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Related Experiment Videos

Transient acute cortical blindness associated with hypoglycemia.

B Z Garty1, G Dinari, M Nitzan

  • 1Division of Allergy-Immunology-Pulmonary, Children's Hospital of Philadelphia, Pennsylvania 19104.

Pediatric Neurology
|May 1, 1987
PubMed
Summary

A child with glycogen storage disease type III experienced temporary blindness and EEG changes due to hypoglycemia. This rare occurrence highlights the neurological complications of metabolic disorders in children.

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Area of Science:

  • Pediatric Neurology
  • Metabolic Disorders
  • Neurophysiology

Background:

  • Glycogen storage disease type III (GSD III) is a rare inherited metabolic disorder.
  • Hypoglycemia is a common complication in GSD III, potentially leading to neurological issues.
  • Acute cortical blindness is a rare but serious neurological manifestation.

Observation:

  • A 7-year-old boy with GSD III presented with recurrent episodes of transient acute cortical blindness.
  • These episodes were temporally associated with documented hypoglycemia.
  • Electroencephalogram (EEG) revealed transient high-voltage slowing in the occipital regions during these events.

Findings:

  • The patient experienced two distinct episodes of acute cortical blindness linked to hypoglycemia.

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  • EEG abnormalities, specifically occipital slowing, correlated with the visual disturbances.
  • This case represents the second documented instance of acute cortical blindness associated with hypoglycemia in the pediatric department within five years.

    • Implications:

    • This case underscores the potential for severe neurological complications, including visual disturbances, in pediatric patients with GSD III.
    • Prompt recognition and management of hypoglycemia are critical to prevent or mitigate neurological sequelae.
    • Further research may elucidate the precise mechanisms linking hypoglycemia to cortical blindness in metabolic disorders.