Parkinson's disease and parkinsonism: Clinicopathological discrepancies on diagnosis in three patients

Abstract

Parkinson's disease (PD) is one of the most common neurodegenerative disorders. The cardinal neuropathological features of PD include selective and progressive loss of pigmented neurons in the substantia nigra, deficiencies in dopaminergic signaling in the striatum, and occurrence of phosphorylated α-synuclein-identified Lewy bodies in the nervous system. Parkinsonism, the clinical presentation of movement disorders seen in PD, is a feature shared commonly by other pathologically distinct neurodegenerative diseases, such as progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), and multiple system atrophy (MSA). Consequently, it is sometimes difficult to distinguish PD from such parkinsonism-related neurological disorders. In addition, parkinsonism is not always a feature of certain neurodegenerative diseases, and it can sometimes develop as a result of various forms of drug intoxication or cerebrovascular disease. Here, we describe the clinicopathological features of three patients (cases 1, 2, and 3) diagnosed as having PSP, MSA, and PD, respectively, in each of whom the postmortem histopathological diagnosis differed from the final clinical diagnosis. Neuropathologically, they had suffered from coexistent disorders: PD, MSA, and argyrophilic grain disease (case 1); PD (case 2); and vascular parkinsonism (case 3). The variety of patients showing features of parkinsonism underlines the importance of careful long-term follow up followed by postmortem neuropathological evaluation.

Neuropathology Case Cluster: Neuropathology of focal cortical, behavioral, and psychiatric syndromes in neurodegenerative diseases (neuropathology cluster case 14–32)

“…The authors disclose no conflict of interest. Shinobu Kawakatsu, 1 Toshiki Uchihara, 2,3,4 Masaki Takao 5 and Noriyuki Shibata…”

“…Traditionally, in Japan, dedicated psychiatrists and neurologists have made great efforts to evaluate the heterogeneity of these neuropsychiatric symptoms neuropathologically as well as neuropsychologically. Herein, neurodegenerative diseases with primary progressive aphasia or late‐onset psychosis that have recently attracted attention in clinical settings are presented as sequels to the Neuropathological Case Cluster ( NCC ), inaugurated in Neuropathology 1–5 . NCC will increase the significance of case reports by contrasting multiple cases and encourage many researchers to report case series.…”

“…Herein, neurodegenerative diseases with primary progressive aphasia or late-onset psychosis that have recently attracted attention in clinical settings are presented as sequels to the Neuropathological Case Cluster (NCC), inaugurated in Neuropathology. [1][2][3][4][5] NCC will increase the significance of case reports by contrasting multiple cases and encourage many researchers to report case series.…”