Abstract
BackgroundWe aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite progressive supranuclear palsy (PSP).MethodsPyramidal motor system pathologies were analyzed in 18 cases with neuropathologically confirmed PSP. Based on a retrospective clinical analysis, cases were subtyped according to Movement Disorder Society criteria for clinical diagnosis of PSP as probable, possible or suggestive of PSP with Richardson’s syndrome (n = 10), PSP with predominant corticobasal syndrome (n = 3), PSP with predominant parkinsonism (n = 3), PSP with predominant speech/language disorder (n = 1), and PSP with progressive gait freezing (n = 1). Clinical manifestations of motor neuron involvement (pseudobulbar or bulbar signs and spasticity) were retrospectively assessed semiquantitatively. Neuropathologically, hyperphosphorylated tau-related pyramidal motor system neuronal, neuritic, and glial pathology using anti-tau AT8 clone immunohistochemistry, was also evaluated.ResultsClinical manifestations of pyramidal motor system involvement were found in patients with different PSP subtypes. A statistically significant higher load of tau pathology was found in the pyramidal system in PSP-Richardson’s syndrome compared to other PSP subtypes (p = 0.016); however, there was no significant correlation between pyramidal system tau pathology and related motor clinical symptoms.ConclusionsTau pathology in the spinal cord and pyramidal motor system structures is very common in progressive supranuclear palsy and may neuropathologically supplement the distinction between classic Richardson’s syndrome from other progressive supranuclear palsy subtypes.
Highlights
We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite progressive supranuclear palsy (PSP)
Clinical manifestation The principal clinical findings are reported in detail as clinical vignettes provided as Additional file 2: Text
We present a systematic clinical and neuropathological analysis of the pyramidal motor system and spinal cord tau pathology in 18 neuropathologically confirmed PSP cases, including different PSP variants: PSP-RS, PSP with predominant corticobasal syndrome (PSP-CBS), PSP with predominant parkinsonism (PSP-P), PSP with progressive gait freezing (PSP-PGF), and PSP with predominant speech/language disorder (PSP-SL)
Summary
We aimed to produce a detailed neuropathological analysis of pyramidal motor system pathology and provide its clinical pathological correlation in cases with definite progressive supranuclear palsy (PSP). Typical clinical findings include vertical gaze palsy, gait disturbance, postural instability, frequent falls, akinesia, and symmetric rigidity. Other PSP variants have different clinical presentations, such as initial PSP with predominant parkinsonism Stejskalova et al BMC Neurology (2019) 19:42 dopa-unresponsive parkinsonism with late dementia and falls); PSP with progressive gait freezing (PSP-PGF); PSP with predominant corticobasal syndrome (PSP-CBS: asymmetric dystonia with parkinsonism, limb apraxia, cortical sensory loss, and alien limb); PSP with predominant speech/language disorder (PSP-SL: overlaps the nonfluent/agrammatical variant of progressive aphasia or speech apraxia with parkinsonism); PSP with predominant ocular motor dysfunction (PSP-OM); PSP with postural instability (PSP-PI); PSP with early frontal lobe cognitive or behavioral presentations (PSP-F: including the behavioral variant of frontotemporal dementia with parkinsonism), and very rare forms involving primary lateral sclerosis (PSP-PLS) and cerebellar ataxia (PSP-C) [1].
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
