosan Disorders
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Accumulation of polyglucosan bodies as intracellular extralysosomal carbohydrate-containing filament aggregates in various tissues in the body.

Lafora disease - Lafora bodies in nerve cells
Polyglucosan neuropathy - in axons
Polyglucosan body myopathy - in skeletal muscle
Polyglucosan body disease - in astrocytes
Type IV and VII glycogenosis - in astrocytes

Polyglucosan disorders can affect one organ or multiorgan systems.

Synonyms

Epidemiology

Genetics

Clinical Features

Macroscopy

Histopathology

Lafora bodies seen in apocrine glands in a skin biopsy:

Lafora bodies in the cortex of a dog with myoclonic epilepsy:


Immunohistochemistry and Ultrastructural Findings

Lafora bodies (temporal lobectomy)


Biochemistry

Pathogenesis



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