Late-onset Myoclonic Seizure in a 78-year-old Woman with Gaucher Disease

Intern Med. 2024 Mar 15;63(6):861-865. doi: 10.2169/internalmedicine.1699-23. Epub 2023 Aug 9.

Abstract

We herein report a 78-year-old woman with Gaucher disease (GD) who was initially diagnosed with GD type 1, had been receiving long-term enzyme replacement therapy since 58 years old, and developed neurological manifestations in her 70s. The neurological manifestations included myoclonic seizures and progressive cognitive decline. Although it is rare for GD patients to first develop neurologic manifestations at such an advanced age, physicians engaged in long-term care for GD patients should be alert for this possibility.

Keywords: GBA1 variant; enzyme replacement therapy (ERT); epilepsy; neurological symptoms; neuronopathic gaucher disease; parkinsonism.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Enzyme Replacement Therapy
  • Female
  • Gaucher Disease* / complications
  • Gaucher Disease* / diagnosis
  • Gaucher Disease* / drug therapy
  • Glucosylceramidase / therapeutic use
  • Humans
  • Long-Term Care
  • Seizures / etiology

Substances

  • Glucosylceramidase