Normal Pressure Hydrocephalus in Down Syndrome: The Report of Two Cases

J Alzheimers Dis. 2020;77(3):979-984. doi: 10.3233/JAD-200409.

Abstract

Down syndrome (DS) is the most common cause of intellectual disability in infants and has a well-known relationship with the Alzheimer's disease. The association between DS and the other pathologies of senescence, such as normal pressure hydrocephalus (NPH), has been poorly investigated. This series included two DS patients with NPH. In both cases, NPH symptoms were initially misdiagnosed as DS associated senescence. Patients were treated with ventricular-peritoneal shunt, showing a sustained improvement (1 and 4 years of follow-up). To our knowledge, this is the first description of the occurrence of NPH in adult patients with DS and surgical outcomes.

Keywords: Cerebrospinal fluid; down syndrome; normal pressure hydrocephalus; trisomy 21; ventricular-peritoneal shunt.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Adult
  • Down Syndrome / complications*
  • Down Syndrome / diagnostic imaging*
  • Down Syndrome / surgery
  • Follow-Up Studies
  • Humans
  • Hydrocephalus, Normal Pressure / complications*
  • Hydrocephalus, Normal Pressure / diagnostic imaging*
  • Hydrocephalus, Normal Pressure / surgery
  • Male
  • Middle Aged
  • Retrospective Studies
  • Ventriculoperitoneal Shunt / methods*