Background: An 82-year-old right-handed man, a retired teacher, reported the occurrence, three years earlier, of difficulties in moving his left arm and foot, tremor in his left hand, and gestures of the left upper limb that appeared to be independent of the patient's will.Objective: We describe an unusual case of corticobasal syndrome (CBS) showing disease-associated biomarkers of dementia with Lewy bodies (DLB).Methods: Clinical, neuropsychological, imaging, and biomarker evaluations were conducted, including tau and amyloid-beta levels in the cerebrospinal fluid (CSF) and a RT-QuIC assay for alpha-synuclein both in the CSF and olfactory mucosa (OM), as well as a QEEG assessment.Results: The patient presented resting tremor, mild extrapyramidal hypertonus, mild bradykinesia on the left side, and severe apraxia on the left upper limb. Brain MRI showed a diffuse right hemisphere atrophy which was prominent in the posterior parietal and temporal cortices, and moderate in the frontal cortex and the precuneus area. F-18-FDG PET imaging showed reduced glucose metabolism in the right lateral parietal, temporal, and frontal cortices with involvement of the right precuneus. The putamen did not appear to be pathological at DaTQUANT. Neuropsychological tests showed memory and visual-perceptual deficits. CSF tau and amyloid measurements did not show clear pathological values. RT-QuIC for alpha-synuclein in CSF and OM samples were positive. The QEEG analysis showed a pre-alpha dominant frequency in posterior derivations, typical of early stages of DLB.Conclusion: Although in the present patient the clinical diagnosis was of probable CBS, unexpectedly positive biomarkers for DLB suggested the co-presence of multiple pathologies.

Patient with corticobasal syndrome showing disease-associated biomarkers of dementia with Lewy Bodies: a Treviso Dementia (TREDEM) registry case report

Zanusso, Gianluigi;
2022

Abstract

Background: An 82-year-old right-handed man, a retired teacher, reported the occurrence, three years earlier, of difficulties in moving his left arm and foot, tremor in his left hand, and gestures of the left upper limb that appeared to be independent of the patient's will.Objective: We describe an unusual case of corticobasal syndrome (CBS) showing disease-associated biomarkers of dementia with Lewy bodies (DLB).Methods: Clinical, neuropsychological, imaging, and biomarker evaluations were conducted, including tau and amyloid-beta levels in the cerebrospinal fluid (CSF) and a RT-QuIC assay for alpha-synuclein both in the CSF and olfactory mucosa (OM), as well as a QEEG assessment.Results: The patient presented resting tremor, mild extrapyramidal hypertonus, mild bradykinesia on the left side, and severe apraxia on the left upper limb. Brain MRI showed a diffuse right hemisphere atrophy which was prominent in the posterior parietal and temporal cortices, and moderate in the frontal cortex and the precuneus area. F-18-FDG PET imaging showed reduced glucose metabolism in the right lateral parietal, temporal, and frontal cortices with involvement of the right precuneus. The putamen did not appear to be pathological at DaTQUANT. Neuropsychological tests showed memory and visual-perceptual deficits. CSF tau and amyloid measurements did not show clear pathological values. RT-QuIC for alpha-synuclein in CSF and OM samples were positive. The QEEG analysis showed a pre-alpha dominant frequency in posterior derivations, typical of early stages of DLB.Conclusion: Although in the present patient the clinical diagnosis was of probable CBS, unexpectedly positive biomarkers for DLB suggested the co-presence of multiple pathologies.
18F-FDG-PET
TREDEM
corticobasal syndrome
dementia with Lewy bodies
magnetic resonance
neurodegenerative diseases
quantitative electroencephalography
real-time quaking-induced conversion
tauopathy
α-synuclein
File in questo prodotto:
File Dimensione Formato  
adr_2022_6-1_adr-6-1-adr220026_adr-6-adr220026.pdf

accesso aperto

Descrizione: CC BY-NC 4.0 publisher version
Tipologia: Versione dell'editore
Licenza: Creative commons
Dimensione 318.99 kB
Formato Adobe PDF
318.99 kB Adobe PDF Visualizza/Apri

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11562/1076127
Citazioni
  • ???jsp.display-item.citation.pmc??? 0
  • Scopus 0
  • ???jsp.display-item.citation.isi??? 0
social impact