Upper motor neuron-predominant motor neuron disease presenting as atypical parkinsonism: A clinicopathological study.

Autor: Murakami A; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA.; Department of Neurology, Kansai Medical University, Osaka, Japan., Koga S; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA.; Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA., Fujioka S; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA.; Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA.; Department of Neurology, Fukuoka University, Fukuoka, Japan., White AE; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA., Bieniek KF; Department of Pathology & Laboratory Medicine, University of Texas Health Science Center San Antonio, Texas, USA., Sekiya H; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA., DeJesus-Hernandez M; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA., Finch NA; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA., van Blitterswijk M; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA., Nakamura M; Department of Neurology, Kansai Medical University, Osaka, Japan., Tsuboi Y; Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA., Murray ME; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA., Wszolek ZK; Department of Neurology, Mayo Clinic, Jacksonville, Florida, USA., Dickson DW; Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA.
Jazyk: angličtina
Zdroj: Brain pathology (Zurich, Switzerland) [Brain Pathol] 2024 Jul 10, pp. e13286. Date of Electronic Publication: 2024 Jul 10.
DOI: 10.1111/bpa.13286
Abstrakt: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by upper and lower motor neuron signs. There are, however, cases where upper motor neurons (UMNs) are predominantly affected, leading to clinical presentations of UMN-dominant ALS or primary lateral sclerosis. Furthermore, cases exhibiting an UMN-predominant pattern of motor neuron disease (MND) presenting with corticobasal syndrome (CBS) have been sparsely reported. This study aims to clarify the clinicopathological features of patients with UMN-predominant MND. We reviewed 24 patients with UMN-predominant MND with TDP-43 pathology in the presence or absence of frontotemporal lobar degeneration. Additionally, we reviewed the medical records of patients with pathologically-confirmed corticobasal degeneration (CBD) who received a final clinical diagnosis of CBS (n = 10) and patients with pathologically-confirmed progressive supranuclear palsy (PSP) who received a final clinical diagnosis of PSP syndrome (n = 10). Of 24 UMN-predominant MND patients, 20 had a clinical diagnosis of an atypical parkinsonian disorder, including CBS (n = 11) and PSP syndrome (n = 8). Only two patients had antemortem diagnoses of motor neuron disease. UMN-predominant MND patients with CBS less frequently exhibited apraxia than those with CBD, and they were less likely to meet clinical criteria for possible or probable CBS. Similarly, UMN-predominant MND patients with PSP syndrome less often met clinical criteria for probable PSP than PSP patients with PSP syndrome. Our findings suggest that UMN-predominant MND can mimic atypical parkinsonism, and should be considered in the differential diagnosis of CBS and PSP syndrome, in particular when criteria are not met.
(© 2024 The Author(s). Brain Pathology published by John Wiley & Sons Ltd on behalf of International Society of Neuropathology.)
Databáze: MEDLINE
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