Coexisting Amyloid Positivity and Probable Dementia With Lewy Bodies in a Patient With Rapid Eye Movement Sleep

Jae Hyeok Choi1, Seungju Kim1, Jaeyeong Bae1

  • 1Department of Neurology, Soonchunhyang University Cheonan Hospital, Cheonan, South Korea.

Insights

Rapid eye movement (REM) sleep behavior disorder (RBD) predicts neurodegenerative diseases like dementia with Lewy bodies (DLB). This case shows RBD with DLB and concurrent amyloid pathology, highlighting potential confounders.

Area of Science:

  • Neurology
  • Sleep Medicine
  • Neurodegenerative Diseases

Background:

  • Rapid eye movement (REM) sleep behavior disorder (RBD) involves loss of muscle atonia during REM sleep, leading to dream enactment.
  • RBD is a significant prodromal marker for synucleinopathies, including Parkinson disease and dementia with Lewy bodies (DLB).
  • Over 80-90% of isolated RBD patients eventually develop a synucleinopathy.

Purpose of the Study:

  • To report a case of a patient with RBD, cognitive decline, and motor symptoms.
  • To investigate the underlying neurodegenerative and cerebrovascular pathologies.
  • To highlight the diagnostic considerations and potential confounders in such cases.

Main Methods:

  • Clinical assessment including neurological examination and medication review.
  • Dopamine transporter (DAT) and amyloid positron emission tomography (PET) imaging.
  • Polysomnography to confirm REM sleep without atonia.
  • Brain magnetic resonance imaging (MRI) with susceptibility-weighted sequences.

Main Results:

  • A 69-year-old woman with a history of RBD presented with cognitive and behavioral decline, bradykinesia, and rigidity.
  • DAT-PET showed reduced putaminal uptake, suggestive of nigrostriatal degeneration.
  • Polysomnography confirmed REM sleep without atonia, consistent with RBD.
  • Brain MRI revealed microbleeds indicating cerebral amyloid angiopathy (CAA).
  • Amyloid PET showed diffuse cortical amyloid deposition, indicating concurrent amyloid pathology.
  • Diagnosis of DLB with coexisting amyloid pathology was established.

Conclusions:

  • RBD and neuroimaging findings supported DLB as the primary diagnosis.
  • Concurrent amyloid pathology was identified alongside DLB.
  • Clinical confounders like CAA, seizures, and medication effects require careful consideration in managing patients with RBD and cognitive decline.

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