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Biotin responsive limb weakness.

B Adhisivam1, Deonath Mahto, S Mahadevan

  • 1Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry 605 006, India. adhisivam1975@yahoo.co.uk

Indian Pediatrics
|April 7, 2007
PubMed
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A ten-year-old boy experienced sudden paralysis due to biotin deficiency, linked to eating raw eggs. Prompt biotin treatment led to a significant recovery, highlighting the vitamin's importance.

Area of Science:

  • Neurology
  • Nutritional Science
  • Pediatrics

Background:

  • Biotin deficiency is a rare condition that can lead to serious neurological complications.
  • Raw egg consumption is a known risk factor for biotin deficiency due to avidin, a protein that binds biotin.

Observation:

  • A ten-year-old boy presented with acute onset quadriplegia.
  • Neuroimaging revealed characteristic lesions in the basal ganglia, indicative of specific metabolic or toxic insults.

Findings:

  • The patient's quadriplegia and basal ganglia lesions were directly attributed to severe biotin deficiency.
  • Laboratory investigations confirmed low biotin levels, correlating with the clinical presentation.

Implications:

Related Experiment Videos

  • This case underscores the critical role of biotin in neurological function, particularly in children.
  • Dietary counseling regarding the risks of prolonged raw egg consumption is essential for preventing biotin deficiency.
  • Early diagnosis and supplementation with biotin can reverse severe neurological deficits, demonstrating the efficacy of targeted nutritional therapy.