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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.
Abstract:
BACKGROUND Rapid eye movement (REM) sleep behavior disorder (RBD) is a REM-related parasomnia in which the normal muscle atonia of REM sleep is lost, leading to complex motor behaviors or dream enactments. RBD is increasingly recognized as a prodromal manifestation of neurodegenerative a-synucleinopathy. It is well established in longitudinal studies that isolated RBD is among the strongest predictors of a-synucleinopathies, with more than 80-90% of patients eventually converting to Parkinson disease, dementia with Lewy bodies (DLB), or multiple system atrophy. CASE REPORT We report a 69-year-old woman with a previous history of RBD who was admitted for progressive cognitive and behavioral deterioration after starting antiseizure and antipsychotic medications. On admission, she exhibited bradykinesia and rigidity and dopamine transporter positron emission tomography (PET) imaging demonstrated reduced uptake in the putamina. During hospitalization, dream-enacting behaviors were observed, leading to the decision to conduct polysomnography, which confirmed loss of muscle atonia during REM sleep. Taken together, these findings supported a diagnosis of DLB and RBD. In addition, brain magnetic resonance imaging with susceptibility-weighted sequences revealed multiple cortical microbleeds consistent with cerebral amyloid angiopathy, prompting amyloid PET imaging, which showed diffuse cortical amyloid deposition and indicated concurrent amyloid pathology. CONCLUSIONS This case highlights that RBD and characteristic neuroimaging findings are consistent with DLB as the primary disease, with coexisting amyloid pathology. Additionally, the potential influence of clinical confounders such as cerebral amyloid angiopathy, seizure, and medication effects must be taken into account.
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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