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Area of Science:

  • Neurology
  • Metabolic Disorders
  • Genetics

Background:

  • Alkaptonuria (AKU) is a rare autosomal recessive metabolic disorder.
  • Characterized by deficiency of the enzyme homogentisate 1,2-dioxygenase (HGD).
  • Leads to accumulation of homogentisic acid (HGA) in various tissues.

Observation:

  • Central nervous system (CNS) effects of AKU are documented.
  • Peripheral nervous system (PNS) involvement is less commonly described.
  • A 72-year-old male with known AKU presented with bilateral carpal tunnel syndrome and left cubital tunnel syndrome.

Findings:

  • The patient's rapid onset of symptoms suggests multifocal pathology.
  • Homogentisic acid deposition in the peripheral nerves is implicated.
  • Nerve compression syndromes may arise from HGA accumulation.

Implications:

  • Highlights the potential for significant peripheral nerve pathology in AKU.
  • Suggests a need for increased awareness and surveillance of PNS complications in AKU patients.
  • May inform future diagnostic and therapeutic strategies for neurological manifestations of AKU.