2023 Volume 60 Issue 2 Pages 76-81
Progressive supranuclear palsy (PSP) is one of the common four-repeat tauopathies. Clinical symptoms of autonomic dysfunction, such as orthostatic hypotension, urogenital disorder, pupillomotor function, and central hypoventilation, frequently recommend differential diagnosis from α-synucleinopathies, Parkinson’s disease, dementia with Lewy bodies, and multiple system atrophy (MSA). In this mini review, we focus on neuropathology of autonomic nervous system in PSP. Pathologically, 4 repeat tau positive neuroglial inclusions, neurofibrillary tangles, pretangles, threads, and coiled bodies are consistently observed in autonomic nervous system in PSP, including intermediolateral nucleus, Onuf ’s nucleus, Edinger-Westphal nucleus, and ventrolateral nucleus of the medulla. Neuronal loss of the intermediolateral nucleus and Onuf ’s nucleus is less prominent in PSP compared to MSA. Furthermore, 4 repeat tau pathology in PSP is also remarkable in higher control circuitry relevant to autonomic function, involving medial fontal cortex, ventral tegmental area of midbrain, and pontine tegmental area. Therefore, autonomic dysfunction of PSP may correlate with combination of the tauopathy in these characteristic autonomic nuclei and higher neuronal circuitry relevant to autonomic system.
