Pyramidal system involvement in progressive supranuclear palsy – a clinicopathological correlation

Autor:	Adam Tesar, Zdenek Rohan, Radoslav Matej, Zuzana Stejskalova, Robert Rusina, Jaromir Kukal, Ales Bartos, Gabor G. Kovacs
Jazyk:	angličtina
Rok vydání:	2019
Předmět:	Male medicine.medical_specialty Pathology Neurology Pyramidal Tracts tau Proteins lcsh:RC346-429 Progressive supranuclear palsy 03 medical and health sciences 0302 clinical medicine Parkinsonian Disorders Motor system medicine Humans Neurochemistry 030212 general & internal medicine Spasticity lcsh:Neurology. Diseases of the nervous system Aged Retrospective Studies Aged 80 and over Cerebral Cortex Spinal cord Movement Disorders Clinical pathology business.industry Parkinsonism food and beverages General Medicine Middle Aged Motor neuron medicine.disease Immunohistochemistry eye diseases medicine.anatomical_structure Atypical parkinsonism Tauopathies Female Supranuclear Palsy Progressive Neurology (clinical) medicine.symptom business 030217 neurology & neurosurgery Research Article
Zdroj:	BMC Neurology, Vol 19, Iss 1, Pp 1-8 (2019) BMC Neurology
ISSN:	1471-2377
DOI:	10.1186/s12883-019-1270-1
Popis:	Background 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). Methods Pyramidal 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. Results Clinical 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. Conclusions Tau 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. Electronic supplementary material The online version of this article (10.1186/s12883-019-1270-1) contains supplementary material, which is available to authorized users.
Databáze:	OpenAIRE
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